11. Obstetrics and Gynecology

Authors: Dahnert, Wolfgang

Title: Radiology Review Manual, 6th Edition

Copyright 2007 Lippincott Williams & Wilkins

> Table of Contents > Obstetrics and Gynecology

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Obstetrics and Gynecology

Differential Diagnosis of Obstetric and Gynecologic Disorders

2 obstetrics

Parity Nomenclature (for pregnancies <20 weeks)

example:	G5P4004
Gravida	5 pregnancies
Parity
mnemonic:	FPAL
Full term	4 full term
Preterm	0 preterm
Abortion	0 abortion
Living	4 living

Level I Obstetric Ultrasound

Indication:

MS-AFP 2.5 multiples of mean (MoM) between 14 and 18 weeks MA

• Limited scope of examination to identify frequent causes of MS-AFP elevation in 20 50% of pregnancies:

  • Gestational age 2 weeks more advanced than estimated clinically (18%)

  • Multiple gestations (10%)

  • Unsuspected fetal demise (5%)

  • Obvious fetal NTD / abdominal wall defect

  Outcome:

  no cause identified in 50 80%

• Recommendation if level I ultrasound is unrevealing:

  • amniocentesis for AF-AFP (with normal results in >90%)

  • level II obstetric ultrasound (skipping amniocentesis)

Level II Obstetric Ultrasound

Indication:	AF-AFP 2 MoM
Accuracy:	identification of abnormal fetuses in 99%

• Examination targeted for:

  • Open neural tube defect:

    • anencephaly, encephalocele, open spina bifida, amniotic band syndrome resulting in open neural tube defect

  • Closed neural axis anomaly:

    • hydrocephalus, Dandy-Walker malformation

  • Abdominal wall defect:

    • gastroschisis, omphalocele, gastropleuroschisis from amniotic band syndrome

  • Upper GI obstruction:

    • esophageal atresia tracheoesophageal fistula, duodenal obstruction

  • Cystic hygroma

  • Teratoma:

    • sacrococcygeal, lingual, retropharyngeal

  • Renal anomalies:

    Alpha-fetoprotein Levels
    Sample Site	Approximate Level (ng/mL)	Peak
    Maternal serum	30	30th 32nd week
    Amniotic fluid	20,000	2nd trimester
    Fetal plasma	3,000,000	14th 15th week

    • obstructive uropathy, renal agenesis, multicystic dysplastic kidney, congenital Finnish nephrosis

• Risk of fetal chromosomal anomaly is only 0.6 1.1% with normal level II sonogram!

Maternal serum screening

Alpha-fetoprotein

• = glycoprotein as major circulatory protein of early fetus

Origin:

formed initially by yolk sac + fetal gut (4 8 weeks), later by fetal liver

• Detectable in

  • fetal serum

    • concentration peaks at 14 15 weeks followed by progressive decline

  • amniotic fluid (AF-AFP) is a result of

    • fetal urination

    • fetal gastrointestinal secretions

    • transudation across fetal membranes (amnion, placenta)

    • transudation across immature fetal epithelium

    • concentration peaks early in 2nd trimester followed by progressive decline

  • maternal circulation (MS-AFP) secondary to leakage from amniotic fluid across the placenta

    • levels start to rise at 7th week, peak at 32nd week, and decline toward end of pregnancy

    • Either high / low MS-AFP is associated with 34% of all major congenital defect

• At the end of the 1st trimester AFP is present:

  in fetal plasma	in milligram	quantities
  in amniotic fluid	in microgram	quantities
  in maternal serum	in nanogram	quantities

  Reported in MoM =

  multiples of mean to standardize interpretation among laboratories

Elevated Alpha-fetoprotein

• screening at 16 18 weeks GA

• Values must be corrected for dates, maternal weight, race, presence of diabetes (diabetes has depressing effect on MS-AFP so that lower levels may be associated with NTDs)

• Associated with:

  • LABORATORY ERROR

  • ERRONEOUS DATES (18%):

    • GA 2 weeks more advanced sonographically than by clinical estimate (AFP levels rise 15% per week during 16 18-week window)

  • MULTIPLE GESTATIONS (14%)

  • FETAL DEMISE (7%) / fetal distress / threatened abortion

  • FETAL ANOMALIES (61%)

    • Neural tube defects (51%):

      • [anencephaly (30%), myelomeningocele (18%), encephalocele (3%), forebrain malformation]

        P.996

        
        

        Prevalence:

        1.6:1,000 births in USA; 6:1,000 births in Great Britain

      • In 90% as 1st time event!

        Risk of recurrence:

        3% after one affected child; 6% after 2 affected children

    • Ventral wall defects (21%)

      • (gastroschisis, omphalocele): sensitivity of 50%

    • Proximal fetal gut obstruction

      • (esophageal / duodenal atresia)

      • = diminished AFP degradation in small bowel

    • Cystic hygroma, teratoma (pharyngeal, sacral)

    • Amniotic band syndrome

      • (asymmetric cephalocele, gastropleuroschisis)

    • Renal abnormalities:

      • multicystic dysplastic kidney, renal agenesis, pelviectasis, congenital Finnish nephrosis (typically 10 MoM + negative amniotic fluid acetylcholinesterase)

    • Oligohydramnios

  • PLACENTAL LESION

    • altering the placentomaternal barrier

      • Chorioangioma

      • Peri- and intraplacental hematoma

        • resulting in fetomaternal hemorrhage

      • Placental lakes, infarct, intervillous thrombosis

  • LOW BIRTH WEIGHT

  • Normal pregnancy + MATERNAL DISORDER

    • Hepatitis

    • Hepatoma

  • Fetal-maternal blood mixing:

    • collection of MS-AFP samples after amniocentesis

      mnemonic:

      GEM MINER CO

    • Gastroschisis

    • Esophageal atresia

    • Multiple gestations

    • Mole

    • Incorrect menstrual dates

    • Neural tube defects

    • Error (laboratory)

    • Renal disease in fetus (autosomal recessive polycystic kidney disease, renal dysplasia, obstructive uropathy, congenital Finnish nephrosis)

    • Chorioangioma

    • Omphalocele

Elevated Maternal Serum AFP (MS-AFP)

• = defined as 2.5 MoM / equivalent to the 5th percentile; 4.5 MoM for multiple gestations

• Power of detection at 2.5 MoM cutoff:

  • 98% of gastroschisis

  • 90% of anencephalic fetuses

  • 75 80% of open spinal defects

  • 70% of omphaloceles

  Incidence:

  2 5% screen-positive rate (in 16% normal MS-AFP on retesting); 6 15% of fetuses have some type of major congenital defect; in 1.3:1,000 tests fetal anomaly detected

• The higher the AFP elevation the higher the probability of fetal anomalies

• 20 38% of women with unexplained high MS-AFP (ie, in absence of fetal abnormality) suffer adverse pregnancy outcomes (premature birth, preeclampsia, 2 4 IUGR, 10 perinatal mortality, 10 placental abruption)!

Elevated Amniotic Fluid AFP (AF-AFP)

• = defined as 2 MoM (<2 MoM has a 97% NPV)

  Incidence:

  <10% of women with elevated MS-AFP and unrevealing level I US exam

• amniotic fluid also tested for karyotype + acetylcholinesterase (= neurotransmitter enzyme present when neural tissue is exposed)

• 66% of fetuses of women with elevated AF-AFP levels are normal!

• A targeted level II ultrasound exam will show fetal anomalies in 33%!

Low Alpha-fetoprotein

• = MS-AFP 0.5 / AF-AFP 0.72 MoM

Incidence:

3%

• Autosomal trisomy syndromes (trisomy 21, 18, 13)

  • 20% of trisomy 21 fetuses are found in women with low MS-AFP after adjustment for age!

• Absence of fetal tissues (eg, hydatidiform mole)

• Fetal demise

• Misdated pregnancy

• Normal pregnancy

• Patient not pregnant

Use of Karyotyping

Frequency:

11 35% of fetuses with sonographically identified abnormalities have chromosomal abnormalities

• FETAL ANOMALIES

  • CNS anomalies: holoprosencephaly (43 59%), Dandy-Walker malformation (29 50%), cerebellar hypoplasia, agenesis of corpus callosum, myelomeningocele (33 50%)

  • Cystic hygroma (72%): Turner syndrome

  • Omphalocele (30 40%)

  • Cardiac malformations

  • Nonimmune hydrops

  • Duodenal atresia

  • Severe early-onset IUGR: trisomy 18, 13, triploidy

  • Diaphragmatic hernia

  • Bone-echodense bowel (20%): trisomy 21

• MATERNAL RISK FACTORS

  • Advanced age

  • Low serum alpha-fetoprotein

  • Abnormal triple screen of maternal serum

  • History of previous chromosomally abnormal pregnancy (1% risk of recurrence)

• PLANNED INTENSE INTRAUTERINE MANAGEMENT

• Fetal anomalies not associated with chromosomal anomalies:

  • Gastroschisis

  • Unilateral renal anomaly

  • Intestinal obstruction distal to duodenal bulb

  • Off-midline unilateral cleft lip

  P.997

  
  

  • Fetal teratoma (sacrococcygeal / anterior cervical)

  • Isolated single umbilical artery

Amniotic fluid volume

• Production:

  • 1st trimester: dialysate of maternal + fetal serum across the noncornified fetal skin

  • 2nd + 3rd trimester: fetal urine (600 800 cm³/day near term), fetal lungs (600 800 cm³/day near term), amniotic membrane

• Absorption:

  • fetal swallowing + GI absorption, fetal lung absorption, clearance by placenta

• Assessment of amniotic fluid volume by:

  • Subjective assessment ( Gestalt method):

    • quick + efficient, accounts for GA-related variations in fluid volume, considered the most accurate if performed by experienced operator, operator + interpreter must be identical, no documentation, variations on serial scans difficult to appreciate

  • Depth of largest vertical pocket:

    • simple + quick (used in BPP), pockets >2 cm may be found in crevices between fetal parts with moderately severe oligohydramnios, does not account for GA-related variations

  • Four-quadrant Amniotic Fluid Index (AFI):

    • fairly quick, probably correlates better with fluid volume than any single measurement, may not accurately reflect overall fluid volume, may be affected by fetal movement during measurements

      Aneuploid Risk of Major Anomalies

      Structural Defect	Incidence	Aneuploidy
      		Risk	Most common
      Cystic hygroma	1:6,000	60-75%	45X,21,18,13, XXY
      Hydrops	1:4,000	30 80%	13,21,18,45X
      Holoprosencephaly	1:16,000	40 60%	13,18,18p
      Cardiac defects	1:125	5 30%	21,18,13,22
      AV canal	40 70%	21
      Omphalocele	1:5,800	30 40%	13,18
      Duodenal atresia	1:10,000	20 30%	21
      Diaphragmatic hernia	1:4,000	20 25%	13,18,21,45X
      Bladder outlet obstruction	1:1,000	20 25%	13,18
      Limb reduction	1:2,000	8%	18
      Clubfoot	1:830	6%	18,13,4p-,18q-
      Hydrocephalus	1:1,250	3 8%	13,18, triploidy
      Facial cleft	1:700	1%	13,18, deletions
      Prune belly	1:40,000	low	18,13,45X
      Single umbilical artery	1:100	minimal
      Bowel obstruction	1:4,000	minimal
      Gastroschisis	1:12,000	minimal

  • Planimetric measurement of total intrauterine volume

  • Dye / para-amino hippurate dilution technique:

    • 800 cm³ at 34 weeks, 500 cm³ >34 weeks

Polyhydramnios

• = amniotic fluid volume >1,500 2,000 cm³ at term

  Incidence:

  1.1 2 3.5%

• fetus does not fill the AP diameter of uterus

• single largest pocket devoid of fetal parts / cord >8 cm in vertical direction

• AFI 20 24 cm

  Prognosis:

  64% perinatal mortality with severe polyhydramnios

• Etiology:

  • IDIOPATHIC (35%)

    Associated with:	macrosomia in 19 37%
    Suggested cause:

    • increased renal vascular flow

    • bulk flow of water across surface of fetus + umbilical cord + placenta + membranes

  • MATERNAL CAUSES (36%)

    • Diabetes (25%)

    • Isoimmunization [Rh incompatibility (11%)]

    • Placental tumors: chorioangioma

  • FETAL ANOMALIES (20%)

    • gastrointestinal anomalies (6 16%):

      • impairment of fetal swallowing (esophageal atresia in 3%); high intestinal atresias / obstruction of duodenum / proximal small bowel (1.2 1.8%), omphalocele, meconium peritonitis

    • nonimmune hydrops (16%)

    • neural tube defects (9 16%):

      • anencephaly, hydranencephaly, holoprosencephaly, myelomeningocele, ventriculomegaly, agenesis of corpus callosum, encephalocele, microcephaly

    • chest anomalies (12%):

      • diaphragmatic hernia, cystic adenomatoid malformation, tracheal atresia, mediastinal teratoma, primary pulmonary hypoplasia, extralobar sequestration, congenital chylothorax

    • skeletal dysplasias (11%):

      • dwarfism (thanatophoric dysplasia, achondroplasia), kyphoscoliosis, platyspondyly

    • chromosomal abnormalities (9%):

      • trisomy 21, 18, 13

    • cardiac anomalies (5%):

      • VSD, truncus arteriosus, ectopia cordis, septal rhabdomyoma, arrhythmia

    • genitourinary malformations:

      • unilateral UPJ obstruction, unilateral multicystic dysplastic kidney, mesoblastic nephroma

      Cause:

      ? hormonally mediated polyuria

    • miscellaneous (8%):

      • cystic hygroma, facial tumors, cleft lip / palate, teratoma, amniotic band syndrome, congenital pancreatic cyst

    • In polyhydramnios efforts to detect fetal anomalies should be directed at SGA fetuses!

      mnemonic:

      TARDI

• Twins

P.998




• Anomalies, fetal

• Rh incompatibility

• Diabetes

• Idiopathic

Oligohydramnios

• = amniotic fluid volume <500 cm³ at term

• single largest pocket devoid of fetal parts / cord 1 2 cm in vertical direction

• AFI 5 7 cm

  Etiology:
  mnemonic:	DRIPP

  • Demise of fetus / Drugs (Motrin therapy for tocolysis of preterm labor)

  • Renal anomalies, bilateral (= inadequate urine production): renal agenesis / dysgenesis, infantile polycystic kidney disease, prune belly syndrome, posterior urethral valves, urethral atresia, cloacal anomalies

    • 20-fold increase in incidence of fetal anomalies with oligohydramnios!

      N.B.:

      bilateral renal obstruction, if combined with intestinal obstruction, may be associated with polyhydramnios

  • IUGR (reduced renal perfusion)

  • Premature rupture of membranes (most common)

  • Postmaturity

Cx:	pulmonary hypoplasia, cord compression
Prognosis:	77 100% perinatal mortality with 2nd trimester oligohydramnios

Abnormal first trimester findings

Time of onset:

prior to 8 10 weeks

First Trimester Bleeding

• = VAGINAL BLEEDING IN FIRST TRIMESTER

Frequency:

15 25% of all pregnancies, of which 50% terminate in abortion

• INTRAUTERINE conceptus IDENTIFIED

  • Threatened abortion

  • Embryonic demise

  • Blighted ovum

  • Gestational trophoblastic disease

  • Implantation bleed (3 4 weeks after last menstrual period)

  • Subchorionic hemorrhage

  • Low-lying placenta previa

  • Twin loss

• NORMAL ENDOMETRIAL CAVITY

  • with -hCG level >1,800 mlU/mL

    • Recent spontaneous abortion

    • Ectopic pregnancy

  • with -hCG level <1,800 mlU/mL

    • Very early IUP

    • Ectopic pregnancy

• SAC VULNERABILITY

  • Leiomyoma

  • Intrauterine contraceptive device

Abnormal Sonographic Findings in 1st Trimester

• Embryonic demise = abortion (clinical term)

• Nondevelopment = blighted ovum

• Maldevelopment = hydatidiform mole

Empty Gestational Sac

• Normal early IUP between 5 and 7 weeks MA

• Blighted ovum

DDx:

Pseudosac of ectopic pregnancy

Gestational Sac in Low Position

• Abortion in progress

  • no placental blood flow

• Cervical ectopic pregnancy

• Fundal fibroid compressing sac downward

Positive -hCG without IUP

mnemonic:

HERE

• HCG-producing tumor (rare)

• Ectopic pregnancy

• Recent / incomplete abortion

• Early intrauterine pregnancy

Thickened Central Cavity Complex

• Intrauterine blood

• Retained products of conception following an incomplete spontaneous abortion

• Early intrauterine not yet visible pregnancy

• Decidual reaction secondary to ectopic pregnancy

Uterus Large for Dates

• Multiple gestation pregnancy

• Inaccurate menstrual history

• Fibroids

• Polyhydramnios

• Hydatidiform mole

• Fetal macrosomia

Intrauterine membrane in pregnancy

• MEMBRANE OF MATERNAL ORIGIN

  • Uterine septum

    • = incomplete resorption of sagittal septum between the fused two m llerian ducts

  • Amniotic sheet / shelf

    • = folding of amniochorionic membrane around uterine synechia

    • synechia often thins during uterine stretching + disappears as pregnancy progresses

• MEMBRANE OF FETAL ORIGIN

  • Intertwin membrane

    • = apposing membrane of multiple pregnancy

  • Amniotic band

    • = rent within amnion

  • Chorioamnionic separation

    • = incomplete fusion / hemorrhagic separation of amnion (= inner membrane) and chorion (= outer membrane)

  • Subchorionic hemorrhage = chorioamnionic elevation

    • = separation of chorionic membrane from decidua

    • implantation bleed of early pregnancy

P.999




• FIBRIN STRAND

  Cause:	hemorrhage during transplacental amniocentesis
  mnemonic:	STABS

  • Separation (chorioamnionic)

  • Twins (intertwin membrane)

  • Abruption

  • Bands (amniotic band syndrome)

  • Synechia

Dilated Cervix

• Inevitable abortion

• Premature labor

  • = spontaneous onset of palpable, regularly occurring uterine contractions between 20 and 37 weeks MA

• Incompetent cervix

Placenta

Abnormal Placental Size

• Placental mass tends to reflect fetal mass!

• ENLARGEMENT OF PLACENTA = Placentomegaly

  • = >5 cm thick in sections obtained at right angles to long axis of placenta

    • maternal disease

      • Maternal diabetes (= villous edema)

      • Chronic intrauterine infections (eg, syphilis)

      • Maternal anemia (= normal histology)

      • Alpha-thalassemia

    • fetal disease

      • Hemolytic disease of the newborn (= villous edema + hyperplasia) due to immunologic incompatibility including Rh sensitization

      • Umbilical vein obstruction

      • Fetal high-output failure:

        • large chorioangioma, arteriovenous fistula

      • Fetal malformation:

        • Beckwith-Wiedemann syndrome, sacrococcygeal teratoma, chromosomal abnormality, fetal hydrops

      • Twin-twin transfusion syndrome

    • fetomaternal hemorrhage

    • placental abnormalities

      • Molar pregnancy

      • Chorioangioma

      • Intraplacental hemorrhage

        mnemonic:

        HAD IT

        • Hydrops

        • Abruption

        • Diabetes mellitus

        • Infection

        • Triploidy

• DECREASE IN PLACENTAL SIZE

  • Preeclampsia

    • associated with placental infarcts in 33 60%

  • IUGR

  • Chromosomal abnormality

  • Intrauterine infection

Vascular Spaces of the Placenta

• Placental cysts

  • = large fetal veins located between amnion + chorion anastomosing with umbilical vein

  • sluggish blood flow (detectable by real-time observation)

• Basal veins

  • = decidual + uterine veins

  • lacy appearing network of veins underneath placenta

    DDx:

    placental abruption

• Intraplacental venous lakes

  • intraplacental sonolucent spaces

  • whirlpool motion pattern of flowing blood

Macroscopic Lesions of the Placenta

• Intervillous thrombosis (36%)

  • = intraplacental areas of hemorrhage

    Etiology:

    breaks in villous capillaries with bleeding from fetal vessels

  • irregular sonolucent intraplacental lesions (mm to cm range)

  • blood flow may be observed within lesion

    Significance:

    fetal-maternal hemorrhage (Rh sensitization, elevated AFP levels)

• Perivillous fibrin deposition (22%)

  • = nonlaminated collection of fibrin deposition

    Etiology:	thrombosis of intervillous space
    Significance:	none

• Septal cyst (19%)

  Etiology:

  obstruction of septal venous drainage by edematous villi

  • 5 10-mm cyst within septum

  Significance:

  none

• Placental infarct (25%)

  • = coagulation necrosis of villi

    Etiology:

    disorder of maternal vessels, retroplacental hemorrhage

  • not visualized unless hemorrhagic

  • well-circumscribed mass with hyperechoic / mixed echo pattern

    Significance:

    dependent on extent + associated maternal condition

• Subchorionic fibrin deposition (20%)

  • = laminated collection of fibrin deposition

    Etiology:

    thrombosis of maternal blood in subchorionic space

  • subchorionic sonolucent area

    Significance:

    none

• Massive subchorial thrombus

  • = Breus MOLE = preplacental HEMORRHAGE

Placental Tumor

• TROPHOBLASTIC

  • Complete hydatidiform mole

  • Partial hydatidiform mole

  • Invasive mole

  • Choriocarcinoma

• Nontrophoblastic

  • Chorioangioma (in up to 1% of placentas)

  • Teratoma (rare)

  P.1000

  
  

  • Metastatic lesion (rare): melanoma, breast carcinoma, bronchial carcinoma

Unbalanced Intertwin Transfusion

• = unbalanced intertwin transfusion through vascular anastomoses between the two circulations of monochorionic twins

• ACUTE = Twin-embolization syndrome

• CHRONIC = Twin-twin transfusion syndrome

• REVERSE = Acardiac twinning

Umbilical cord

Abnormal Cord Attachment

• Marginal cord attachment (7%)

  • = battledore placenta (flat wooden paddle used in an early form of badminton)

  • no clinical significance

• Velamentous insertion of cord (1%)

• Vasa previa

Umbilical Cord Lesions

• Umbilical cord cysts persisting into 2nd + 3rd trimester are frequently accompanied by fetal anomalies (hernia, intestinal obstruction, urinary tract obstruction, urachal anomalies, omphalocele, cardiac defect, trisomy 18)!

• DEVELOPMENTAL CORD LESION

  • Umbilical hernia

    • = protrusion from anterior abdominal wall with normal insertion of umbilical vessels

    • Predisposed:

      • Blacks, low-birth-weight infants, trisomy 21, congenital hypothyroidism, Beckwith-Wiedemann syndrome, mucopolysaccharidoses

        Prognosis:

        spontaneous closure in first 3 years of life

  • Omphalomesenteric duct cyst

    • near fetal end of cord + eccentric in cord

  • Allantoic cyst

    • = remnant of umbilical vesicle / allantois; usually degenerates by 6 weeks

      Histo:

      lined by single layer of flattened epithelium

    • near fetal end of cord + in center of cord

  • Amniotic inclusion cyst

    • = amniotic epithelium trapped within umbilical cord

  • Mucoid degeneration of umbilical cord

    • = umbilical cord pseudocyst

    • = liquefaction of Wharton jelly / edema

    • focal thickening of Wharton jelly, usually near umbilicus

    • usually resolved by 12 weeks MA

    • Associated commonly with omphalocele

  • Noncoiled straight cord

    • counterclockwise:clockwise umbilical cords = 7:1

    • right-handed:left-handed persons = 7:1

      Incidence:

      3.7 5%

    • absent vascular coiling for entire length of visible cord

      At risk for:

      intrauterine death (8%), stillbirth, fetal anomalies (24%), prematurity, intrapartum heart rate decelerations, fetal distress, meconium staining

• ACQUIRED CORD LESION

  • False knot

    • exaggerated looping of cord vessels causing focal dilatation of cord

    • focal accumulation of Wharton jelly

    • varix of umbilical vessel

    • knoblike protrusion / bulge of cord

  • True knot

    Incidence:	1% of pregnancies
    Cause:	excessive fetal movements
    Predisposed:	long cord, polyhydramnios, small fetus, monoamniotic twins

    • local distension / thrombosis of umbilical vein near cord knot resembling an umbilical cyst

    • tortuosity of cord at level of knot

      Cx:	vascular occlusion + fetal death in utero
      OB management:	expectant

  • Umbilical cord hematoma

    • = rupture of the wall of the umbilical vein secondary to mechanical trauma (torsion, loops, knots, traction) / congenital weakness of vessel wall

      Incidence:	1:5,505 to 1:12,699 deliveries
      Location:	near fetal insertion of umbilical cord (most common)

    • hyper- / hypoechoic mass 1 2 cm in size, multiple (in 18%)

      Cx:	rupture into amniotic cavity with exsanguination
      Prognosis:	52% overall perinatal fetal mortality

  • Neoplasm

    • Angiomyxoma / hemangioma of cord

      Incidence:	22 cases in literature
      Histo:	multiple vascular channels lined by benign endothelium surrounded by edema + myxomatous degeneration of Wharton jelly
      Associated with:	elevated -fetoprotein level
      Location:	more frequently near placental end of umbilical cord

      • hyperechoic / multicystic mass within cord

      • may be associated with pseudocyst

        • (= localized collection of edema)

          Cx:

          premature delivery, stillbirth, hydramnios, nonimmune hydrops, massive hemorrhage due to rupture

    • Other tumors: myxosarcoma, dermoid, teratoma

  • Umbilical vein varix

    Incidence:	<4% of all umbilical cord abnormalities
    Site:	intraamniotic, intraabdominal

    • fusiform dilatation of umbilical vein

    Cx:	(1) Thrombosis with subsequent fetal death
    	(2) Partial thrombosis with IUGR
    Prognosis:	usually no clinical significance

  • Umbilical artery aneurysm

P.1001

Small-for-gestational age fetus (SGA)

• = generic clinical term describing a group of perinates at / below the 10th percentile for gestational age without reference to etiology

• Fetus of appropriate growth (misdiagnosed as small)

• Small normal fetus = constitutionally small fetus (80 85%)

  • No indication for surveillance / intervention!

• Small abnormal fetus = primary growth failure associated with karyotype anomaly / fetal infection (5 10%)

  • Active intervention is of no benefit!

• Dysmature fetus = IUGR = growth failure as a result of uteroplacental insufficiency (10 15%)

  • Intensive management is likely of benefit!

Fetal Overgrowth Disorder

• Beckwith-Wiedemann syndrome

• Simpson-Golabi-Behmenl syndrome

• Perlmann syndrome

Fetal skeletal dysplasia

• = DWARFISM

• = heterogeneous group of bone growth disorders resulting in abnormal shape + size of the skeleton

• More than 200 skeletal dysplasias are known, but only a few are frequent:

  • thanatophoric dysplasia

  • osteogenesis imperfecta type II

  • achondrogenesis

  • heterozygous achondroplasia

• Birth prevalence:

  • 3:10,000 7.6:10,000 births for all skeletal dysplasias;

  • 5:10,000 births for lethal skeletal dysplasias

    Comparison of Antenatal Sonographic Findings in Fetal Overgrowth Syndromes

    Finding	Perlman	BWS	SGBS
    Macrosomia	X	X	X
    Polyhydramnios	X	X	X
    Hepatomegaly	X	X	X
    Nephromegaly	X	X	X
    Hydronephrosis	X	X	X
    Cystic hygroma	X
    Choroid plexus cyst	X
    Agenesis of corpus callosum	X
    Ascites	X
    Macrocephaly	X		X
    Cardiomegaly	X	X
    Cardiac defects		X	X
    Macroglossia		X	X
    Cleft lip / palate		X	X
    Hydrops		X	X
    Placentomegaly		X
    Omphalocele / umbilical hernia		X
    Polydactyly / syndactyly			X
    BWS = Beckwith-Wiedemann syndrome; SGBS =Simpson-Golabi-Behmenl syndrome

    Prevalence of fetal skeletal Dysplasias

    	Birth Prevalence	Perinatal Deaths
    Thanatophoric dysplasia	0.69:10,000*	1:246
    Achondroplasia	0.37:10,000*	none
    Achondrogenesis, type I	0.23:10,000*	1:639
    Achondrogenesis, type II	0.25:10,000*
    Osteogenesis imperf. type II	0.18:10,000*	1:799
    Osteogenesis imperf., others	0.18:10,000*	none
    Asphyxiating thoracic dysplasia	0.14:10,000*
    Hypophosphatasia	0.10:10,000*
    Chondrodysplasia punctata, rhizo	0.09:10,000*	none
    Camptomelic dysplasia	0.05:10,000*	1:3,196
    Chondroectodermal dysplasia	0.05:10,000*	1:3,196
    Cleidocranial dysplasia	0.05:10,000*
    Diastrophic dysplasia	0.02:10,000*
    	* = lethal dysplasias

    Prognosis:	51% lethal due to hypoplastic lungs:
    	23% stillbirths, 32% death in 1st week of life

• Associated with:

  • polyhydramnios

  • small thorax

  • morphologically abnormal bones

• shortening of long bones (common characteristic)

  • Femur length >5 mm below 2 standard deviations suggests skeletal dysplasia!

• femur length / foot length ratio <0.9

• moderate limb shortening of 40 60% of the mean in thanatophoric dysplasia + OI type II

• severe limb shortening of >30% of the mean in achondrogenesis

  DDx features:	mineralization, bowing, fractures, number of digits, fetal movement, thoracic measurement, associated anomalies, age of onset
  DDx:	constitutionally short limbs, severe IUGR

• see also DWARFISM

Fetal Hand Malformation

Polydactyly

• trisomy 13, short-rib-polydactyly syndrome, asphyxiating thoracic dystrophy (Jeune syndrome), Smith-Lemli-Opitz syndrome

  • Postaxial polydactyly

    • chondroectodermal dysplasia (Ellis-van Creveld syndrome), Meckel-Gruber syndrome, hydrolethalus syndrome

  • Preaxial polydactyly

    • orofaciodigital syndrome

Syndactyly

• Apert syndrome, triploidy, Roberts syndrome

Clinodactyly

• trisomy 21, triploidy

Overlapping Digit

• trisomy 18

P.1002

Hitchhiker's Thumb

• diastrophic dysplasia

Flexion Contractures

• trisomy 13 + 18, fetal akinesia deformation sequence

Limb Reduction

• congenital varicella, hypoglossia-hyperdactyly syndrome

Amputation

• amniotic band syndrome

Fetal CNS anomalies

Incidence:	2:1,000 births in USA; 90% as 1st time occurrence
Recurrence:	2 3% after 1st, 6% after 2nd occurrence

• ventricular atrium + cisterna magna are two sensitive anatomic markers for normal brain development!

• HYDROCEPHALUS

  • Aqueductal stenosis

  • Communicating hydrocephalus

  • Dandy-Walker malformation

  • Choroid plexus papilloma

• NEURAL TUBE DEFECT

  Incidence:	1:500 600 live births
  Risk of recurrence:	3 4%

  • Spina bifida

  • Anencephaly

  • Acrania

  • Encephalocele (8 15%)

  • Porencephaly

  • Hydranencephaly

  • Holoprosencephaly

  • Iniencephaly

  • Microcephaly

  • Agenesis of corpus callosum

  • Lissencephaly

  • Arachnoid cyst

  • Choroid plexus cyst

  • Vein of Galen aneurysm

  Associated with:	trisomy 13 and 18
  Increased risk:	low parity, low socioeconomic status, relative infertility, diabetes, obesity, anticonvulsants, folate deficiency

• INTRACRANIAL NEOPLASM

  • Teratoma (>50%): benign / malignant

    Location:

    originate from base of skull

  • Glioblastoma

  • Astrocytoma

Fetal Ventriculomegaly

• Cause:

  • Morphologic anomaly (70 80%):

    • Spina bifida (30 65%)

    • Dandy-Walker malformation

    • Encephalocele

    • Holoprosencephaly

    • Agenesis of corpus callosum

  • Abnormal karyotype (10 20%)

  • Viral infection

  • 20 40% of concurrent anomalies are missed by ultrasound!

• dangling choroid plexus

• width of ventricular atrium >10 mm

Prognosis:

21% survival rate; 50% with intellectual impairment

Isolated Mild Ventriculomegaly

• = atrial width of 10 15 mm

  Prevalence:	1:700 in low-risk population
  	Most common brain anomaly on prenatal sonograms

• Associated structural anomalies (9%):

  • periventricular leukomalacia, subependymal / germinal matrix hemorrhage, partial agenesis of corpus callosum, heterotopia, parenchymal dysplasia

    Associated chromosomal anomalies:	in 4%
    Recommendation:	MRI to diagnose associated structural anomalies
    Prognosis:	80% with isolated mild ventriculomegaly have normal motor + intellectual function at 12 months of age

Lemon Sign

• = flat / inwardly scalloped contour of both frontal bones

• Spina bifida

  Prevalence for fetuses 24 weeks:

  98%

  • (90 93% sensitive, 98 99% specific, 84% PPV for high-risk population, 6% PPV for low-risk population)

    Prevalence for fetuses >24 weeks:

    13%

  • (disappears in 3rd trimester)

• Encephalocele

• Agenesis of corpus callosum

• Thanatophoric dysplasia

• Cystic hygroma

• Diaphragmatic hernia

• Fetal hydronephrosis

• Umbilical vein varix

• Normal fetus (in 0.7 1.3%)

Prenatal Intracranial Calcifications

• Toxoplasmosis

• CMV infection

• Tuberous sclerosis

• Sturge-Weber syndrome

• Venous sinus thrombosis

• Teratoma

Cystic Intracranial Lesion

mnemonic:

CHAP VAN

• Choroid plexus cyst

• Hydrocephalus, Holoprosencephaly, Hydranencephaly

• Agenesis of corpus callosum + cystic dilatation of 3rd ventricle

• Porencephaly

• Vein of Galen aneurysm

• Arachnoid cyst

• Neoplasm (cystic teratoma)

Abnormal Cisterna Magna

• Normal size between 15 and 25 weeks MA:

  • >2 to <10 mm (usually 4 9 mm) in 94 97% of fetuses

• SMALL CISTERNA MAGNA + banana sign

  • Chiari II malformation (with myelomeningocele)

  • Occipital cephalocele

  P.1003

  
  

  • Severe hydrocephalus

• LARGE CISTERNA MAGNA

  • Megacisterna magna

    • cerebellum + vermis remain intact

  • Arachnoid cyst

    • en bloc displacement of cerebellum + vermis

  • Cerebellar hypoplasia

  • Dandy-Walker syndrome (with vermian agenesis)

Fetal orbital anomalies

Hypotelorism

• Holoprosencephaly

• Chromosomal abnormalities: trisomy 13

• Microcephaly, trigonocephaly

• Maternal phenylketonuria

• Meckel-Gruber syndrome

• Myotonic dystrophy

• Williams syndrome

• Oculodental dysplasia

Hypertelorism

• Median cleft syndrome: cleft lip / palate

• Craniosynostosis: Apert /Crouzon syndrome

• Pena-Shokeir syndrome

• Frontal / ethmoidal, sphenoidal encephalocele

• Dilantin / phenytoin effect

Orbital and Periorbital Masses

• Dacryocystocele

• Anterior encephalocele

• Glioma

• Hemangioma

• Teratoma

Fetal neck anomalies

• Cervical myelomeningocele

• Occipital cephalocele

• Cystic hygroma / lymphangioma

• Teratoma

• Branchial cleft cyst

• Enlarged thyroid

• Sarcoma

Nuchal Skin Thickening

• = NUCHAL SONOLUCENCY / FULLNESS / EDEMA

• = skin thickening of posterior neck measured between calvarium + dorsal skin margin

  • 3 mm during 9 13 weeks MA

  • 5 mm during 14 21 weeks MA

  • 6 mm during 19 24 weeks MA

  • The smallest measurement should be used!

  Image plane:	axial / transverse image (slightly craniad to that of the BPD measurement) that includes cavum septi pellucidi, cerebellar hemisphere and cisterna magna (transcerebellar diameter view)
  Incidence:	among the most common anomaly in 1st trimester + early 2nd trimester

• Cause:

  • NORMAL VARIANT (0.06%)

  • CHROMOSOMAL DISORDERS

    • trisomy 21 (in 45 80%), Turner syndrome (45 X0), Noonan syndrome, trisomy 18, XXX syndrome, XYY syndrome, XXXX syndrome, XXXXY syndrome, 18p-syndrome, 13q-syndrome

    • 30 40% of fetuses with Down syndrome have nuchal skin thickening!

  • nonchromosomal DISORDERS

    • Multiple pterygium syndrome = Escobar syndrome

    • Klippel-Feil syndrome (fusion of cervical vertebrae, CHD, deafness (30%), cleft palate

    • Zellweger syndrome = cerebrohepatorenal syndrome (large forehead, flat facies, macrogyria, hepatomegaly, cystic kidney disease, contractures of extremities)

    • Robert syndrome

    • Cumming syndrome

• larger lymphangiomas with radiating septations are usually found with trisomy 18

• nuchal fullness 3 mm during 1st trimester is seen in trisomy 21 / 18 / 13 (30 50% PPV)

• often reverting to normal by 16 18 weeks

• septations within nuchal translucency carries a 20- to 200-fold risk for chromosomal anomalies compared with normal

  Sensitivity:	2 44 75% for detection of trisomy 21
  Specificity:	99% for detection of trisomy 21
  PPV:	69%
  Positive screen:	1.2 3% in general population (exceeding 0.5% risk of amniocentesis)
  False positives:	0.5 2 8.5%
  OB management:	thorough sonographic evaluation at 18 20 weeks MA
  DDx:	chorioamnionic separation

Protruding Tongue

• Macroglossia

• Lymphangioma of the tongue

Macroglossia

• Beckwith-Wiedemann syndrome

• Down syndrome

• Hypothyroidism

• Mental retardation

Fetal chest anomalies

Pulmonary Hypoplasia

Path:

absolute decrease in lung volume / weight for gestational age

• Cause:

  • Prolonged oligohydramnios (20 25%)

  • Skeletal dysplasia (small thorax)

  • Intrathoracic mass (lung compression)

  • Large hydrothorax (lung compression)

  • Neurologic condition (reduced breathing activity)

  • Chromosomal abnormality

  • CHD with R-sided cardiac obstructing lesion

P.1004




• thoracic circumference (TC) <5th percentile for EGA

• declining TC:AC ratio from >0.80 (75% sensitive, 80 90% specific); not applicable for intrathoracic masses

Intrathoracic Mass

• in order of frequency:

  • Diaphragmatic hernia / eventration

  • Cystic adenomatoid malformation

  • Bronchopulmonary sequestration

  • Bronchogenic cyst with bronchial compression

  • Bronchial atresia

Unilateral Chest Mass

• Congenital diaphragmatic hernia

• Cystic adenomatoid malformation

• Bronchopulmonary sequestration

• Bronchogenic cyst

• Unilateral bronchial atresia / stenosis

Bilateral Chest Masses

• Laryngeal / tracheal atresia

• Bilateral cystic adenomatoid malformation

• Bilateral congenital diaphragmatic herniae

Mediastinal Mass

• Goiter

• Cystic hygroma

• Pericardial teratoma

• Neuroblastoma

Cystic Chest Mass

• Bronchogenic cyst

• Enteric cyst

• Neurenteric cyst

• Cystic adenomatoid malformation (type I)

• Congenital diaphragmatic hernia

• Pericardial cyst

• Mediastinal meningocele

Complex Chest Mass

• Congenital diaphragmatic hernia

• Cystic adenomatoid malformation (type I, II, III)

• Pulmonary sequestration

• Complex enteric cyst

• Pericardial teratoma

Solid Chest Mass

• Congenital diaphragmatic hernia (bowel liver)

• Cystic adenomatoid malformation type III

• Pulmonary sequestration

• Obstructed lung from bronchial atresia, laryngeal atresia, bronchogenic cyst

• Bronchopulmonary foregut malformation

• Pericardial tumor

• Heterotopic brain tissue

Regressing Fetal Chest Mass

• Cystic adenomatoid malformation

• Bronchopulmonary sequestration

Chest Wall Mass

• Hemangioma

• Cystic hygroma

• Teratoma

• Hamartoma

• Thoracic myelomeningocele

Pleural Effusion

• Primary idiopathic chylothorax (most common)

• Hydrops fetalis (multiple causes)

• Chromosome anomaly: trisomy 21, 45 XO (mostly)

• Pulmonary lymphangiectasia / cystic hygroma

• Lung mass: cystic adenomatoid malformation, bronchopulmonary sequestration, congenital diaphragmatic hernia, chest wall hamartoma (uncommon)

• Pulmonary vein atresia

• Idiopathic

Fetal cardiac anomalies

Incidence:

1:125 births = 0.8% of population; most common of all congenital malformations (40%)

• 90% occur as isolated multifactorial traits with a recurrence risk of 2 4%

• 10% are associated with multiple birth defects

• responsible for 50% of childhood deaths from congenital malformations

• Antenatal sonographic diagnosis to prompt cardiac evaluation:

  • ABNORMALITIES IN CARDIAC POSITION

  • CNS

    • Hydrocephalus

    • Microcephaly

    • Agenesis of corpus callosum

    • Encephalocele (Meckel-Gruber syndrome)

  • GASTROINTESTINAL

    • Esophageal atresia

    • Duodenal atresia

    • Situs abnormalities

    • Diaphragmatic hernia

  • VENTRAL WALL DEFECT

    • Omphalocele

    • Ectopia cordis

  • RENAL

    • Bilateral renal agenesis

    • Dysplastic kidneys

  • TWINS

    • Conjoined twins

Prenatal Risk Factors for Congenital Heart Disease

• FETAL RISK FACTORS

  • Symmetric IUGR

  • Arrhythmias

    • fixed fetal bradycardia (50%) 110 bpm

    • tachycardia (low risk)

    • irregular: PACs, PVCs (low risk)

  • Abnormal fetal karyotype

    • (CHD in Down syndrome in 40%; in trisomy 18 / 13 in >90%; in Turner syndrome in 35%)

  • Extracardiac somatic anomalies by US

    • omphaloceles (20%), duodenal atresia, hydrocephaly, spina bifida, VACTERL

  • Nonimmune hydrops (30 35%)

  • Oligo- / polyhydramnios

P.1005




• MATERNAL RISK FACTORS

  • Maternal heart disease (10%)

  • Insulin-dependent diabetes mellitus (4 5%)

  • Phenylketonuria (in 15% if maternal phenylalanine >15%)

  • Collagen vascular disease: SLE

  • Viral infection: rubella

  • Drugs

    • phenytoin (in 2% PS, AS, coarctation, PDA)

    • trimethadione (in 20% transposition, tetralogy, hypoplastic left heart)

    • sex hormones (in 3%)

    • lithium (7%): Ebstein anomaly, tricuspid atresia

    • alcohol (25% of fetal alcohol syndrome): VSD, ASD

    • retinoic acid = isotretinoin (?15%)

  • Paternal CHD (risk uncertain)

• MENDELIAN SYNDROMES

  • Tuberous sclerosis

  • Ellis-van Creveld syndrome

  • Noonan syndrome

• FAMILIAL RISK FACTORS FOR RECURRENCE OF HEART DISEASE

  overall incidence	: 6 8:1,000 live births
  affected sibling	: 1 4% (risk doubled)
  affected parent	: 2.5 4%

• In 50% of neonates with CHD there is no identifiable risk factor!

• Poor prognostic features:

  • Intrauterine cardiac failure (hydrops)

  • Severe trisomy (18, 13)

  • Hypoplastic left heart + endocardial fibroelastosis

  • Delivery in center without pediatric cardiology

In Utero Detection of Cardiac Anomalies

• ABNORMAL HEART POSITION

  • Diaphragmatic hernia

  • Lung anomaly

  • Pleural effusion

  • Cardiac defect

• CHAMBER ENLARGEMENT

  RA:	LA:
  1. Tricuspid regurgitation	1. Mitral stenosis
  2. Tricuspid valve dysplasia	2. Aortic stenosis
  3. Ebstein anomaly
  RV:	LV:
  1. Coarctation	1. Aortic stenosis
  2. Normal in 3rd trimester	2. Cardiomyopathy

• ABNORMAL FOUR-CHAMBER VIEW

  • Septal rhabdomyoma

  • Endocardial cushion defect

  • Ventricular septal defect

  • Ebstein anomaly

  • Single ventricle

• VENTRICULAR DISPROPORTION

  • Hypoplastic right / left ventricle

  • Hypoplastic aortic arch

  • Aortic / subaortic stenosis

  • Coarctation of aorta

  • Ostium primum defect

• INCREASED AORTIC ROOT DIMENSION

  • Tetralogy of Fallot

  • Truncus arteriosus

  • Hypoplastic left ventricle with transposition

• DECREASED AORTIC ROOT DIMENSION

  • Coarctation of aorta

  • Hypoplastic left ventricle

• 26 80% of serious cardiac anomalies can be detected on four-chamber view!

• Increased sensitivity >20 weeks + by including outflow views!

Structural Cardiac Abnormalities & Fetal Hydrops

• Atrioventricular septal defect + complete heart block

• Hypoplastic left heart

• Critical aortic stenosis

• Cardiac tumor

• Ectopia cordis

• Dilated cardiomyopathy

• Ebstein anomaly

• Pulmonary atresia

Fetal Echocardiographic Views

• FOUR-CHAMBER VIEW

  • Position of heart within thorax

  • Number of cardiac chambers

  • Ventricular proportion

  • Integrity of atrial + ventricular septa

  • Position + size + excursion of AV valves

• PARASTERNAL LONG-AXIS VIEW

  • = LEFT VENTRICULAR OUTFLOW TRACT VIEW

  • Continuity between ventricular septum + anterior aortic wall

  • Caliber of aortic outflow tract

  • Excursion of aortic valve leaflets

• SHORT-AXIS VIEW OF OUTFLOW TRACTS

  • Spatial relationship between aorta + pulmonary artery

  • Caliber of aortic + pulmonary outflow tracts

• AORTIC ARCH VIEW

• Identification of fetal RV:

  • RV lies closest to anterior chest wall

  • foramen ovale flap seen within LA

  • prominent moderator band + papillary muscles in RV

Echogenic Intracardiac Focus

Cause:

mineralization of a papillary muscle

• Isolated anomaly in 90%!

  

  Fetal Short-Axis View

  

  Fetal Four-Chamber View

  P.1006

  
  

  • Trisomy 21 (in 30% of affected fetuses)

  • Trisomy 13 (in 50% of affected fetuses)

  • Normal pregnancy (4% in a population at high risk for fetal anomalies)

Fetal gastrointestinal anomalies

• Esophageal atresia TE fistula

• Duodenal atresia

• Meconium peritonitis

• Hirschsprung disease

• Choledochal cyst

• Mesenteric cyst

Fetal Abdominal Wall Defect

Prevalence:

1:2,000 pregnancies

• 1. Gastroschisis

• 2. Omphalocele spectrum:

  • upper abdominal wall defect

    • 3. Ectopia cordis

    • 4. Pentalogy of Cantrell

  • midabdominal wall defect: classic omphalocele

  • lower abdominal wall defect

    • 5. Bladder exstrophy

    • 6. Cloacal exstrophy

• 7.Amniotic band syndrome

• 8. Limb-body wall complex

Fetal Hepatomegaly

• CONGENITAL INFECTIONS

  • CMV

• SEVERE HEMOLYTIC DISEASE

• SYNDROMES

  • Beckwith-Wiedemann syndrome

  • Zellweger syndrome

Intraabdominal Echogenic Mass in Fetus

• Abdomen

  • Echogenic bowel

  • Enteric duplication cyst (rarely echogenic)

  • Subdiaphragmatic extralobar pulmonary sequestration (4:1 left-sided predominance)

• Liver

  • Hepatic hemangioma

  • Hepatic mesenchymal hamartoma composed of multiple microcysts

• Adrenal / renal

  • Neuroblastoma

  • Adrenal hemorrhage

  • Mesoblastic nephroma

Nonvisualization of Fetal Stomach

• Fetal swallowing begins at 11 weeks MA

Normal:	stomach is visualized in almost all normal fetuses by 13 14 weeks (definitely by 19 weeks)
Incidence:	2%

• Cause:

  • Physiologic gastric emptying / intermittent swallowing

    • Repeat scan after 30 minutes!

  • Oligohydramnios

  • CNS depression / abnormalities impairing swallowing

  • Abnormal position of stomach:

    • stomach on contralateral side (situs inversus)

    • congenital diaphragmatic hernia

  • Esophageal atresia TE fistula

    • Nonvisualization of fetal stomach and polyhydramnios in 33% fetuses with esophageal atresia after 24 weeks MA!

  • Cleft lip / palate (impairing normal swallowing)

Rx:

repeat ultrasound scan

Double Bubble Sign

• = fluid-filled stomach + proximal duodenum

• A persistently fluid-filled duodenum is always abnormal!

• DUODENAL OBSTRUCTION

  • Duodenal atresia (usually not seen <24 weeks MA)

    Cause:

    in 30% due to Down syndrome

  • Severe duodenal stenosis

  • Duodenal web

  • Duodenal duplication cyst

• PERIDUODENAL ANOMALY

  • Ladd bands

  • Annular pancreas

  • Preduodenal portal vein

• DISTAL OBSTRUCTION

  • Midgut volvulus

  • Malrotation

mnemonic:

LADS

• Ladd bands / malrotation

• Annular pancreas

• Duodenal atresia

• Stenosis (duodenal)

Dilated Bowel in Fetus

• Meconium ileus

  • All newborns with meconium ileus have cystic fibrosis!

  • 10 15% of newborns with cystic fibrosis present with meconium ileus!

• Apple peel atresia of small bowel

• Jejunal atresia

• Megacystis-microcolon-intestinal hypoperistalsis syndrome

• Colonic aganglionosis = Hirschsprung disease

  • (may be associated with Down syndrome)

• Anorectal atresia (associated with CNS abnormalities, part of VACTERL complex)

Bowel Obstruction in Fetus

Etiology:	intestinal atresia / stenosis secondary to vascular accident, volvulus, meconium ileus, intussusception after organogenesis
Incidence:	imperforate anus 1:3,000;
	small bowel 1:5,000; colon 1:20,000

• Pathologic types:

  • one / more transverse diaphragms

  • blind-ending loops connected by fibrous string

  • complete separation of blind-ending loops

  • apple-peel atresia of small bowel (occlusion of SMA branch)

    Associated with:

    GI anomalies in 45% (malrotation, duplication, microcolon, esophageal atresia)

P.1007




• multiple distended bowel loops >7 mm in diameter

• increased peristalsis

• polyhydramnios (if obstruction above level of mid jejunum; exceptions are esophageal atresia + TE fistula) due to fetal inability to cycle amniotic fluid through gut

Cx:	Meconium peritonitis (50%)
DDx:	(1) Other cystic masses: duodenal atresia, hydronephrosis, ovarian cyst, mesenteric cyst
	(2) Chronic chloride diarrhea

Hyperechoic Fetal Bowel

• Most common echogenic mass in fetal abdomen

Definition:	bowel echogenicity bone
Incidence:	0.2 1.0% of 2nd trimester fetuses
Cause:	(?) constipation in utero due to decreased swallowing, hypoperistalsis, bowel obstruction + increased fluid absorption, ingestion of blood

• Normal small bowel variant (especially <20 weeks MA) with resolution on follow-up sonogram toward end of 2nd trimester (in 50 70%)

• Normal colon variant in 3rd trimester 2 to echogenic meconium

• Meconium ileus

  • Increased abdominal echogenicity is seen in 60 70% of fetuses with cystic fibrosis!

• Chromosomal abnormality (3 25%)

  • Down syndrome (5 14%)

  • Trisomy 13, 18

  • Turner syndrome

• Severe IUGR (16%)

• Intraamniotic bleeding

  • with subsequent swallowing of blood by fetus

• Prognosis:

  • 5-fold increase in risk for adverse fetal outcome (due to chromosomal abnormality, other anomalies, placental abruption, perinatal death [8 16%], IUGR [67 23%])

  • 30 50% of fetuses with echogenic bowel in 2nd trimester will have poor outcome!

Management:

parental testing for cystic fibrosis, careful fetal anatomic survey, follow-up for growth assessment

Intraabdominal Calcifications in Fetus

• Isolated liver calcifications are relatively frequent (1:1,750 pregnancies) and of no clinical significance!

Scattered Calcifications

• PERITONEAL

  • Meconium peritonitis

  • Plastic peritonitis associated with hydrometrocolpos

• CONGENITAL INFECTION

  • Toxoplasmosis

  • Cytomegalovirus

Focal Calcification

• TUMOR

  • Hepatoblastoma

  • Metastatic neuroblastoma

  • Hemangioma / hemangioendothelioma

  • Teratoma

  • Ovarian dermoid

• OTHERS

  • Fetal gallstones (>28 weeks EGA)

    Cause:	hemolytic disease, cholestasis, maternal drug use
    Prognosis:	resolution before / after delivery

Cystic Mass in Fetal Abdomen

• POSTERIOR MID ABDOMEN

  • Cysts of renal origin

  • Hydroureteronephrosis

  • Multicystic dysplastic kidney

  • Paranephric collection

• RIGHT UPPER QUADRANT

  • Liver cyst

  • Choledochal cyst

• LEFT UPPER QUADRANT

  • Splenic cyst

• ANTERIOR MID ABDOMEN

  • Gastrointestinal duplication cyst

  • Mesenteric cyst

  • Meconium pseudocyst

  • Dilated bowel

  • Urachal cyst

• LOWER ABDOMEN

  • Adnexal cyst: follicular cyst (most), corpus luteum cyst, theca lutein cyst, paraovarian cyst, teratoma, cystadenoma

    Cx of large cysts:	polyhydramnios, dystocia, torsion, respiratory distress
    Prognosis:	60% resolve within first 6 months of life

  • Hydrometrocolpos

  • Meningocele

  • Sacrococcygeal teratoma

Fetal Ascites

• ASCITES + FETAL HYDROPS

  • Immune hydrops

  • Nonimmune hydrops

• ISOLATED ASCITES

  • Urinary ascites

  • Meconium peritonitis

  • Bowel rupture

  • Ruptured ovarian cyst

  • Hydrometrocolpos

  • Glycogen storage disease

Fetal urinary tract anomalies

Incidence:	0.25% 1% liveborn infants (OB-US);
	1:100 1:200 neonates (pediatrics)

• Types:

  • Bilateral renal agenesis

  • Infantile polycystic kidney disease

  • Adult polycystic kidney disease

  • Multicystic dysplastic kidney

  • Ureteropelvic junction obstruction

  • Megaureter

  • Posterior urethral valves

  • Prune belly syndrome

  • Megacystis-microcolon-intestinal hypoperistalsis syndrome

  P.1008

  
  

  • Mesoblastic nephroma

  • Wilms tumor

  • Neuroblastoma

    Associated with:	chromosome abnormalities in 12% (74% trisomy, 10% deletion, 9% sex chromosome aneuploidy, 6% triploidy)
    fetal urine production:	5 mL/hr at 20 weeks MA;
    	56 mL/hr at 40 weeks MA
    bladder volume:	1 mL at 20 weeks MA;
    	36 mL at 40 weeks MA

• filling + emptying of fetal urinary bladder occurs every 10 30 (range 7 43) minutes

• increased renal parenchymal echogenicity indicates renal abnormality in 80%

• fetal hydronephrosis

  • = AP diameter of renal pelvis >5 mm at 15 20 weeks, 8 mm at 20 30 weeks, 10 mm at >30 weeks

General gynecology

Pelvic Features of Estrogen Stimulation

• increased thickness + volume of uterus

• fundocervical ratio >2

• echogenic endometrium

• appearance of ovaries NOT USEFUL (because of widely varying ovarian volumes + normal visualization of follicles at all ages)

Precocious Puberty

• = complete sexual development with secondary sex characteristics appearing <8 years of age in girls / <9 years of age in boys

• premature thelarche / adrenarche / menarche

• Terminology:

  • isosexual = secondary sex characteristics appropriate for patient's gender

  • heterosexual = secondary sex characteristics inappropriate for patient's gender

    • virilization in girls

    • feminization in boys

  • gonadotropin-dependent = true precocious puberty

  • gonadotropin-independent = pseudoprecocious puberty

Isolated Premature Adrenarche

• = pubic hair development due to action of adrenal androgens

• increased levels of adrenal androgens

• prepubertal uterus + ovaries (0.1 1 cm³)

Isolated Premature Thelarche

• = breast enlargement

• May occur without endocrine abnormalities

• prepubertal uterus + ovaries

Pseudoprecocious Puberty

• = pseudosexual PRECOCITY = PERIPHERAL PRECOCIOUS PUBERTY= incomplete precocious puberty

• = pubertal changes occurring independently of the action of pituitary gonadotropins, ie, early development of secondary sex characteristics without ovulation

• Cause:

  • Autonomous ovarian follicular cyst (most common cause)

  • Estrogen-secreting ovarian tumor:

    • eg, granulosa theca-cell tumor, gonadoblastoma, thecoma, choriocarcinoma

  • McCune-Albright syndrome

  • Adrenocortical neoplasm

  • Hypothyroidism

  • Neurofibromatosis

  • Hepatoblastoma

  • Estrogen ingestion

• low gonadotropin levels after LHRH stimulation

• high estradiol level

• low levels of FSH and LH

• normal bone age

• prepubertal uterus + ovaries

• asymmetric ovarian enlargement (one ovary 2.4 7 cm³) with macrocysts (>9 mm)

• unilateral follicular ovarian cyst characterized by internal daughter cyst

True Precocious Puberty

• = CENTRAL PRECOCIOUS PUBERTY = TRUE ISOSEXUAL PRECOCITY = complete precocious puberty

• = gonadotropin-dependent early development of gonads + secondary sex characteristics with ovulation before 8 years of age

• Cause:

  • Idiopathic activation of hypothalamic-pituitary-gonadal axis (66 80%)

  • Lesion of pituitary gland / hypothalamus:

    • eg, tuber cinereum hamartoma

  • Increased intracranial pressure:

    • eg, postmeningitis hydrocephalus

• increased levels of estrogen

• increased gonadotropin levels after LHRH stimulation

• advanced bone age

• adult-sized ovaries (1.2 12 cm³)

• dominance of corpus over cervix length

Rx:

long-acting gonadotropin-releasing hormone analogue

Amenorrhea

Primary Amenorrhea

• Definition:

  • no menarche by 16 years of age

  • no thelarche / adrenarche by 14 years of age

  • no menarche >3 years after adrenarche + thelarche

• Cause:

  • FEMALE ANATOMIC ANOMALIES

    • = M llerian (uterovaginal) anomalies (20%)

  • CONGENITAL DISORDERS OF SEXUAL DIFFERENTIATION

    • pure gonadal dysgenesis = Turner syndrome (33%)

      • bilateral dysfunctional / streak gonads

    • mixed gonadal dysgenesis

      • testis + streak gonad

    P.1009

    
    

    Risk:

    in 25% development of dysgerminoma / gonadoblastoma in dysgenetic gonads with Y chromosome

  • OVARIAN FAILURE / DYSFUNCTION

  • HYPOTHALAMIC / PITUITARY CAUSES (15%)

  • CONSTITUTIONAL DELAY (10%)

  • OTHERS: eg, systemic, psychiatric illness (22%)

• absent / streak gonads + infantile uterus:

  • Hypogonadotrophic hypogonadism

    • low / normal LH + FSH levels

      • hypothalamic dysfunction: hypothalamic tumor, Kallmann disease (= lack of pulsatile GnRH release), systemic illness, constitutional growth delay, extreme physical / psychological / nutritional stress (cystic fibrosis, sickle cell disease, Crohn disease), irradiation

      • pituitary dysfunction: disruption of pituitary stalk from child abuse, head trauma

  • Hypergonadotropic hypogonadism

    • = ovarian tissue fails to respond to endogenous gonadotropins

    • high LH + FSH levels

    • abnormal karyotype: Turner syndrome, XY gonadal dysgenesis

    • irradiation, chemotherapy, autoimmune disease (eg, autoimmune oophoritis)

• absent uterus:

  • Testicular feminization = male intersex = male pseudohermaphroditism (end-organ insensitivity to testosterone)

  • M llerian dysgenesis (= Mayer-Rokitansky-K ster-Hauser syndrome)

    • normal fallopian tubes + ovaries

    associated with:

    unilateral renal abnormality (50%), skeletal abnormality (12%)

• small infantile uterus:

  • Androgen-producing virilizing tumors of adolescent ovary (usually Sertoli-Leydig cell tumor)

    • unilateral adnexal mass

  • Turner syndrome

  • In utero exposure to diethylstilbestrol

• normal uterus + unilateral ovarian tumor:

  • Estrogen-producing ovarian tumor with disruption of menstrual cycle:

    • granulosa cell tumor, thecoma

• hematometrocolpos:

  • neonate = congenital uterovaginal obstruction

    • Urogenital sinus / cloacal malformation

      • pelviabdominal cystic mass with fluid-debris level in fetal US during 3rd trimester

      • renal dysplasia / obstruction

  • teenager

    • Imperforate hymen

    • Transverse vaginal septum

      • in upper vagina (45%)

      • in mid vagina (40%)

      • in lower vagina (15%)

• hematometra

  • Cervical dysgenesis

• bilateral ovarian enlargement:

  • Polycystic ovary syndrome

    • (= Stein-Leventhal syndrome): most common cause of secondary amenorrhea

Secondary Amenorrhea

• Pregnancy: most common cause in girls >9 years of age

• Polycystic ovary syndrome (main pathologic cause)

• Asherman syndrome

• All causes of primary amenorrhea

Calcifications of Female Genital Tract

• UTERUS

  • Uterine fibroid

  • Arcuate arteries

• OVARIES

  • Dermoid cyst (50%)

  • Papillary cystadenoma (psammomatous bodies)

  • Cystadenocarcinoma

  • Hemangiopericytoma

  • Gonadoblastoma

  • Chronic ovarian torsion

  • Pseudomyxoma peritonei

• FALLOPIAN TUBES

  • Tuberculous salpingitis

• PLACENTA

• LITHOPEDION

Psammoma Bodies in Tumors

• Papillary serous cystadenoma / cystadenocarcinoma

• Mucinous carcinoma of colon

• Papillary thyroid cancer

• Meningioma

Free Fluid in Cul-de-sac

• Follicular rupture

• Ovulation

• Ectopic pregnancy

• S/P culdocentesis

• Ovarian neoplasm

• Pelvic inflammatory disease

Pelvic mass

Frequency of Pelvic Masses

1. Benign adnexal cyst	34%
2. Leiomyoma	14%
3. Cancers	14%
4. Dermoid	13%
5. Endometriosis	10%
6. Pelvic inflammatory disease	8%

Cystic Pelvic Masses

• CYSTIC ADNEXAL MASS

• EXTRAADNEXAL CYSTIC MASS

  • Peritoneal inclusion cyst

  • Mesenteric cyst

  • Lymphocele

  • Bladder diverticulum

  P.1010

  
  

  • Ectopic gestation

  • Fluid-distended bowel

  • Loculated pelvic abscess: appendiceal, diverticular, postoperative, Crohn disease, tuberculous, pelvic actinomycosis

Complex Pelvic Mass

mnemonic:

CHEETAH

• Cystadenoma / cystadenocarcinoma

• Hemorrhagic cyst

• Endometrioma

• Ectopic pregnancy

• Teratoma (dermoid)

• Abscess (from adjacent appendicitis, etc.)

• Hematoma in pelvis

Solid Pelvic Masses

• Pedunculated myoma (most common)

• Fibroma

• Adenofibroma

• Thecoma

• Brenner tumor

Fatty Pelvic Mass

• UTERUS

  • Lipoleiomyoma

  • Fibromyolipoma

• OVARY

  • Benign cystic ovarian teratoma

  • Malignant degeneration of cystic teratoma

  • Nonteratomatous lipomatous ovarian tumor

• PELVIS

  • Benign pelvic lipoma

  • Liposarcoma

  • Lipoblastic lymphadenopathy

Extrauterine Pelvic Masses

• Solid adnexal mass

• Metastatic disease

• Lymphoma

• Pelvic kidney

• Rectosigmoid carcinoma

• Bladder carcinoma

• Retroperitoneal tumor / fibrosis

• Intraperitoneal fat

• Vascular mass / malformation

• Hematoma

• Bowel

Pelvic Pain in Pediatric Age Group

• Ovarian torsion

  • of normal ovary

    Cause:

    excessive mobility of ovary in childhood

  • with ovarian mass:

    • functional cyst (60%)

    • neoplasm (40%):

      • benign mature teratoma (66%)

      • malignancy (33%): germ cell tumor (60 75%), epithelial tumor (10 20%), stromal tumor (10%)

• Hemorrhagic ovarian cyst

• Pelvic inflammatory disease

• Ectopic pregnancy

Adnexa

Adnexal Masses

• CYSTIC

  • Physiologic ovarian cyst:

    Graafian follicle:	at midcycle <25 mm
    Corpus luteum:	after midcycle <15 mm

  • Functional / retention cyst

  • Endometrioma

  • Tuboovarian abscess

  • Dermoid cyst

  • Ectopic pregnancy

  • Paraovarian cyst / cystadenoma

  • Serous / mucinous ovarian tumor

  • Hyperstimulation cysts

  • Peritoneal inclusion cyst

  • Massive ovarian edema

  • Hydrosalpinx

• SOLID

  • Ovarian tumor

  • Ovarian torsion

  • Oophoritis

  • Polycystic ovaries

  • Fallopian tube carcinoma

  • Pedunculated fibroid

Hemorrhagic Adnexal Lesion

• Endometriosis

• Hemorrhagic ovarian cyst

• Hemorrhagic foci of adenomyosis

• Hematosalpinx

Low-intensity Adnexal Lesion on T1WI

• Fibroma

• Fibrothecoma

• Cystadenofibroma

• Brenner tumor

• Wall of chronic pelvic abscess

• Pedunculated leiomyoma

High-intensity Adnexal Lesion on T1WI

• Endometrioma

  • frequently multilocular + bilateral

  • shading (= signal loss) on T2WI

• Dermoid

  • chemical shift artifact

  • signal drop-out after fat suppression

• Mucinous cystic neoplasm

  • signal intensity less than fat / blood

• Hemorrhagic cyst

  • unilocular

  • no shading

  • resolution with time

• Ovarian carcinoma

  • solid components, septations

  • large size

Adnexal Mass in Pregnancy

Incidence:

0.5 1.2%

P.1011

• RESOLVING BY 14 16 WEEKS EGA

  • Corpus luteum cyst

  • Theca lutein cyst

• PERSISTENT ADNEXAL MASS

  • Benign

    • correctly diagnosed by US: 95% of dermoids, 80% of endometriomas, 71% of simple cysts

  • Malignant (0.1 0.8%)

Ovarian Tumors

• pressure symptoms: abdominal discomfort, vomiting, flatulence, dyspnea

• acute pain from torsion, hemorrhage

• chronic pain from slowly enlarging mass, impaction, adhesions

• menstrual irregularity

• Radiologic guidelines:

  • Imaging features of ovarian neoplasms virtually never allow a specific diagnosis. Regardless of further differentiation patients always undergo surgery!

  • Signs suggestive of benignancy:

    • unilocularity of cyst

    • thin wall <3 mm

    • minimal septations

    • absence of papillary projection

  • Signs suggestive of malignancy:

    • solid nonfatty nonfibrous tissue (most powerful predictor of malignancy!)

    • many solid-tissue elements in a complex lesion

    • wall thickness >3 mm

    • inner wall irregularities / papillary projections

    • thick septations >3 mm

    • increased echogenicity within a cyst

      Age:	13% of neoplasms malignant in premenopause; 45% of neoplasms malignant in postmenopause
      Cx:	(1) Torsion (in 10 20%)
      	(2)Rupture (rare)
      (3) Infection

• Classification:

  • 75% of ovarian neoplasms are benign

  • 21% of ovarian neoplasms are malignant

  • 4% of ovarian neoplasms are borderline malignant

Tumors of Surface Epithelium (60 70%)

• = 85 95% of all ovarian cancers

• propensity for early peritoneal + lymphatic spread

• peritoneal studding

• omental cake

• perihepatic diaphragmatic implants

  Subclassification of Ovarian Tumors

  	Benign	Borderline	Malignant
  [all types combined	75%	4%	21%]
  Serous	60%	15%	25%
  Mucinous	80%	10%	10%
  Endometrioid	~0%	~0%	~100%
  Clear cell	~0%	~0%	~100%
  Undifferentiated	0%	0%	100%

  • Serous ovarian tumor (50%)

  • Endometrioid tumor (15 30%)

  • Mucinous ovarian tumor (15%)

  • Clear cell adenocarcinoma (5%)

  • Undifferentiated carcinoma (<5%)

  • Brenner tumor (2.5%)

  • Cystadenofibroma

Germ Cell Tumors (15 30%)

• 40% of germ cell tumors are malignant

• benign (10%)

  • Dermoid cyst = mature teratoma (most common)

• malignant

  • account for 75% of ovarian cancers seen in 1st 2nd decade of life; <5% of all ovarian tumors; in order of frequency:

    • Dysgerminoma (1.9%)

    • Immature teratoma (1.3%)

    • Endodermal sinus tumor (1%)

    • Malignant mixed germ cell tumor (0.7%)

    • Embryonal carcinoma (0.1%)

    • Choriocarcinoma (0.1%)

Sex Cord-Stromal Tumors (5 8%)

• usually have more than one cell type + arise from two groups of cells:

• primitive sex cord cells, which form from the coelomic epithelium (= primordial peritoneum) differentiate into granulosa cells + Sertoli cells

• stromal cells (fibroblasts, theca cells, Leydig cells) derive from the genital ridge mesonephros mesenchyma

• broad range of ages

• most present at stage I with good prognosis

• absent tumor markers

• Often manifest with tumor-mediated hormonal effects

• NO papillary projections

• lack of fat + calcifications

  • hyperestrogenic tumors: granulosa cell tumor, thecoma, stromal luteoma

  • virilizing tumors: Sertoli-Leydig cell tumor, steroid cell tumor (Sertoli cell tumor, Leydig cell tumor)

• Granulosa-stromal cell tumors

  1. Juvenile granulosa cell tumor	multicystic
  2. Adult granulosa cell tumor	solid
  3. Thecoma	solid
  4. Fibroma	solid
  5. Fibrosarcoma
  6. Sclerosing stromal tumor

• Sertoli-stromal cell tumors

  1. Sertoli-Leydig tumor	solid
  2. Sertoli cell = arrhenoblastoma	solid
  3. Leydig cell

• Steroid cell tumors = lipid cell tumors

  • Stromal luteoma

  • Leydig cell tumor = hilus cell tumor

  • Steroid cell tumor, not otherwise specified

• Other

  • Gynandroblastoma

  • Sex cord tumor with annular tubules

    • associated with Peutz-Jeghers syndrome (30% of all tumors with annular tubules)

  P.1012

  
  

  • Sclerosing stromal tumor

• SECONDARY OVARIAN TUMORS (5%)

  • Metastases from: pelvic organs, upper GI tract, breast, bronchus, reticuloendothelial tumors, leukemia

• Terminology:

prefix cyst-	: cystic component present
suffix -fibroma	: >50% fibrous component
	tumor of low malignant potential : borderline malignant

Solid Ovarian Tumor

• Fibroma

• Thecoma

• Granulosa cell tumor

• Sertoli-Leydig cell tumor

• Brenner tumor

• Sarcoma

• Dysgerminoma

• Endodermal sinus tumor

• Teratoma

• Metastasis

• Endometrioma

• Massive ovarian edema

• Leiomyoma

Proximal Fallopian Tube Obstruction

• Extensive fibrosis / salpingitis isthmica nodosa (40%)

• Amorphous debris / minimal adhesions (40%)

• Tubal spasm (20%)

Uterus

Prepubertal Vaginal Bleeding

• Vaginal foreign body

  Incidence:

  in 18% of children with vaginal bleeding + discharge; in 50% of children with vaginal bleeding + no discharge

• Vaginal rhabdomyosarcoma

• Precocious puberty

• Hemangioma

• Vascular malformation

Postmenopausal Vaginal Bleeding

• Endometrial atrophy (in 60 75%)

  • thin atrophic endometrium is prone to superficial ulceration

  • in 75% endometrial thickness <4 5 mm

    • Patient may forego endometrial biopsy!

  • in 25% endometrial thickness of 6 15 mm

• Endometrial hyperplasia

• Endometrial polyp

• Submucosal fibroid

  • hypoechoic mass with an overlying normal echogenic endometrium

  • acoustic attenuation

  • prolapse into endometrial cavity

    Rx:

    can be removed at hysteroscopy if >50% of mass projects into endometrial cavity

• Adenomyoma

  • indistinguishable from submucosal fibroid

• Endometrial carcinoma (in 7 20%)

• Estrogen withdrawal

• Optimal time of imaging:

  • immediately after cessation of bleeding when endometrium is presumed to be thinnest

Rx:

any focal / generalized thickness >5 mm at transvaginal US requires further investigation (sonohysterography, guided biopsy, hysteroscopy)

Diffusely Thickened Irregular Endometrium

• Normal endometrial thickness: see Anatomy

• Sensitivity for detection of endometrial abnormalities: 80% for transvaginal US; 30% for endometrial biopsy

Time of sonohysterography:

day 4, 5, or 6 of menstrual cycle

• Endometrial hyperplasia

  Age:	peri- / postmenopausal women
  Cause:	prolonged endogenous / exogenous unopposed estrogen stimulation

  • focal / diffuse endometrial thickening >5 6 mm

  • formation of polyps of up to 5 cm

  • Types:

    • glandular-cystic hyperplasia (most common)

      Histo:

      dilated glands lined by tall columnar / cuboidal epithelium

      • small cysts within evenly echogenic endometrium

      Prognosis:

      NO premalignant condition

    • adenomatous hyperplasia

      • endometrium with irregular hypoechoic areas

      Prognosis:

      precursor of endometrial cancer

• Secretory endometrium

  • Improve timing of the examination!

• Endometrial cancer

• Endometritis

• Tamoxifen-related endometrial changes

  • = nonsteroidal antiestrogen in breast acts as a weak estrogen agonist causing proliferative effects on the endometrium

    Increased prevalence of:	endometrial hyperplasia, polyps, carcinoma
    	50% of women treated with tamoxifen will develop endometrial abnormalities within 6 36 months

  • Histo:

    • endometrial thickness increased to 10.4 mm; 4.2 mm in control subjects

    • polyps (36%); 10% in control subjects

    • atrophic changes (28%); 87% in control group

  • hemorrhage (requires further evaluation)

  • endometrial thickening >5 9 mm:

    • endometrial hyperplasia

    • endometrial polyp

    • subendometrial cystic changes (= glandular distension within a polyp / reactivated adenomyosis within inner myometrium)

  • MR:

    • endometrium-myometrium interface (due to endometrial atrophy / proliferative changes):

      • homogeneously hyperintense on T2WI

      • signal void + enhancement on T1WI

    • polyps:

      • heterogeneous intensity on T2WI

      P.1013

      
      

      • laticelike enhancement traversing endometrial canal on T1WI

Focally Thickened Endometrium

• Endometrial polyp

  • = focal hyperplasia of stratum basale; in 20% multiple

    Age:	mainly 30 60 years
    Histo:	projections of endometrial glands + stroma into uterine cavity

    • hyperplastic polyp resembling endometrial hyperplasia

    • functional polyp resembling surrounding endometrium (least frequent)

    • atrophic polyp

    Frequently associated with:

    tamoxifen therapy

  • US:

    • sessile broad-based / pedunculated well-defined smooth hyperechoic homogeneous intracavitary mass (79%) / variable echogenicity (best seen on sonohysterography):

      • cystic spaces (in 59%) due to enlarged dilated glands filled with proteinaceous fluid

      • heterogeneous texture suggests infarction, inflammation, hemorrhage

      • vessel visualized within stalk on color Doppler

        Malignant transformation:

        in 0.4 3.7%

• Primary carcinoma of the endometrium

  Risk factors:	exposure to unopposed estrogen, obesity, nulliparity, hypertension, diabetes
  Location:	predominantly in uterine fundus; 24% in isthmic portion)

  • 10% cancer rate with endometrial thickness of 6 15 mm

  • 50% cancer rate with endometrial thickness of >15 mm

  • irregular heterogeneous endometrium >5 mm in thickness

  • focal / diffuse endometrial thickening (mean thickness of 18.2 mm)

  • irregular poorly defined endometrial-myometrial interface

  • increased echogenicity in myometrium (= invasive endometrial cancer)

  • Doppler waveforms with resistive index <0.7 suggest malignancy

• Metastatic carcinoma:

  • ovary, cervix, fallopian tube, leukemia

• Hydatidiform mole

  • echogenic mass with irregular sonolucent areas

• Incomplete abortion

• Submucosal leiomyoma

  • mass hypo- / hyperechoic relative to myometrium

  • wide attachment to myometrium

  • intracavitary margin outlined by echogenic rim of endometrium

  • acoustic attenuation

    Rx:

    hysteroscopic removal if >50% of mass projects into endometrial cavity

• Focal adenomyoma

  • hypoechoic mass with an overlying echogenic endometrium

    DDx:

    indistinguishable from submucosal fibroid

• Intrauterine synechiae

  • echogenic bands extending from one endometrial surface to the other

DDx:

adherent blood clots

Fluid Collection within Endometrial Canal

Types:

blood, mucus, purulent material

• PREMENOPAUSAL

  • Infection: endometritis, pyometrium

  • Congenital obstructive lesion: imperforate hymen, vaginal septum, vaginal / cervical atresia

  • Acquired obstructive lesion: cervical stenosis (following instrumentation / cone biopsy / radiation), cervical carcinoma

  • Spontaneous hematometra in bleeding disorders

  • Pregnancy: intrauterine, ectopic, incomplete abortion

  • Endometrial cancer

  • Endometrial polyp, submucosal fibroid

  • Functional: during menstruation

• POSTMENOPAUSAL

  • Endometrial / cervical cancer

  • Cervical stenosis

  • Normal if amount small

Endometrial Cysts

• Endometrial cystic atrophy

  Histo:

  cystically dilated atrophic glands lined by single layer of flattened / low cuboidal epithelium

  • very thin endometrium of <4 5 mm

• Endometrial cystic hyperplasia

Diffuse Uterine Enlargement

• Diffuse leiomyomatosis

• Adenomyosis

• Endometrial carcinoma (15%)

Uterine Masses

• BENIGN

  • Uterine fibroids (99%)

  • Pyometra

  • Hemato- / hydrocolpos

  • Transient uterine contraction (during pregnancy)

  • Bicornuate uterus

  • Adenomyosis

  • Intrauterine pregnancy

  • Lipoleiomyoma (<50 cases in world literature)

• MALIGNANT

  • Cervical carcinoma

  • Endometrial carcinoma

  • Leiomyosarcoma

  • Invasive trophoblastic disease

Cervical Mass

• Fibroid

• Carcinoma

• Endometrial polyp

• Nabothian cyst (= retention cyst related to chronic cervicitis)

P.1014

Fundic Depression on HSG

• with normal outer contour

  • Septate uterus

  • Arcuate uterus

• with abnormal outer contour

  • Bicornuate uterus

  • Fundal myoma

Filling Defect on HSG

• Synechia

• Endometrial polyp

• Submucosal myoma

• Uterine folds = parallel to long axis of uterus

• Air bubbles

Abnormal Uterine Contour on HSG

• Submucosal myoma

• Adenomyosis

• Trauma (cesarian section, myomectomy)

Cervical Stenosis

• = obliteration + obstruction of endocervical canal

  • CONGENITAL

    • Transverse vaginal septum in uterus didelphys

    • Congenital absence of cervix

  • ACQUIRED

    • Cervical carcinoma (postmenopausal)

    • Endometrial cancer (premenopausal)

    • S/P radiation treatment

    • S/P endocervical curettage

• distension of endometrial cavity by secretions + blood products;

Cx:

reflux endometriosis

Postpartum Hemorrhage

• Uterine atony

  • hemorrhage in immediate postpartum period

  • normal uterus

• Retained products of conception

  • hemorrhage several days after delivery

  • echogenic intracavitary mass attached to endometrium

  • high-velocity flow >21 cm/sec of low resistance (supplying residual trophoblastic tissue)

  • calcification of retained products (late finding)

Vagina

Vaginal Cyst

• Gartner duct cyst

• Bartholin gland cyst

  • = female homologue of male Cowper glands

    Location:

    posterolateral portion of lower vagina

• Paramesonephric / m llerian duct cyst

  • = aberrant remnant of paramesonephric duct

    Location:

    anterior wall of vagina near cervix

• Epithelial inclusion cyst

  • = arise from urogenital sinus

    Histo:

    lined by transitional epithelium containing thick caseous material

Vaginal Fistula

• Enterovaginal fistula:

  • rectovaginal: incomplete healing of perineal laceration from obstetric trauma, radiation therapy

  • anovaginal: inflammatory bowel disease (10% of patients with Crohn disease)

  • colovaginal: diverticulitis

• Vesicovaginal fistula:

  • hysterectomy, radiation therapy

• Ureterovaginal fistula: vaginal hysterectomy

Vaginal & Paravaginal Neoplasm

• PRIMARY

  • Cavernous hemangioma of vulva

  • Pedunculated submucosal leiomyoma prolapsed into vagina

  • Adenoid cystic carcinoma of Bartholin gland

  • Vaginal carcinoma

    • squamous cell carcinoma (90%)

    • adenocarcinoma (3%)

  • Rhabdomyosarcoma

• SECONDARY (80% of all vaginal tumors)

  • direct extension from bladder, rectum, cervix, uterus

Gas in genital tract

• UTERUS

  • Endometritis

  • Superinfection of leiomyoma: more common in submucosal leiomyoma (insufficient blood supply)

  • Bacterial metabolism of necrotic neoplastic tissue

  • Fistula to GI tract: uterine cancer

  • Pyometra secondary to obstruction by cervical cancer

  • Gas gangrene: due to clostridial infection from septic abortion

• OVARY

  • Superinfected ovarian neoplasm

• VAGINA

  • Vaginitis emphysematosa

    • = nonbacterial self-limiting process mostly occurring during pregnancy characterized by numerous gas-filled spaces in submucosa of vagina + exocervix

P.1015

Anatomy and Physiology of Female Reproductive System

Human chorionic gonadotropin

= hCG = glycoprotein elaborated by placental trophoblastic cells beginning the 8th day after conception

Immunologic Pregnancy Test

• = indirect agglutination test for hCG in urine; cross-reaction with other hormones / medications possible

• Becomes positive at 5 weeks MA

Advantages:	readily available, easily + rapidly performed
Disadvantages:	frequently false-positive + false-negative results
Sensitivity: slide: 400 15,000 mIU/mL (2-minute test time) test tube: 1,000 3,000 mIU/mL (2-hour test time)

Radioimmunoassay (RIA) Pregnancy Test

• = measures beta subunit of hCG in serum with a sensitivity as low as 1 2 mIU/mL

• Serum -hCG becomes positive at 3 weeks MA / 7 10 days following conception!

• Standards:

  • (1) Second International Standard (SIS)

  • (2) International Reference Preparation (IRP)

  • (3) Third International Standard (TIS)

  • 1 mIU/mL (SIS) = 2 mIU/mL (IRP) = 2 mIU/mL (TIS)

  • 1 ng/mL = 5 6 mIU/mL (SIS)

    = 10 12 mIU/mL (IRP or TIS)

    Variations of lab values of up to 50% can occur among different laboratories!

  • 6 15% between-run precision!

Advantages:	specific for hCG, sensitive
Disadvantages:	requires specialized lab + 3 24 hours for completion
Sensitivity: qualitative: 25 30 mIU/mL (3 hours test time) quantitative: 3 4 mIU/mL (24 hours test time)
Rise: >66% increase of initial -hCG level over 48 hours in 86% of NORMAL pregnancies <66% increase of initial -hCG level over 48 hours in 87% of ECTOPIC pregnancies -hCG levels double every 2 3 days during first 60 days of pregnancy!

Anatomy of gestation

Choriodecidua

Chorion

=trophoblast + fetal mesenchyme with villous stems protruding into decidua; provides nutrition for developing embryo

• chorion frondosum = part adjacent to decidua basalis, forms primordial placenta

• chorion laeve = smooth portion of chorion with atrophied villi

• chorionic plate = amnionic membrane covering the chorionic plate of the placenta

1 7 11 rule :

-hCG (IRP)	US Landmarks	Gestational Age
1,000 mIU/mL	gestational sac	32 d (4.5 weeks)
7,200 mIU/mL	yolk sac	36 d (5.0 weeks)
10,800 mIU/mL	embryo + heart motion	40 d (6.0 weeks)

Decidua

• decidua basalis = between chorion frondosum + myometrium

• decidua capsularis = portion protruding into uterine cavity

• decidua parietalis = decidua vera = portion lining the uterine cavity elsewhere

Gestational Sac (GS)

• Arises from blastocyst, which implants into secretory endometrium 6 9 days after ovulation (= 20 23 days of MA), surrounded by echogenic trophoblast

• GS measures 0.1 mm at time of implantation

• intradecidual sign (earliest sign) = intrauterine fluid collection corresponding to gestational sac completely embedded within decidua (48% sensitive, 66% specific, 45% accurate) at <5 weeks GA

• double decidual sac sign (DDS) [most useful >5 weeks GA] = 2 concentric hyperechoic rings surrounding a portion of the gestational sac:

  • outer echogenic ring (= decidua parietalis)

  • interposed hypoechoic line (apposed endometrial walls)

  • inner echogenic ring (= decidua capsularis)

  • DDS present with a mean sac diameter of 10 mm (= 40 days GA)

  • A double decidual sac sign correlates with the presence of pregnancy in 98%!

• GS surrounded by endometrial thickening >12 mm

• continuous hyperechoic inner rim >2 mm thick

• spherical / ovoid shape without angulations

• mean sac diameter grows 1.13 (range 0.71 1.75) mm/day

  

  Anatomy of Gestation

P.1016

Gestational Sac Size

• linear growth: 10 mm by 5th week MA

  60 mm by 12th week MA

• fills chorionic cavity by 11th 12th weeks MA

Visualization of Gestational Sac

• Earliest visualization: mean sac diameter of 2 3 mm

• A. GS VISUALIZATION VERSUS -hCG LEVEL (2^nd International Standard):

  • on transabdominal scan:

    • in 100% with -hCG levels of >1,800 IU/L

  • on transvaginal scan:

    in	20% with -hCG levels of	<500 IU/L
    in	80% with -hCG levels of	500 1,000 IU/L
    in	100% with -hCG levels of	>1,000 IU/L

• B. GS VISUALIZATION VERSUS MENSTRUAL AGE

  5.0 1 weeks	= 10 mm
  5.5 1 weeks	= 13 mm
  6.0 1 weeks	= 17 mm
  6.5 1 week	= 20 mm

Secondary Yolk Sac

• = rounded sonolucent structure (outside amniotic cavity) within chorionic sac (= extracoelomic cavity) connected to umbilicus via a narrow stalk; formed by proliferation of endodermal cells; part of yolk sac is incorporated into fetal gut; the rest persists as a sac connected to the fetus by the vitelline duct

• Function:

  • transfer of nutrients from trophoblast to embryo prior to functioning placental circulation

  • early formation of blood vessels + blood precursors on sac wall

  • formation of primitive gut

  • source of primordial germ cells

• Time of formation: at around 28 days MA

• Mean size:

  1.0 mm by 4.7 weeks MA; 2.0 mm by 5.6 weeks MA; 3.0 mm by 7.1 weeks MA; 4.0 (2.2 5.3) mm by 10 weeks MA; disappears around 12 weeks MA

  First visible structure within gestational sac

• Definite visualization on transvaginal scan:

  at 5.5 weeks MA

  

  No Caption Available.

  earliest detection at 5 weeks GA, embryo 2 mm in length

  Vitelline Duct

  8 weeks GA

  Early Coiled Umbilical Cord

  9 weeks GA

  in GS with a mean sac diameter of 8 mm

• Definite visualization on transabdominal scan:

  in GS with a mean diameter of 20 mm

  at a gestational age of 7 weeks MA

Embryo

• Developmental stages:

  Preembryonic period:	2nd 4th week MA
  Trilaminar embryonic disk:	during 5th week MA
  Embryonic period:	6th 10th week MA
  Physiologic umbilical herniation:	8th 12th week MA
  Fetal period:	beginning at 11th week MA

• Average growth rate:

  0.7 mm per day / 1.5 mm every 2 days;

  curvilinear growth from 7 mm at 6.3 weeks MA to 50 mm at 12.0 weeks MA

• Earliest visualization (on endovaginal scan):

  at 5.4 weeks MA at CRL of 1.2 mm

VISUALIZATION OF EMBRYO VERSUS GS

• on transabdominal scan

  100% visualization if gestational sac 27 mm

• on transvaginal scan

  100% visualization if gestational sac 12 mm

  Transvaginal scan not necessary if on transabdominal scan gestational sac >27 mm without evidence of embryo!

• Failed pregnancy:

  nonvisualization of embryo with mean gestational sac size of 18 mm

Cardiac Activity of Embryo

• Heart begins to contract at a CRL of 1.5 3 mm = 22 days GA = 36 days MA

  • Definite visualization on endovaginal scan:

    • at 46 days GA

    • mean sac diameter of 16 mm

    • CRL 5 mm CRL = 6.2 weeks

  • Definite visualization on transabdominal scan:

    • at 55 days GA

    • mean sac diameter of 25 mm

  • Rate:

    at 5-6 weeks GA	101 bpm
    at 8-9 weeks GA	143 bpm

P.1017

Amnionic Membrane

• = curvilinear echogenic line within chorionic sac; fills chorionic cavity by 11 12 weeks MA;

• Fusion:

  • fuses with chorionic membrane at approximately 16 weeks MA to form the chorionic plate

  • incomplete fusion with chorion frequent (DDx: subchorionic hemorrhage, twin abortion, coexistent with limb-body wall complex)

Umbilical Cord

• Embryology:

  • cord forms between 5th and 12th postmenstrual week with contributions from body stalk, omphalo-mesenteric or vitelline duct, yolk sac, allantois

  • junction of the amnion with ventral surface of embryo will form umbilicus

  • midgut undergoes physiologic herniation into the base of the umbilical cord 7 12 postmenstrual weeks

  • cord grows until end of 2nd trimester: average diameter of 17 mm, length of 50 60 cm

• Anatomy:

  • 1 2 cm in diameter

  • two umbilical arteries = branches of the two internal iliac arteries

  • one umbilical vein (remains after regression of right umbilical vein in early embryonic period)

  • Wharton jelly = compressible matrix of cord

  • covered by amnion

  • spiraling of cord with 0 40 twists established by 9 weeks

Placental Grading

• according to echo appearance of basal zone, chorionic plate, placental substance

  Premature placental calcifications are associated with cigarette smoking, hypertension, IUGR!

  Not considered useful because placental grading is imprecise for fetal dating or for fetal lung maturity!

• GRADE 0

  homogeneous placenta + straight line of chorionic plate

  Time:

  <30 weeks MA

• GRADE 1

  undulated chorionic plate + scattered bright placental echoes

  Time:

  seen at any time during pregnancy; in 40% at term in 68% L/S ratio >2.0

• GRADE 2

  linear bright echoes parallel to basal plate

  confluent stippled echoes within placenta indentations of chorionic plate

  Time:

  rarely seen in gestations <32 weeks MA; seen in 40% at term

  in 87% L/S ratio >2.0

• GRADE 3

  calcified intercotyledonary septa, often surrounding sonolucent center

  Time:

  rarely seen in gestations <34 weeks MA; in 15 20% at term

  in 100% L/S ratio >2.0 (= strongly correlated with lung maturity)

Premature Placental Senescence

= grade 3 placenta seen in gestation <34 weeks MA

In 50% suggestive of maternal hypertension / IUGR

Uteroplacental Circulation

By 20 weeks MA trophoblast invades maternal vessels and transforms spiral arteries into distended tortuous vessels = uteroplacental arteries

Histo:

• in the decidual portion of spiral arteries: proliferating trophoblast from anchoring villi invades lumen of spiral arteries + partially replaces endothelium

• in the myometrial portion of spiral arteries: disintegration of smooth muscle elements (loss of elastic lamina) leads to easily distensible vascular system of low resistance

Uterine Blood Volume Flow

• 50 mL/min shortly after conception

• 500 900 mL/min by term

  Intervillous blood flow:

  140 53 mL/min (by Xe-133 washout)

Umbilical Artery Doppler

Variables affecting Doppler measurements:

• site of Doppler (close to placenta preferred), fetal heart rate, fetal breathing, drugs (ritodrine hydrochloride decreases S/D ratio)

• degree of diastolic flow increases as gestation progresses

• S/D ratio between 3.3 and 4.3 at 20 weeks

• S/D ratio between 1.7 and 2.4 at term

• highly turbulent flow

IUGR Lesions

= narrowing of vascular lumen through

• thrombosis of decidual segments of uteroplacental arteries

• failure of development of myometrial segments of uteroplacental arteries

Fetal mensuration

US is more reliable than LMP / physical examination!

Ultrasound Milestones

gestational sac w/o embryo or yolk sac	= 5.0 weeks
gestational sac + yolk sac w/o embryo	= 5.5 weeks
heartbeat embryo <5 mm	= 6.0 weeks

Accuracy: 0.5 week

Fetal Age

• = GESTATIONAL AGE (GA) = MENSTRUAL AGE (MA)

• = age of pregnancy based on woman's regular last menstrual period (LMP) projecting the estimated date of confinement (EDC) at 40 weeks

  Note the inaccurate clinical usage of gestational age, which strictly speaking refers to the true age of the pregnancy counting from the day of conception, whereas menstrual age refers to the true age of the pregnancy + approximately 2 weeks counting from the first day of the last menstruation!

  P.1018

  
  

  On subsequent scans GA = GA assigned at 1st ultrasound + number of intervening weeks!

• ACCURACY OF CLINICAL ASSESSMENT

  menstrual history	2 3 weeks
  1st-trimester exam	2 weeks
  fundal height	4 weeks

Gestational Sac (GS)

• = average of 3 diameters (craniocaudad, AP, TRV) of anechoic space within sac walls

  Used for dating between 6 and 12 weeks MA (identified as early as 5 weeks MA on transabdominal scan)

• EGA [in wks] = (GS [in mm] + 25.43) 7.02

Accuracy:

7 days

Early Embryonic Size

• = length of embryo <25 mm on transvaginal scan performed at <11 weeks MA

• Gestational age (days) = embryonic size (mm) + 42

Accuracy:

3 days

Crown-Rump Length (CRL)

• = length of fetus; useful up to 12 weeks MA (usually identified by 7 weeks MA on transabdominal scan)

Rule of thumb:	MA (in weeks) = CRL (in cm) + 6
Accuracy:	5 7 days

Biparietal Diameter (BPD)

• = measured from leading edge to leading edge of calvarial table at widest transaxial plane of skull = level of thalami + cavum septi pellucidi + sylvian fissures with middle cerebral arteries

  Excellent means of estimating GA in 2^nd trimester >12 weeks MA

• Accuracy:

  2 mm for between occasion error

  Most accurate for dating if combined with HC, AC, FL provided body ratios are normal!

  Less reliable for dating in 3^rd trimester because of increasing biologic variability!

  Accuracy of Biometry (95% confidence range)

  Stage	Based on	Accuracy [weeks]
  1st trimester
  5 6 weeks	US milestones	0.5
  6 13 weeks	CRL	0.7
  2nd trimester
  14 20 weeks	cBPD / HC	1.2
  	BPD / FL	1.4
  20 26 weeks	cBPD / HC	1.9
  	BPD / FL	2.1 2.5
  3rd trimester
  26 32 weeks	cBPD / HC / FL	3.1
  	FL	3.1
  32 42 weeks	cBPD / HC / FL	3.5
  	FL	3.5

Discordant Estimated Date of Confinement (EDC) by LMP and BPD

• Methodological error in measurement

  • wrong axial section

  • cranial compression (multiple gestation, breech presentation, oligohydramnios, dolichocephaly)

• Erroneous LMP

  • other measurements (AC, FL) correlate with BPD

• Abnormal head growth

  • BPD less than AC:

    microcephaly, fetal macrosomia

  • BPD more than AC:

    intracranial abnormality, asymmetric IUGR

Cephalic Index (CI)

= BPD / OFD; measurements of BPD and occipitofrontal diameter (OFD) are both taken from outer to outer edge of calvarium

Confirms appropriate use of BPD if ratio is between 0.70 and 0.86 (2 SD)

Corrected BPD (cBPD)

= BPD and OFD are used to adjust for variations in head shape

Head Circumference (HC)

Used if ratio of BPD/OFD outside 0.70 0.86

HC	= ([BPD + OFD]/2) x
	= ([BPD + OFD] 1.62) 3.1417

Accuracy:	slightly less than for BPD
HC too large:	hydrocephalus, hydranencephalus, intracranial hemorrhage, short limb dystrophies, tumor
HC too small:	anencephaly, cerebral infarction, synostosis, microcephaly vera

Abdominal Circumference (AC)

• = measured at level of vascular junction of umbilical vein with left portal vein ( hockey-stick appearance) where it is equidistant from the lateral walls in a plane perpendicular to long axis of fetus; measured from outer edge to outer edge of soft tissues

  Allows evaluation of head-to-body disproportion

  Better predictor of fetal weight than BPD

AC too large:	GI tract obstructions, obstructive uropathy, ascites, hepatosplenomegaly, congenital nephrosis, abdominal tumor
AC too small:	diaphragmatic hernia, omphalocele, gastroschisis, renal agenesis

Femur Length (FL)

• = measurement of ossified femoral diaphysis

Error:

flare at distal end included in measurement (= reflection from cartilaginous condyle)

Thoracic Circumference (TC)

• = measured in axial plane of chest, which includes four-chamber view of heart without inclusion of SQ tissue

  Linear growth between 16 and 40 weeks similar to AC

Useful age-independent parameter:

TC:AC >0.80

P.1019

Estimated Fetal Weight (EFW)

• based on measurements of head size (BPD / HC), abdominal size (AD / AC), and femur length (FL)

• Accuracy:

  body part used	95% confidence range
  abdomen	22%
  head + abdomen	17 20%
  head + abdomen + femur	15%

Appearance of Epiphyseal Bone Centers

in 95% of all cases

• distal femoral epiphysis (DFE): >33 weeks GA

• distal femoral epiphysis (DFE) >5 mm: >35 weeks

• proximal tibial epiphysis (PTE): >35 weeks GA

• proximal humeral epiphysis (PHE): >38 weeks GA

CNS Ventricles

width of 3^rd ventricle:

<3.5 mm (any gestational age)

Diameter of Cisterna Magna

measured from inner margin of occiput to vermis cerebelli: 2 10 mm

Assessment of fetal well-being

Amniotic Fluid Index

= sum of vertical depths of largest clear amniotic fluid pockets in the 4 uterine quadrants measured in mm

Method:	patient supine, uterus viewed as 4 equal quadrants, transducer perpendicular to plane of floor + aligned longitudinally with patient's spine
Variation:	3.1% intraobserver, 6.7% interobserver
Result:
95th percentile:	185 mm at 16 weeks GA, rising to 280 mm at 35 weeks, declining to 190 at 42 weeks
5th percentile:	80 mm at 16 weeks GA, rising to 100 mm at 23 weeks, declining to 70 mm at 42 weeks

Biophysical Profile (Platt and Manning) = BPP

= in utero Apgar score = assessment of fetal well-being

Gestational age at entry:	25 weeks MA
Observation period:	30 (occasionally 60) minutes; ordinarily <8 minutes needed; in 2% full 30 minutes required

A. ACUTE BIOPHYSICAL VARIABLES

Subject to rhythmic variation coincident with sleep-wake cycle!

• Fetal breathing movement (FBM):

  1 episode of chest + abdominal wall movement for a period lasting 30 seconds (time is arbitrary to avoid confusion with general body movements / maternal respiration)

  stimulated by:	glucose, catecholamine, caffeine, prostaglandin synthetase inhibitor
  suppressed by:	barbiturates, benzodiazepine, labor, hypoxia, asphyxia, prostaglandin E2

• Fetal body movement:

  3 discrete movements of limbs / trunk

  influenced by:

  glucose, gestational age, time of day, maternal drugs, intrinsic rhythm, labor

• Fetal tone

  • upper + lower limbs usually fully flexed with head on chest; least sensitive test parameter

    1 episode of opening + closing of hand / extension + flexion of limb

B. CHRONIC FETAL CONDITION

• 4. Amniotic fluid volume

  at least one pocket 2 cm in vertical diameter in two perpendicular planes

  Avoid inclusion of loops of cord!

BPP Score

for each test:	2 points if normal; 0 points if abnormal
False-negative rate:	0.7:1,000

The probability of fetal death within a week of a BPP score of 8/8 is 1:1,000!

Stress Tests

Nonstress Test (NST)

• Test needed in less than 5% of cases!

• reactive fetal heart rate tracing (normal) = at least 4 fetal heart accelerations (>15 bpm over baseline lasting >15 seconds) in a 20-minute period subsequent to fetal movement >34 weeks GA

• nonreactive (abnormal) fetal heart rate tracing = absence of acceleration in a continuous 40-minute observation period

N.B.:	no heart accelerations in immaturity, during sleep cycle, with maternal sedative use
Accuracy:	false-negative rate of 3.2:1,000 (if done weekly) or 1.6:1,000 (if done biweekly); 50% false-positive rate for neonatal morbidity + 80% for neonatal mortality

Contraction Stress Test (CST)

• = external monitoring after injection of oxytocin / maternal breast stimulation

  >3 uterine contractions in 10-minute period

Accuracy:

false-negative rate of 0.4/1000; 50% false-positive rate

Results of Biophysical Profile Score (including NST for a maximum of 10 points)

Score	Fluid	Interpretation	Perinatal Mortality
10		asphyxia rare	0.0%
8	normal	asphyxia rare	<0.1%
8	abnormal	chronic compromise	8.9%
6	normal	equivocal	variable
6	abnormal	asphyxia probable	8.9%
4		asphyxia highly probable	9.1%
2		asphyxia almost certain	12.5%
0		asphyxia certain	60.0%

P.1020

Invasive fetal assessment

Amniocentesis

• Indications:

  • Inadequate sonographic fetal anatomic survey due to fetal position / maternal body habitus

  • Equivocal sonographic findings (eg, abnormal posterior fossa but spinal defect not seen)

  • Experienced sonographer not available

  • Nonlethal anomaly detected on level I sonogram for which karyotype testing is appropriate

• Risk: fetal loss rate generally quoted as 1:200 (0.5%)

  • FETAL RISK

    • Spontaneous abortion (0.3 1.5%)

    • Amniotic fluid leak

    • Chorioamnionitis

    • Fetal injury: skin dimple, limb gangrene, porencephalic cyst, hemothorax, spleen laceration, orthopedic abnormality, amniotic band syndrome

  • MATERNAL RISK (rare)

    • Bowel perforation

    • Hemorrhage

    • Isoimmunization

Diagnostic Amniocentesis

• Genetic studies: karyotype, DNA analysis, biochemical assay

  Timing: early (11 15 weeks), late (15 18 weeks)

• Neural tube defect: -fetoprotein, acetylcholinesterase

• Isoimmunization: -OD 450

• Fetal lung maturity

• Intraamniotic infection

• Confirmation of ruptured membranes

Advantage over CVS:

• Error rate (<1% versus 2%)

• Culture failure rate (0.6% versus 2.2%)

• Fetal loss rate (0.6 0.8% less)

Therapeutic Amniocentesis

• Polyhydramnios

• Twin-twin transfusion syndrome

  Technique:

  avoid fetus, placenta, umbilical cord, uterine contraction, fibroid, large uterine vessel

  use continuous ultrasound guidance

  inject 2 5 mL of indigo carmine dye in first sac of twin (colorless fluid assures that second sac has been entered)

Chorionic Villus Sampling (CVS)

• = aspiration of cells from chorion frondosum for genetic studies (karyotype, DNA analysis, biochemical assay)

  Transabdominal CVS for rapid karyotyping in 2nd + 3rd trimester = placental biopsy

• Advantage: >2 weeks earlier results compared with amniocentesis

• Timing: 9 11 weeks

• Approach:

  • transcervical route = catheter introduced through cervix into chorion frondosum, easier for posterior placenta; contamination by cervical flora possible

    CONTRAINDICATED in cervical infections!

  • transabdominal route = 20 22-gauge needle inserted from anterior abdominal wall; easier for anterior / fundal placenta; sterile technique

• Chromosome analysis:

  • direct preparation = analysis of cytotrophoblasts (may have different karyotype than fetus) analysis can be performed immediately

  • villus culture = cells from central mesenchymal core (same karyotype as fetus) cultured for several days before analysis

• Errors (2%):

  • Mosaicism = cell line forming cytotrophoblast may develop abnormal karyotype while fetal cell line is normal

  • Maternal contamination = cells from maternal decidua may overgrow mesenchymal core cells

• Risks:

  • Spontaneous abortion (1%)

  • Perforation of amniotic sac

  • Infection

  • Teratogenesis: limb reduction defect

Cordocentesis

• = PERCUTANEOUS UMBILICAL BLOOD SAMPLING (PUBS)

  • DIAGNOSTIC CORDOCENTESIS

    • Hematocrit

    • Karyotype

    • Immunodeficiency: chronic granulomatous disease, severe combined immunodeficiency

    • Coagulopathy: von Willebrand syndrome, factor deficiency

    • Platelet disorder: alloimmune / idiopathic thrombocytopenic purpura

    • Hemoglobinopathy: sickle cell anemia, thalassemia

    • Infection: toxoplasmosis, rubella, varicella, cytomegalovirus, parvovirus

    • Hypoxia / acidosis

  • THERAPEUTIC CORDOCENTESIS

    • Intravascular fetal transfusion (fresh rh-negative CMV-negative leukodepleted irradiated packed cells compatible with mother infused at 10 15 mL/min)

    • Direct delivery of medication to fetus

Cx:

1. Chorioamnionitis
2. Rupture of membranes
3. Umbilical cord hematoma
4. Umbilical cord thrombosis
5. Bleeding from insertion site
6. Fetal bradycardia

Multiple gestations

Incidence:

1.2% of all births;
in 5 50% clinically undiagnosed at term

Occurrence:

P.1021

twins	in	1:85 pregnancies (= 851)
triplets	in	1:7,600 pregnancies (~ 852)
quadruplets	in	1:729,000 pregnancies (~ 853)
quintuplets	in	1:65,610,000 pregnancies (~ 854)

• uterus large for dates

• may have elevated hCG, HPL (human placental lactogen), AFP levels

Perinatal morbidity & mortality compared with singletons:

twins:	up to 5-fold increase
triplets:	up to 18-fold increase

Twin Pregnancy

Zygote = fertilized egg

Monozygotic Twins (1/3)

• = identical TWINS

• = division of a single fertilized ovum during earliest stages of embryogenesis (chorion differentiates 4 days and amnion 8 days after fertilization)

• Incidence: 1:250 birth (constant around the world)

• Predisposing factors:

  • Advanced maternal age

  • In vitro fertilization

    same sex + identical genotype

Cx:	(1) perinatal mortality 2.5 times greater than for dizygotic twins
	(2) Fetal anomalies 3 7 times higher than in dizygotic twins / singletons (often only affecting one twin): anencephaly, hydrocephalus, holoprosencephaly, cloacal exstrophy, VATER syndrome, sirenomelia, sacrococcygeal teratoma

Twinning

Dichorionic Diamniotic Twins (30%)

• = separation at two-cell stage (= blastomere) approximately 60 hours / <4 days after fertilization

• 2 separate fused / unfused placentas

• membrane >2 mm due to 2 separate chorionic sacs + 2 separate amniotic sacs (92% accurate for dichorionic diamniotic twins)

• twin peak sign = triangular projection of placental tissue insinuated between layers of intertwin membrane

Monochorionic Diamniotic Twins (69 80%)

• = separation in blastocyst stage between 4th and 7th day after fertilization (chorion already developed and separated from embryo)

• 2 separate amniotic sacs in single chorionic sac

• Common monochorionic placenta has vascular communications in 100%!

Cx:	(1) Twin-twin transfusion syndrome
	(2) Twin embolization syndrome = DIC in surviving twin from transfer of thromboplastin; 17% morbidity / mortality of survivor after fetal death of twin
	(3) Acardiac parabiotic twin

Monochorionic Monoamniotic Twins (1%)

• = division of embryonic disk between 8th and 12th day after fertilization (amniotic cavity already developed) common amniotic + chorionic sac, no separating membrane

  P.1022

  
  

  Embryologic Events in Monozygotic Twinning

  Days after Fertilization	Embryologic Event	Cleavage Chorion	results in Amnion
  1 2	cell divisions morula	di~	di~
  3 4	chorionic differentiation
  6	blastocyst implants in endometrium	mono~	di~
  8	amnionic differentiation	mono~	mono~
  >13	division of embryonic disk	mono~	mono~
  		but conjoined

• entanglement of cords (the only definitive positive sonographic sign of monoamnionicity)

Cx:	double perinatal mortality up to 45%
	(1) Entangled umbilical cord (70%)
	(2) True knot of cord
	(3) Conjoined twins (umbilical cord with >3 vessels, shared fetal organs, continuous fetal skin contour)
Prognosis:	40% survival rate

Dizygotic Twins (2/3)

• = FRATERNAL TWINS

• (a) fertilization of two ova by two separate spermatozoa during two simultaneous ovulations (occurring either in both ovaries or in one ovary)

• (b) superfetation = fertilization of two ova by two separate spermatozoa during two subsequent ovulations (frequency unknown)

• (c) superfecundation = two ova fertilized by two different fathers (very rare)

• Incidence: 1:80 to 1:90 births

• Predisposing factors:

  • Advanced maternal age (increased up to age 35): reduced gonadal-hypothalamic feedback with increase of FSH levels

  • Ovulation-inducing agents (multiple pregnancies in 6 17% with clomiphene, in 18 53% with Pergonal )

  • Maternal history of twinning (3 times as frequent compared with normal population)

  • Increased parity

  • Maternal obesity

  • Race with inherited predisposition for multiple ovulations (Blacks > Whites > Asians)

    different phenotypes; same / opposite sex

    always dichorionic diamniotic

Growth Rates of Twins

Twins should be scanned every 3 4 weeks >26 28 weeks GA

• Below 30 32 weeks GA

  • normal individual twins grow at same rate as singletons

  • BPD growth rates similar to singleton fetuses

• Beyond 30 32 weeks GA

  • combined weight gain of both twins equals that of a singleton pregnancy (AC of twins < AC of singleton)

  • Weight of twin fetus falls below that of singleton when combined weight of twins >4,000 g!

  • BPD + HC growth may / may not be affected (controversial)

  • FL not affected

Discordant Growth

= weight difference at birth >25%

Cause:	(1) Twin-twin transfusion syndrome
	(2) IUGR of one fetus

BPD difference >5 mm (discordant growth in 20 30%)

discordant HC increases probability of IUGR

AC is single most sensitive parameter for IUGR

EFW is most sensitive set of combined parameters for IUGR

>15% S/D ratio difference of umbilical artery Doppler waveforms between twins

Amnionicity & Chorionicity

• Rules:

  Only monozygotic twins can give rise to monochorionic + monoamniotic pregnancies!

  All monoamniotic twins must also be monochorionic!

  All dizygotic twins must be dichorionic + diamniotic!

  77% of all twin pregnancies are dichorionic (ie, all dizygotics [2/3 of all twins], which equals 67% + 30% of all monozygotics [1/3 of all twins], which equals 10%)

• GESTATIONAL SACS (<10 weeks MA)

  • Accuracy: 100% in 1st trimester, 80 90% in 2nd trimester

  • 2 gestational sacs, each with a live fetus, indicates dichorionic twinning

  • single gestational sac with 2 live fetuses indicates monochorionic twins

  • single extraembryonic coelom indicates monochorionic twins

• YOLK SAC

  • number of yolk sacs = number of amnions

• FETAL GENDER

  • different genders (in 25% of twin pregnancies) must be dizygotic twins and thus dichorionic!

  • [DDx: testicular feminization demonstrates female external genitalia with a 46, XY karyotype]

• PLACENTAL SITES

  • 2 placentas (in 45% of twin pregnancies) indicate dichorionic diamniotic pregnancy

  • 1 placenta indicates

  • (a) monochorionic pregnancy

  • (b) dichorionic pregnancy with fused placenta (occurs in 50% of dichorionic twin pregnancies)

• CHORIONIC PEAK

  • twin peak sign (= triangular projection of placental tissue extending beyond chorionic surface of the placenta + insinuated between layers of intertwin membrane + wider at chorionic surface and tapering to a point some distance inward from surface) indicates dichorionic pregnancy

P.1023




• MEMBRANE

  • separating membrane confirms diamniotic pregnancy, but does not distinguish between mono- or dichorionic pregnancy

  • dichorionic membrane (two layers of chorion + two layers of amnion) is thicker (>2 mm) than monochorionic membrane (two layers of amnion <1 mm): 88 92% accuracy in 1st trimester, 39 83% accuracy in 2^nd + 3^rd trimester

    All membranes appear to be thin in 3^rd trimester! absence of membrane suggests a monoamniotic monochorionic twin pregnancy

    Nonvisualization of membrane is not sufficient evidence of monoamnionicity due to technical factors!

• CORD

  • entanglement of cords is the only definitive positive sonographic sign of monoamnionicity

  • simultaneous recording of fetal arterial signals at nonsynchronous rates within wide Doppler gate

• AMNIOGRAPHY

  • detection of imbibed intestinal contrast in both twins by CT following single sac contrast injection proves monoamniotic monochorionic twin pregnancy

Risks in Multiple Gestations

1. Placental abruption	3-fold
2. Anemia	2.5-fold
3. Hypertension	2.5-fold
4. Congenital anomaly	2 3-fold
5. Preterm delivery	12-fold
6. Perinatal mortality	4 6-fold

Risk increases with number of fetuses, monozygosity, monochorionicity

Risk for IUGR

• monochorionic-monoamniotic > monochorionic-diamniotic > dichorionic-diamniotic

Risk for Perinatal Mortality

• 1% for singletons, 9% for diamniotic dichorionic twins,

  • 26% for diamniotic monochorionic twins,

  • 50% for monoamniotic monochorionic twins

• Prognosis:

  • Perinatal mortality 5 10 times that of singleton pregnancy (91 124:1,000 births)

    • 9% for dichorionic diamniotic twins

    • 26% for monochorionic diamniotic twins

    • 50% for monochorionic monoamniotic twins

    • preterm delivery with birth weight <2,500 g

    • IUGR (25 32%; 2nd most common cause of perinatal mortality + morbidity)

    • amniotic fluid infection (60%)

    • premature rupture of membranes (11%)

    • twin-twin transfusion syndrome (8%)

    • large placental infarct (8%)

    • placenta previa

    • abruptio placentae

    • preeclampsia

    • cord accidents

    • malpresentations

    • velamentous cord insertion (7-fold increase compared with singleton pregnancy)

  • Fetal death in utero (0.5 6.8%; 3 times as often in monochorionic than in dichorionic gestations)

    50% of twin gestations seen at 10 weeks GA will be singletons at birth!

  • Increased risk of congenital anomalies (23:1,000 births = twice as frequent as in singletons; 3 7 times more frequent in monozygotic twins than in dizygotic twins)

Uterus

Uterine Size

Overfilling of urinary bladder can modify uterine shape!

• PREPUBERTAL UTERUS

  • NEONATAL UTERUS

    • Length of 2.3 4.6 cm (mean 3.4 cm),

    • fundal width of 0.8 2.1 cm (mean 1.2 cm),

    • cervical width of 0.8 2.2 cm (mean 1.4 cm)

    • spade-shaped uterus (58%) with cervix often twice as thick as fundus

    • tube-shaped uterus (32%) with cervical + fundal AP measurements identical

    • adult pear-shaped uterus (10%) with fundus wider than cervix

    •    thin echogenic endometrium

    •    endometrial fluid (in 23%) secondary to maternal hormonal stimulation

    •    becomes smaller by 4th month of life (2.6 3.0 cm)

    •    Best time to evaluate uterus in child with ambiguous genitalia is first few months of life!

  • INFANTILE UTERUS

    • Age: infancy to 7 years of age

    • Length of 2.5 3.3 cm, fundal width of 0.4 1.0 cm, cervical width of 0.6 1.0 cm

      cervix occupies 2/3 of uterine length

  • PREMENARCHAL / PREPUBERTAL UTERUS

    • Mean length of 4.3 cm

    • usually tubular configuration with fundocervical ratio of 1:1

• POSTPUBERTAL UTERUS

  • nulliparous:

    5 8 cm (L); 3 cm (AP); 1.6 3.0 cm (TRV)

  • multiparous:

    6 11 cm (L); 3 4 cm (AP); 3 5 cm (TRV)

    fundocervical ratio of 2:1 to 3:1

    mean uterine volume of 90 cm³

• POSTMENOPAUSAL UTERUS

  • cervix occupies 1/3 of uterine length;

  • 5 6.5 cm (L); 2 cm (AP); 1.2 1.8 cm (TRV)

Uterine Zonal Anatomy (on T2WI)

Thickness of zones depends on menstrual cycle + hormonal medication

• ENDOMETRIUM

  • high signal intensity similar to fat

• JUNCTIONAL ZONE

  • = basal layer of myometrium

P.1024

Mean thickness:	2 8 mm
Histo:	compact smooth muscle fibers with 3-fold increase in number + size of nuclei compared with outer myometrium
low signal intensity (lower water content); seen in 40 60%, may not be visible in premenarchal + postmenopausal women

• MYOMETRIUM

  • intermediate signal intensity, increases during secretory phase

• Outer Surface of Uterus

  • thin low-signal intensity line

Cervical Zones (on T2WI)

• Central stripe of high signal intensity

  Histo:	secretions in endocervical canal + cervical mucosa + plicae palmatae
  arbor vitae / plicae palmatae = irregular branched mucosal pattern of cervical canal

• Middle layer of low signal intensity continuous with junctional zone of corpus uteri

  Histo:

  inner zone of fibromuscular stroma with percentage of nuclear area 2.5 times greater than in outer zone

• Outer layer of intermediate signal intensity

  Histo:

  outer zone of fibromuscular stroma

• Outermost thin low-intensity serosal layer continuous with uterine serosa

Uterine Enhancement Pattern

• Myometrium

  demarcates level of internal cervical os

• Inner mucosal + outer stromal layers of cervix

• Inner stromal layer of cervix

Endometrium

• Measurements refer to

  • AP diameter of both apposed endometrial layers (= bilayer thickness) excluding intrauterine fluid

  • the level of the uterine fundus

  • midline long-axis image of uterus

  Measurements increase by 1 2 mm in patients with large body habitus

  If there is a discrepancy between concomitant endometrial + ovarian findings bleeding is usually associated with anovulatory cycles

• MENSTRUAL PHASE (usually days 1 5)

  Thickness:	1 4 mm
  interrupted thin echogenic line of central interface

• PROLIFERATIVE PHASE (days 6 14)

  • Thickness: 5 7 mm

    • bright echogenic central line (= apposed borders of endometrial canal)

    • thickened isoechoic to hyperechoic endometrium compared with myometrium (due to development of glands, blood vessels, stroma)

• PERIOVULATORY PHASE (day 14)

  • Thickness: up to 11 mm

    • triple ring sign = multilayered endometrium:

      • echogenic basal layer

      • hypoechoic inner functional layer

      • thin echogenic median layer arising from the central interface

        

        Normal Premenopausal Endometrium

    • concomitant with mature preovulatory follicle

    • disappears within 48 hours after ovulation

• SECRETORY PHASE (days 15 28)

  • Thickness: 7 12 16 mm

    • bright central line

    • markedly echogenic thick endometrium (due to stromal edema + distended glands filled with mucus and glycogen):

      • posterior acoustic enhancement

      • maximum thickness during midsecretory phase

      • concomitant with a corpus luteum

    • thin hypoechoic halo of inner myometrial zone

Postmenopausal Endometrium

• NO HORMONAL REPLACEMENT THERAPY

  • bilayer thickness of <5 mm with a homogeneous echogenic endometrium

Histo:

consistently associated with atrophic inactive endometrium

• WITH HORMONAL REPLACEMENT THERAPY

  • cyclic estrogen + progestin therapy

    • endometrial thickness may increase to 8 mm:

      • thickest prior to progestin exposure

      • thinnest after progestin phase (imaging should be done at the beginning / end of a treatment cycle)

  • unopposed estrogen therapy / continuous estrogen + progesterone

    • endometrial thickness may increase to 15 mm

    • Rx: biopsy / D&C recommended if endometrial thickness >8 mm

Normal Postpartum Endometrium

• endometrial cavity <20 mm in diameter:

  • small echogenic foci of retained membranes / clot / debris (in up to 24% of healthy patients)

  • intrauterine air (in up to 21% of healthy patients)

• cavity wall:

  • smooth well-defined border

  • irregular heterogeneous lining

  • endometrial stripe thickness decreases with uterine involution

Pelvic Spaces

• Rectouterine pouch = cul-de-sac

  • Anterior boundary: broad ligaments + uterus

  • Most dependent portion of pelvis in women!

• Rectovesical recess

  P.1025

  
  

  Most dependent portion of pelvis in men!

• Vesicouterine recess

• Inguinal fossa

  located between lateral + medial umbilical folds

Pelvic Ligaments

• 1. Broad ligament

Histo:	2 layers of peritoneum
Origin:	uterine peritoneum
Attachment:	pelvic sidewall

• 2. medial superior free edge:

  formed by fallopian tube

• 3. lateral superior free edge:

  suspensory ligament of ovary

• 4. lower margin: cardinal ligament

• 5. Contents (= parametrium):

  • extraperitoneal connective tissue, smooth muscle, fat, fallopian tube, round ligament, ovarian ligament, uterine + ovarian blood vessels, nerves, lymphatics, mesonephric remnants

• 5. Round ligament

  • = anterior suspensory ligament of uterus

  Histo:	band of fibromuscular tissue + lymphatic channels
  Origin:	anterolateral uterine fundus, just below + anterior to ovarian ligament
  Attachment:	through internal inguinal canal (lateral to deep inferior epigastric vessels) to labia majora

• Cardinal ligament = transverse cervical ligament = Mackenrodt ligament

  Origin:	cervix + upper vagina
  Attachment:	fascia of obturator internus muscle
  Relationship:     uterine artery runs along its superior aspect     forms the base of the broad ligament

• Uterosacral ligament

  Origin:	posterolateral cervix + vagina
  Attachment:	anterior body of sacrum at S2 or S3

• Ovarian ligament = round ligament of the ovary

  Origin: medial aspect of ovary

  Attachment: uterus, just inferior + posterior to fallopian tube + round ligament

• Suspensory ligament of ovary = infundibulopelvic lig.

  Origin:	anterolateral aspect of ovary
  Attachment:	connective tissue over psoas muscle
  Contents:	ovarian artery + vein

• Lateral umbilical fold / ligament

  • = reflection of peritoneum over deep inferior epigastric vessels

• Medial umbilical fold / ligament

  • = reflection of peritoneum over obliterated umbilical arteries

• Median umbilical ligament

  • = reflection of peritoneum over obliterated urachus

  • Origin: dome of urinary bladder

  • Attachment: umbilicus

Fallopian Tube

Location: superior aspect of broad ligament

Length: 10 12 cm

Segments:

• Interstitial / cornual portion

  • = short segment that traverses muscular wall of the uterus

• Isthmic portion

  • = long narrow segment between interstitial + ampullary end

• Ampullary portion

  • = widened region near ovary

Ovaries

• Fixation:

  fairly mobile with attachments to

  anterior pelvic wall	by	broad ligament
  uterine body	by	uteroovarian ligament
  fallopian tube	by	tuboovarian ligament
  lateral pelvic wall	by	infundibulopelvic ligament

• Histology:

  • Stromal cells

    • form cortex, medulla, hilus

    • surround all developing follicles

    • cell surface has estrogen-, progesterone-, testosterone-binding sites

    • major source of androgens in postmenopause

  • Cells derived from stroma cells

    • Fibroblasts

    • Luteinizing stromal cells containing lipid

    • Hilus cells

      morphologically similar to Leydig cells in testis; contain intracellular crystalline structures (= crystals of Reinke)

    • Theca cells

      • surround granulosa cells

      • produce estradiol under control of FSH

      • stimulated by LH after ovulation into theca-lutein cells, which develop lipid-laden cytoplasm involved in steroidogenesis

  • Cells derived from sex cords

    • Granulosa cells

      • derived from sec cords

      • surround each primordial oocyte

      • form lining of developing follicle

      • granulosa cell layer contains small cystic cavities lined by basal lamina (= Call-Exner bodies)

      • produce estradiol under control of FSH

      • stimulated by LH after ovulation into granulosa-lutein cells, which develop lipid-laden cytoplasm involved in steroidogenesis

    • Sertoli cells

      • not seen in normal ovary

• Embryology:

  coelomic (surface) epithelium invaginates into mesenchymal substance (= primary sex cords) and incorporates primordial germ cells, which develop into primordial follicles

Ovarian Size

Ovarian volume = length height width 0.523

P.1026

<3 months:	1.06 3.56 cm3
4 12 months:	up to 2.71 cm3
1 year:	1.05 0.7 (S.D.) cm3
2 6 years:	1.0 0.4 (S.D.) cm3
6 10 years:	1.2 2.3 cm3
11 12 years:	2 4 cm3
after puberty:	2.5 5 cm (L), 0.6 1.5 cm (H),1.5 3 cm (W) = 8 (range 2.5 20) cm3

An ovary >20 cm³ is enlarged!

An ovary between 15 and 20 cm³ requires follow-up!

Ovarian Morphology

neonate:

• follicles occasionally fail to involute + undergo growth

<8 years:

• solid ovoid structures with homogeneous / finely heterogeneous texture

• up to 68 80% of ovaries contain cystic follicles (in 95% <9 mm, in 5% >9 mm)

Visualization of Ovaries

• after menopause (average onset at age 50):

  <5 years after menopause:	in 78%
  >10 years after menopause:	in 64%
  both ovaries:	in 85%
  one ovary:	in 60%

• following hysterectomy: in 43%

Ovarian Cycle

• FOLLICULAR PHASE = days 1 14

  • a number of immature primordial follicles begin to mature in response to FSH

    multiple small cysts:

    • unstimulated follicles are <2 mm in size

    • stimulated follicles grow >2 mm in size 2 3 follicles in each ovary of day 4 enlarge subsequently to approximately 10 mm

    • 2 3 follicles capture the most FSH and aromatize the most estradiol from their granulosa cells

      single ascendant / dominant follicle of 8 12 mm (= graafian follicle) appears by day 10:

      • grows by 2 mm/day

      • subsequently enlarges to 21 (range 17 24) mm by day 14

  • rapidly rising estradiol production triggers the hypothalamic arcuate nucleus to increase GnRH secretion, which prompts the anterior pituitary to disgorge the stored LH over 24 hours

  • LH binds to ovarian receptors + releases cAMP from granulosa cells halting granulosa cell mitosis + increasing peptidase, collagenase, growth factor, angiotensin, prostaglandin as the cause for follicular rupture + conversion to corpus luteum

  progressively increasing diastolic flow on the side of maturing follicle

• OVULATORY PHASE = day 14

  • Mittelschmerz = pain just prior to ovulation (pressure of graafian follicle distending ovarian capsule)

    sudden decrease in follicular size over minutes / hours (= rupture of mature graafian follicle with extrusion of ovum)

• LUTEAL PHASE = days 15 28

  • All corpora lutea evolve over days and change size and texture constantly

  • All corpora lutea contain hemorrhage:

  • uncontrolled bleeding into center frequently at time of ovulation + on day 8 when regression begins

  • round / ovoid bulging protrusion on one side of ovary = corpus luteum of menstruation:

    • mean diameter of 10 25 mm

    • hyperechoic 1 4-mm thick wall

    • hyperechoic central blood clot gradually transforming to weblike fibrin net

    • blood flow in main arteriole supplying the corpus luteum is approximately 100 cm/sec

    • surrounded by wreath-like color flow

  • involution + atrophy of corpus luteum on about 24th day of cycle = corpus luteum atreticum

Prognosis:

large painful corpora lutea will resolve in a week

Graafian Follicle

Size of mature graafian follicle:

17 29 mm

• growth rate 3 mm/day until the last preovulatory 24 hours followed by a sudden increase in diameter

• cumulus = 1-mm mural echogenic focus projecting into antrum of follicle + containing oocyte, followed by ovulation within next 36 hours

Signs of Ovulation

• development of solid echoes within graafian follicle

• decrease in diameter / sudden collapse of dominant follicle 28 35 hours after LH peak

• ring structure within uterine fundus

• free fluid appearing in pouch of Douglas

Signs of Ovulatory Failure

• development of internal echoes prior to 18 mm size

• continuous cystic enlargement up to 30 40 mm

Ovarian Doppler Signals

• NONFUNCTIONING OVARY

  • high-impedance waveform

• FUNCTIONING OVARY

  • days 1 6:

    high-impedance waveform with RI close to 1.0

  • days 7 22 = midfollicular to midluteal phase

    • = developing dominant follicle + ovulation + corpus luteal phase:

    • continuous diastolic flow with RI close to 0.5

  • days 23 28 = late luteal phase:

    high-impedance waveform with RI close to 1.0

Hormonal Status

Thelarche	=	onset and progress of breast development
		Mean age: 8 years
Adrenarche	=	onset and progress of pubic (pubarche) + axillary hair development
		Mean age: 9.8 years
Menarche	=	first episode of vaginal bleeding originating from the uterus
		Mean age: 12.7 years in USA

P.1027

Obstetric and Gynecologic Disorders

Abortion

• = loss of products of conception <20 weeks of MA (definitions may vary)

• INDUCED ABORTION

  • medical / therapeutic abortion

  • nonmedical abortion

• SPONTANEOUS ABORTION

Spontaneous Abortion

• = FAILED PREGNANCY = PREGNANCY LOSS

• = MISCARRIAGE

• Incidence:

  • >50% of all fertilized ova (estimate)

  • 31 43% of all implantations (estimate)

  • 10 25% of clinically diagnosed pregnancies

  • 2 4% with normal cardiac activity

  • decreases with increasing gestational age

    Time of loss:

    <8 10 weeks MA; the majority occur before 7th week MA

• Etiology (usually due to abnormal karyotype):

  • autosomal trisomy (52%), triploidy (20%), monosomy (15%)

• Spontaneous pregnancy loss at <8 weeks gestation occurs in 10 17% of embryos with cardiac activity!

  Signs of Abnormal Pregnancy

  thin decidual reaction of <2 mm

  abnormally shaped sac

  gestational sac low in uterus

Impending / Inevitable Abortion

• = ABORTION IN PROGRESS

• = gestational sac with embryo having become detached from implantation site; leading to spontaneous abortion within next few hours

• Clinical triad:

  • bleeding >7 days

  • persistent painful uterine contractions

  • rupture of membranes

• moderate effacement of cervix

• dilated cervix >3 cm

• sac located low within uterus (DDX: cervical ectopic with closed internal os)

• progressive migration of sac toward / into cervical canal (on rescanning a short time later)

• dilated cervix

• sac surrounded by anechoic zone of blood

Threatened Abortion

• = 1st trimester bleeding (after period of implantation bleed at 3 4 weeks MA) with a live embryo

  Incidence:

  20 25% of all pregnancies

• Clinical triad:

  • mild bleeding

  • cramping

  • closed cervix

Nonviability diagnosis with certainty (transvaginal scan)

• @ Cardiac activity:

  • no cardiac activity with a CRL 5 mm

  • no cardiac activity with certain GA 6.5 weeks

  • no cardiac activity with a GS diameter >16 mm

• @ Yolk sac:

  • no yolk sac with a GS diameter 20 mm

  • embryo visualized without demonstrable yolk sac

  • yolk sac diameter >5.6 mm at <10 weeks MA

• @ GS contents:

  • fibrinous strands / residual embryonic debris (in 25%)

Nonviability diagnosis with high probability (transvaginal scan)

• @ Ultrasound milestone not met as expected:

  5.0 weeks	gestational sac first identifiable
  5.5 weeks	yolk sac first identifiable
  6.0 weeks	embryo and FHM first identifiable

• @ Yolk sac:

  • no yolk sac with GS of 6 9 mm

  • distorted sac configuration

• @ Choriodecidua

  • thinning of choriodecidual reaction with hypoechoic clefts

• @ Cardiac activity:

  • no cardiac activity with GS of 9 mm

  • slow embryonic heart rate (=bradycardia):

    6.2 Weeks	7.0 Weeks	Mortality Rate
    >100 bpm	>120 bpm	11%
    90 99 bpm	110 119 bpm	32%
    80 89 bpm	100 109 bpm	64%
    <80 bpm	<100 bpm	100%

• Predictors of poor outcome:

  • @ Bradycardia

    • <85 bpm during 5 8 weeks EGA

  • @ Small sac size = first-trimester oligohydramnios

    • [misnomer: amnionic cavity is not diminished in size but rather the chorionic cavity]

    • = MSD (mean sac diameter) CRL 5 mm (with a live embryo at 5.5 9.0 weeks)

      Prognosis:

      miscarriage in 94%

  • @ Abnormal yolk sac

    • failure to visualize YS at 5.5 weeks MA = mean

    • diameter of GS 8 mm

    • yolk sac size >5 mm

    • calcification / debris within yolk sac

    • double appearance of yolk sac

  • @ Abnormal amnion

    • mean diameter of amniotic cavity > CRL

  • @ Subchorionic hemorrhage

    Frequency:	in up to 18% of pregnancies during first half
    N.B.:	significance controversial
    Prognosis:	50% develop normally; 15 39% blighted ovum, 4% mole, 4 13% ectopic pregnancy, 0 15% incomplete abortion, 17 57% missed abortion

P.1028

Complete Abortion

• cervix closed

• abrupt decline of serum -hCG

• IUP documented previously

• thin regular endometrium with apposed surfaces

• absence of central / eccentric fluid-filled sac

  DDx:	nongravid state, very early IUP, ectopic pregnancy
  Rx:	dilatation & curettage may be avoided if IUP was documented previously

Incomplete Abortion

• = RETAINED PRODUCTS OF CONCEPTION

• = portion of chorionic villi (placental tissue) / tropho-blastic tissue (fetal tissue) remaining within uterus

• continued (occasionally massive) genital bleeding (may occur months / years after last abortion / delivery)

• patulous cervix

• US (overall accuracy 96%):

  Finding	Retained Products
  gestational sac / collection	100%
  sac with dead fetus	100%
  endometrium >5 mm thick	100%
  endometrium 2 5 mm thick	43%
  endometrium <2 mm thick	14%

• usually no embryo / fetus seen

• irregular / angulated small gestational sac containing amorphous echogenic material

• ragged disrupted choriodecidual reaction

• subchorionic fluid hemorrhage

  Cx:	endometritis, myometritis, peritonitis, septic shock, diffuse intravascular coagulation (with retention >1 month)
  Rx:	suction D&C after IV oxytocin
  DDx:	mole, blighted ovum, embryonic demise, intrauterine fetal death

Placental Polyp

• = intrauterine polypoid mass formed by a retained fragment of placental tissue after an abortion / term pregnancy

  Predilection in:

  placenta accreta

• MR:

  • hyperintense polypoid mass on T2WI

    DDx:

    arteriovenous malformation, trophoblastic disease, endometrial polyp, submucosal myoma

Missed Abortion

= dead conceptus within uterine cavity 8 weeks occurring prior to 28 weeks MA

Time of diagnosis:

not before 13 weeks MA

• brownish vaginal discharge

• closed firm cervix

• no cardiac activity in a well-defined embryo with CRL >9 mm (on abdominal scan) / CRL >5 mm (on transvaginal scan)

• gestation not in correspondence with menstrual age

• sac >25 mm in diameter without an embryo (DDx: anembryonic pregnancy)

• sac >20 mm without yolk sac

• crenated irregular / distorted angular sac configuration

• stringlike debris within gestational sac (in 25%)

• discontinuous / irregular / thin (2 mm) choriodecidual reaction

• no double decidual sac

• low sac position

• subchorionic collection

  Cx:	coagulopathy secondary to low plasma fibrinogen (after 4 weeks in 2nd trimester pregnancy)
  Rx:	suction D&C (in 1st trimester); prostaglandin E suppositories (in 2nd trimester)
  DDx:	blighted ovum

Acardia

• = ACARDIAC MONSTER = TWIN REVERSED ARTERIAL PERFUSION SEQUENCE (TRAP)

• = rare developmental anomaly of monochorionic twinning in which one twin develops without a functioning heart

  Incidence:

  1:30,000 35,000 births; in 1% of monozygotic twins

• Pathophysiology:

  • normal twin perfuses acardiac twin through artery-to-artery + vein-to-vein anastomoses in shared placenta; reversed circulation alters hemodynamic forces, which result in abnormal cardiac morphogenesis

• Spectrum:

  • Holoacardia = no heart at all

  • Pseudoacardia = rudimentary cardiac tissue

• proximity of the two cord insertions on placental surface

• linked by an arterioarterial anastomosis

• reversed arterial flow in cord toward acardiac twin

• fused placentas

• polyhydramnios

• PUMP TWIN

  • at increased risk for fetal demise + preterm labor

  • morphologically normal

  • cardiac overload signs: hydrops, IUGR, hypertrophy of right ventricle, increased cardiothoracic ratio, hepatosplenomegaly, ascites

• PERFUSED TWIN = ACARDIAC TWIN

  • monochorial placenta (same gender) with vascular anastomosis sustains life of acardiac monster; wide range of associated abnormalities

  • absent / rudimentary heart ( acardius )

  • tiny / absent cranium (acephalus)

  • small upper torso absent / deformed upper extremities

  • marked integumentary edema + cystic hygroma

    Prognosis:	mortality of 100% for perfused twin, 50% for pump twin (increased with increased size of acardiac twin)
    Rx:	laser ablation of umbilical cord to acardiac twin (up to 20 22 weeks)

Adenomyosis

• = ENDOMETRIOSIS INTERNA

• = focal / diffuse benign invasion of myometrium by endometrium (heterotopic endometrial islands ), which incites reactive myometrial hyperplasia

P.1029

Cause:	? uterine trauma (parturition, myomectomy, curettage), chronic endometritis, hyperestrogenemia
Incidence:	9 20 31% of hysterectomy specimens
Hormonal dependency:	adenomyosis involves only basal layer of endometrium; largely nonfunctioning due to resistance to hormonal stimulation unlike endometriosis with some degree of proliferative + secretory changes during menstrual cycle
Path:	endometrial glands deeper than 1/4 of thickness of junctional zone
Histo:	endometrial glands + stroma within myometrium surrounded by smooth muscle hyperplasia
Age:	multiparous women >30 years during menstrual life (later reproductive years)
Associated with:	endometriosis (in 36 40%)

• asymptomatic in 5 70%

• pelvic pain, menorrhagia, dysmenorrhea (abates after menopause)

• HSG:

  • small diverticula extending into myometrium

  • irregular masslike filling defect in uterine fundus in focal adenomyosis

• MR (86 100% sensitive, 66 100% specific, 85 90% accurate):

  • myometrial mass with indistinct margins of primarily low signal intensity on all sequences (due to surrounding reactive dense smooth muscle hypertrophy)

  • diffuse / focal widening of junctional zone (= inner myometrium) 12 mm on T2WI, T2-weighted SE images, contrast-enhanced T1WI

  • pseudowidening of endometrium (= indistinct foci of endometrial invasion of myometrium)

  • central high-intensity spots / linear striations on T2WI (due to ectopic endometrial tissue / endometrial cyst / hemorrhagic foci) in 50%

  • cystic adenomyosis = well-circumscribed cystic myometrial lesions of hemorrhage in different stages of organization (in 40% of diffuse adenomyosis, in 100% of focal adenomyosis)

  • enhancement always less than adjacent myometrium

• US (80 86% sensitive, 50 96% specific, 68 86% accurate):

  • focal / diffuse heterogeneous myometrial echotexture (in 75%)

  • nodular / linear areas of increased myometrial echogenicity (= heterotopic endometrial tissue)

  • area of decreased myometrial echogenicity (= smooth muscle hyperplasia)

  • <5 mm small myometrial cysts (in 50%) due to dilated cystic glands / hemorrhagic foci:

    • Swiss cheese appearance of myometrium in cystic adenomyosis

  • poor definition of endomyometrial junctional zone (= endometrial tissue extending into myometrium)

  • pseudowidening of endometrium due to increased myometrial echogenicity

  • thickening + asymmetry of anterior and posterior myometrial walls

  • lack of uterine contour abnormality / mass effect

    Cx:

    infertility

• DDx:

  • Leiomyoma (well-defined borders, mass effect, globular shape, large vessels at lesion margin, calcifications, edge shadowing, whorled appearance)

  • Endometrial carcinoma (error in staging if adenomyosis coexists)

  • Myometrial contraction (transient nature, distortion of endometrial lining)

  • Muscular hypertrophy (hypoechoic inner myometrium, diffuse junctional zone thickening)

• DDx of cystic adenomyosis:

  • Leiomyoma with hemorrhagic degeneration

  • Hematometra

    Rx:

    hysterectomy (the only definitive cure for debilitating adenomyosis)

Diffuse Adenomyosis (67%)

• smooth uterine enlargement (DDx: diffuse leiomyomatosis)

• widening of junctional zone >12 mm on T2WI

Focal Adenomyosis (33%)

• = ADENOMYOMA

• myometrial mass of 2 7 cm in diameter

• oval / elongated shape (DDx: leiomyoma is round)

• ill-defined margins (DDx: sharp margin in leiomyoma)

• contiguity with junctional zone (DDx: leiomyomas may occur anywhere in myometrium)

Amniotic Band Syndrome

• = EARLY AMNION RUPTURE SYNDROME

• = rupture of the amnion exposing the fetus to the injurious environment of fibrous mesodermic bands that emanate from the chorionic side of the amnion

  Prevalence:

  1:1,200 1:2,000 1:15,000 live births

• very thin membrane that flaps with fetal movement or attaches to fetus

• abnormal sheet / bands of tissue that attach to the fetus (DDx: uterine synechiae, incomplete amniochorionic fusion, amniochorionic separation due to subchorionic hemorrhage, fibrin deposits, venous lakes, residual sac of blighted twin pregnancy, wisps of umbilical cord)

• restriction of fetal motion secondary to entrapment of fetal parts by bands

• Associated with fetal deformities in 77%:

  • Limb defects (multiple + asymmetric)

    • amputation / constriction rings of limbs / digits

    • distal syndactyly

    • clubbed feet (30%)

  • Craniofacial defects

    • = asymmetric nonanatomic defects of skull + brain

    • anencephaly

    • asymmetric lateral encephalocele

    • facial clefting of lip / palate

    • asymmetric microphthalmia

    • incomplete / absent cranial calcification

    • attachment of head to uterine wall

  • Visceral defects

    • gastroschisis exteriorization of liver

    • omphalocele

    • gibbus deformity of spine

• DDx:

  • Chorioamnionic separation

  P.1030

  
  

  • Intrauterine synechiae

Anembryonic Pregnancy

• = BLIGHTED OVUM

• = abnormal intrauterine pregnancy with developmental arrest prior to formation of embryo; may occur as a blighted twin

  Cause:

  early arrest of embryonic development related to chromosomal abnormality

• vaginal bleeding

• empty gestational sac (>6.5 weeks MA)

• yolk sac identified without embryo:

  • vanishing (passed) yolk sac on serial scans

• gestational sac small / appropriate / large for dates:

  • decrease in gestational sac (GS) size

  • GS fails to grow by >0.6 mm/day on serial scans

• irregular weakly echogenic decidual reaction of <2 mm

• distorted sac shape

• by transabdominal scan:

  • GS usually not visualized before 5 5.5 weeks MA; yolk sac forms at 4 weeks MA when GS is 3 mm; embryo usually visualized by 6 weeks MA

  • GS size 10 mm of mean diameter without DDS

  • GS size 20 mm of mean diameter without yolk sac

  • GS size 25 mm of mean diameter without embryo

• by transvaginal scan

  • normal intradecidual GS routinely detected at 4 5 weeks with a mean sac diameter of 5 mm

  • GS size 8 mm of mean diameter without yolk sac

  • GS size 16 mm of mean diameter without cardiac activity

    Cx:

    first trimester bleeding

Arteriovenous Malformation of Uterus

• = UTERINE ARTERIOVENOUS FISTULA = UTERINE CIRSOID ANEURYSM

  Associated with:

  dilatation & curettage, endometrial carcinoma, gestational trophoblastic disease

• genital bleeding, often requiring blood transfusions

• MR:

  • tortuous tubular signal voids in myometrium /parametrium / protruding into endometrial cavity on T1WI + T2WI

  • vascular lake with sluggish flow hyperintense on T2WI

  • intensely enhancing lesion isointense to vessels on contrast-enhanced dynamic subtraction MR

Asherman Syndrome

• = association of multiple intrauterine synechiae (= adhesions consisting of fibrous tissue or smooth muscle) with menstrual dysfunction + infertility

Cause:	sequelae of endometrial trauma (vigorous instrumentation during dilatation & curettage) usually during postpartum or postabortion period / severe endometritis
Path:	scars / bands of fibrous tissue (synechiae) connect opposing sides of the endometrium by crossing the endometrial cavity; scars cause the endometrial cavity to contract resulting in an irregular surface

• hypomenorrhea / amenorrhea

• habitual abortion / sterility

• HSG:

  • solitary / multiple filling defects

  • bands of tissue traversing endometrial cavity

  • irregular surface contour of uterine cavity

  • small uterine cavity partially / near completely obliterated (DDx: DES exposure)

• Sonohysterography:

  • echogenic bands bridging the uterine cavity

    • Thick fibrotic bands may prevent complete uterine distension

• MR:

  • small thin irregular endometrial cavity

Beckwith-Wiedemann Syndrome

• = EMG SYNDROME (Exomphalos = omphalocele, Macroglossia, Gigantism)

• = common autosomal dominant overgrowth syndrome with reduced penetrance + variable expressivity related to short arm of chromosome 11; sporadic in 85%

• Increased risk of benign + malignant tumors of multiple organs: Wilms tumor > adrenocortical neoplasm > hepatoblastoma

Incidence:

1:13,700 to 1:14,300 live births; M:F = 1:1

• neonatal polycythemia

• advanced bone age

• Constellation:

  (1) Hemihypertrophy	13 33%
  (2) Hyperplastic visceromegaly: kidney, liver, spleen, pancreas, clitoris, penis, ovaries, uterus, bladder	57%
  (3) Abdominal wall defects
  (a) Omphalocele	76%
  (b) Umbilical hernia	49%
  (c) Diastasis recti	33%
  (4) Macroglossia	98%
  (5) Facial nevus flammeus	63%
  (6) Ear lobe creases and pits	66%
  (7) Prominent eyes with intraorbital creases
  (8) Infraorbital hypoplasia	81%
  (9) Gastrointestinal malrotation	83%
  (10) Pancreatic islet hyperplasia
  (11) Cardiac anomalies
  (12) Natal / postnatal gigantism	77%

• @ Adrenal gland

  Histo:

  adrenocortical hyperplasia, hyperplastic adrenal medulla, cystic adrenal cortex, bilateral adrenal cytomegaly (= enlargement of fetal cortical cells)

• @ Kidney

  Histo:

  disordered lobar arrangement, medullary dysplasia

  • nephromegaly

  • increased cortical echogenicity (due to glomeruloneogenesis)

  • accentuation of corticomedullary definition

  • medullary sponge kidney

  • pyelocaliceal diverticula

• OB-US:

  • LGA fetus with growth along 95th percentile

  • polyhydramnios (51%)

  • thickened placenta

  • long umbilical cord

Cx:	(1) Development of malignant tumors (in 10%)
	(2) Neonatal hypoglycemia (50 61%)

P.1031

Brenner Tumor

• = almost always benign ovarian tumor

Incidence:	1.5 2.5%
Histo:	transitional epithelial cells within prominent fibrous connective tissue stroma
Associated with:	mucinous cystadenoma / other epithelial tumor in 20 30%
Peak age:	40 70 years

• may have estrogenic activity

• usually hypoechoic solid homogeneous tumor with well-defined back wall

• mostly 1 2 cm (up to 30 cm) in diameter

• extensive calcifications

• bilateral in 5 7%

Cervical Cancer

6th most common cause of death from cancer in women; 3rd most common gynecologic malignancy (after endometrial + ovarian cancer); 12,800 new cases + 4,600 deaths in 2000 in United States

Incidence:	12:100,000 women per year
Peak age:	45 55 years
Histo:	squamous cell carcinoma (95%) arising low in endocervical canal, adenocarcinoma (5%) arising from endocervical columnar epithelium, clear cell adeno-carcinoma (unusual) in women exposed to DES in utero
Risk factors:	lower socioeconomic class, Black race, early marriage, increased parity, early age at first intercourse, numerous sexual partners, cigarette smoking, human papillomavirus infection (HPV type 16 DNA)

• Significance of tumor size:

  >4 cm:	nodal metastases (80%), local recurrence (40%), distant metastases (28%)
  <4 cm:	nodal metastases (16%), local recurrence (5%), distant metastases (0%)

• Spread:

  • direct extension to lower uterine segment + vagina + paracervical space along uterosacral and broad ligaments

  • lymphatic: paracervical > parametrial > hypogastric + obturator > external iliac > common iliac + presacral > para-aortic nodes

  • hematogenous: lung, liver, bone

  • leukorrhea vaginal bleeding (<30%)

  • postcoital bleeding / metrorrhagia

• Location:

  • centered at level of cervix, originating from

    • squamocolumnar junction (in young woman)

    • endocervical canal (older woman)

  • with protrusion into vagina / invasion of lower myometrium

• CT:

  • @ Primary tumor

    • growth pattern: exophytic, infiltrating, endocervical

    • bulky enlargement of cervix >3.5 cm (DDx: cervical fibroid)

    • iso- (50%) / hypoattenuating (due to necrosis, ulceration, reduced vascularity) after IV contrast

    • gas within tumor (necrosis / prior biopsy)

    • fluid-filled uterus (blood, serous fluid, pus) secondary to obstruction

    • hypoattenuating lesion of myometrium / with vaginal distension

  • @ Parametrial spread (30 58% accuracy):

    • parametrial soft-tissue mass

    • ureteral encasement

    • thickening of ureterosacral ligaments

    • >4-mm soft-tissue strands of increased attenuation extending from cervix into parametria, cardinal / sacrouterine ligaments

    • obliteration of fat planes

    • ill-defined irregular cervical margins

    • eccentric parametrial enlargement

      DDx:

      parametrial inflammation due to instrumentation, ulceration, infection, prior pelvic surgery, endometriosis

  • @ Pelvic side wall disease

    • tumor <3 mm from side wall

    • enlarged piriform / obturator internus muscles

    • encasement of iliac vessels

    • destruction of pelvic bones

  • @ Pelvic visceral disease (60% PPV)

    • loss of perivesical / perirectal fat plane

      Staging of Cervical Cancer F d ration Internationale de Gyn cologie et d'Obst trique

      FIGO Stage	Description	Nodal Metastases
      0	Carcinoma in situ (before invasion)	0.3%
      I	Confined to cervix
      IA	preclinical invasive carcinoma
      IA1	microinvasion of stroma (<3 mm deep and <7 mm wide)	0.3%
      IA2	tumor >3 mm but 7 mm horizontal spread	14%
      IB	tumor larger than IA	16%
      IB-1	4 cm
      IB-2	>4 cm
      II	Extension beyond cervix but not to pelvic wall / lower one-third of vagina
      IIA	vaginal extension excluding lower 1/3	33%
      IIB	parametrial invasion excepting pelvic sidewall	37%
      III	Extension to pelvic wall / lower third of vagina
      IIIA	invasion of lower 1/3 of vagina
      IIIB	pelvic side wall invasion + hydronephrosis
      IV	Located outside true pelvis	55%
      IVA	invasion of bladder / rectal mucosa
      IVB	spread to distant organs (paraaortic / inguinal nodes, intraperitoneal metastasis)

    P.1032

    
    

    • asymmetric nodular thickening of bladder / rectal wall

    • intraluminal mass

    • air in bladder due to fistula

  • @ Lymphatic spread (65 77 80% accuracy)

    • nodes >1 cm in diameter (>7 mm for internal iliac nodes, 9 mm for common iliac nodes, >10 mm for external iliac nodes) with 44% sensitivity

    • lymph node necrosis (100% PPV)

      DDx:

      adenopathy from secondary tumor infection

• MR (76 78 91% accuracy for staging, 82 94% accuracy for parametrial involvement):

  • @ Primary tumor

    • focal bulge / mass in cervix:

      • mass isointense on T1WI

      • hyperintense on T2WI compared with fibrous stroma (DDx: postbiopsy changes, inflammation, nabothian cysts)

      • size of tumor accurately depicted (on T2WI rarely overestimated due to inflammation / edema)

        • Tumor diameter and probability of recurrence + metastases are related; tumor diameter >4 cm (stage IB2) means no surgical treatment

      • early contrast enhancement on fat-saturated T1WI

    • blurring + widening of junctional zone secondary to obstruction of cervical os (retained secretions in uterine cavity)

    • disruption of hypointense vaginal wall by hyperintense thickening on T2WI

    • disruption of hypointense cervical fibrous stromal ring on T2WI by nodular / irregular tumor signal intensity

  • @ Parametrial invasion (best seen on T1WI):

    • low-intensity spiculated areas of soft tissue radiating from periphery of cervical mass

    • irregular lateral margins of cervix = linear stranding around cervical mass

    • thickening of uterosacral ligament

  • @ Pelvic sidewall invasion

    • tumor involves obturator internus, piriform, levator ani muscles

    • dilatation of ureter + hydronephrosis

  • @ Pelvic visceral disease

    • disruption of hypointense walls of bladder / rectum (DDx: hyperintense thickening of bladder wall on T2WI due to bullous edema)

  • @ Lymphatic spread

    • lymphadenopathy >10 mm, hyperintense compared with muscle / blood vessels on T2WI

Prognosis:

depending on tumor stage + volume of primary mass + histologic grade + lymph node metastases; in 30% recurrent / persistent disease (usually within 2 years)

• 5-year survival rate:

  tumor diameter 1 cm	84%
  tumor diameter >4 cm	67%
  stage IIB	65%
  stage III	40%
  stage IVA	<20%

  Rx:	(1) Surgery for stages <IIA / tumor <4 cm
  	(2) Radiation therapy chemotherapy for stages >IIB
  DDx:	(1) Endometrial polyp / adenocarcinoma (centered in endometrial cavity protruding into endocervical canal)
  	(2) Prolapsed submucosal fibroid (more hypointense on T2WI)

Recurrent Cervical Carcinoma

• = local tumor growth / development of distant metastasis 6 months after complete regression

• @ Pelvic recurrence

  Prevalence:	varies with stage, histologic type, adequacy of therapy, host response; 11% in stage IB
  Site:	cervix, uterus, vagina / vaginal cuff, parametria, ovaries, bladder, ureters, rectum, anterior abdominal wall, pelvic side wall

  • lower extremity swelling (lymphatic obstruction)

  • pain (nerve compression, ureteral obstruction)

  • hydrometra (obstruction by preserved cervix)

  • rectovaginal fistula

  • hydronephrosis (70% by autopsy)

  • vesicovaginal fistula

  • pelvic side wall mass

  DDx:

  radiation fibrosis (82% MR accuracy)

• @ Nodal recurrence

  • Prognosis worsens as nodal involvement progresses!

    • primary: paracervical, parametrial, internal + external iliac, obturator nodes (= medial group of the external iliac nodes)

      Frequency:

      in 75% of adenocarcinoma, in 61% of squamous cell carcinoma (autopsy)

    • secondary: sacral, common iliac, inguinal, paraaortic nodes

      Frequency:

      in 62% of adenocarcinoma, in 30% of squamous cell carcinoma (autopsy)

• @ Solid abdominal organ recurrence

  Location:

  liver (33%) > adrenal gland (15%) > spleen, pancreas, kidney

• @ Peritoneal recurrence

  • Peritoneal carcinomatosis (5 27% by autopsy)

  • Tumor deposits in mesentery + omentum

  • Sister Joseph nodule = umbilical metastasis developing from anterior peritoneal surface

• @ GI tract recurrence

  Location:

  rectosigmoid junction (17%), colon, small bowel

  • fistula formation

  • focal bowel wall thickening + tethering

  • intestinal obstruction (12% by autopsy)

  Prognosis:

  immediate cause of death in 7%

• @ Chest recurrence

  • Lung metastases (33 38% by autopsy)

  • Pleural metastases associated with hydrothorax

  • Pericardial metastasis

  • Lymphangitic carcinomatosis (<5%)

  • Mediastinal / hilar adenopathy + pleural lesions / effusion

• @ Osseous recurrence

  P.1033

  
  

  Prevalence:	15 29% by autopsy
  Location:	vertebra > pelvis > rib > extremity
  Mechanism:	direct extension from paraaortic nodes (most common) / lymphatic / hematogenous spread

• @ Skin + subcutaneous tissue recurrence (in up to 10%)

Chorioamnionic Separation

• normally seen <16 weeks

  • = incomplete fusion of amniotic membrane with chorionic plate

• abnormal >17 weeks MA

  • = secondary to hemorrhage / amniocentesis (10%)

  • membrane extends over fetal surface + stops at origin of umbilical cord

  • elevated membrane thinner than chorionic membrane

Cx:	rupture of amniotic membrane may lead to amniotic band syndrome
DDx:	amniotic band syndrome, uterine synechia, fibrin strand after amniocentesis, cystic hygroma (moves with embryo)

Chorioangioma

= benign vascular malformation of proliferating capillaries (= hamartoma)

Incidence:	1:3,500 to 1:20,000 births
Location:	usually near the umbilical cord insertion site

• well-circumscribed intraplacental mass with complex echo pattern protruding from the fetal surface of the placenta

• polyhydramnios (in 1/3)

• arterial signal on Doppler ultrasound in angiomatous chorioangioma

Cx:

hemorrhage, fetal hydrops, cardiomegaly, congestive heart failure, IUGR, premature labor, fetal demise (with large lesion)

Choriocarcinoma

Prevalence:	5% of gestational trophoblastic diseases
Age:	child-bearing age
Histo:	biphasic pattern including syncytiotrophoblastic + cytotrophoblastic proliferation without villous structures; extensive necrosis + hemorrhage; early + extensive vascular invasion

Preceded by:	mnemonic:	MEAN
	Mole (hydatidiform)	in 50.0%
	Ectopic pregnancy	in 2.5%
	Abortion, spontaneous	in 25.0%
	Normal pregnancy	in 22.5%

• continued vaginal bleeding

• continued elevation of hCG after expulsion of molar / normal pregnancy (25%)

• mass enlarging the uterus

• mixed hyperechoic pattern (hemorrhage, necrosis)

• Metastases:

  • hematogenous (usually): lung, kidney (10 50%), brain

    • radiodense pulmonary masses with hazy borders due to hemorrhage + necrosis

    • hyperechoic hepatic foci

  • lymphatic + direct extension (occasionally): vagina

Prognosis:	85% cure rate (even with metastases); fatal with spread to kidneys + brain
Rx:	(1) Chemotherapy: methotrexate, actinomycin D cyclophosphamide
	(2) Hysterectomy (if at risk for uterine rupture)

DDx:	mnemonic:	THE CLIP
		True mole
		Hydropic degeneration of placenta
		Endometrial proliferation
		Coexistent mole and fetus
		Leiomyoma (degenerated)
		Incomplete abortion
		Products of conception (retained)

Clear Cell Neoplasm of Ovary

• = mesonephroid TUMOR

• = almost always invasive carcinoma

Incidence:	2 5 10% of all ovarian cancers
Histo:	clear cells (cuboidal cells with clear cytoplasm) + hobnail cells (columnar cells with large nuclei projecting into the lumina of glandular elements); identical to clear cell carcinoma of endometrium, cervix, vagina, kidney; ~100% malignant
Not associated with:	in utero DES exposure (like lesions of the vagina + cervix)

• 75% of patients present with stage I disease

• frequently unilocular cyst + mural nodule(s)

Prognosis:

50% 5-year survival rate (better than for other ovarian cancers)

Conjoined Twins

• = incomplete division of embryonic cell mass in monozygotic twins occurring at 13 16 days GA but before the 3rd week of gestation

  Prevalence:

  1:50,000 to 1:200,000 deliveries (1:14,000 to 1:25,000 in Southeast Asia + Africa); 1:600 twin births; M:F = 1:3

• OB-US (diagnosed as early as 12 weeks GA):

  • single placenta without separating amniotic membrane (monochorionic, monoamniotic = hallmark of monozygotic twinning)

  • inseparable fetal bodies + skin contours:

    • fetuses commonly face each other

    • both fetal heads persistently at same level

    • no change in relative position of fetuses

    • bibreech (more common) / bicephalic presentation (cephalic-breech presentation is most common presentation for omphalopagus)

    • backward flexion of cervical spine (in anterior fusion)

    • single cardiac motion (if heart shared)

  • polyhydramnios (in almost 50%)

  • single umbilical cord with >3 vessels

  • fewer limbs than expected

• Associated malformations:

  • omphalocele

  • congenital heart disease (high frequency in all types of conjoined twinning)

Prognosis:

40 60% stillborn; 35% die within 24 hours of life

Craniopagus

= united at any part of the skull except face / foramen magnum (usually vertical / parietal in >60%)

P.1034

shared cranium, meninges, dural venous sinuses (brains commonly remain separate connecting bridge of neural tissue)

Conjoined Twins classified according to most prominent site of connection

Superior Conjunction

Dipygus (<1%)

single head, thorax, abdomen + two pelves and four legs

Syncephalus (<1%)

facial fusion thoracic fusion

Craniopagus (2%)

joined between homologous portions of cranial vault

Middle Conjunction

Thoracopagus (40%)

between thoracic walls; conjoined hearts (75%)

Omphalopagus (33%)

joined between umbilicus + xiphoid

Xiphopagus

joined at xiphoid

Rachipagus

joined at any level of spinal column above sacrum

Thoracoomphalopagus

Inferior Conjunction

Diprosopus (<1%)

two faces + one head and body

Dicephalus (<1%)

two heads + one body

Ischiopagus (6%)

joined by inferior sacrum and coccyx

Pygopagus (19%)

joined by posterolateral sacrum and coccyx

Incomplete Duplication (10%)

duplication of only one part of body

The more fused twins are usually joined laterally, whereas the more separate twins are joined anteriorly, posteriorly, cranially, and caudally!

Ischiopagus

• = united from umbilicus to large conjoined pelvis, face to face / end to end

• Types: tetrapus (4 legs), tripus (3 legs), bipus (2 legs)

• usually two sacra single symphysis pubis

• varying degrees of renal fusion + ectopia

• one / two urinary bladders

• single external urethral orifice (usually)

• shared sex organs (frequently born as females)

• lower GI tract usually shared with anal atresia + colovesical fistulas

• large pelvic vessel connecting both aortas

Omphalopagus

• = joined ventrally in umbilical region, often with inclusion of lower thorax

• liver fusion (80%)

• shared terminal ileum (join at Meckel diverticulum) + proximal colon (33%)

Parapagus

• = side-to-side position with ventrolateral fusion sharing umbilicus, abdomen, pelvis

  Types:

  dithoracic (= separate thoraces), dicephalic (= separate heads)

• conjoined pelvis with single symphysis pubis

• one / two sacra

• multiple other anomalies

Pygopagus

• = united dorsally sharing sacrococcygeal + perineal region

• fusion of sacral vertebrae (spinal cords usually separate)

• single anus single rectum

• single urinary bladder + urethra (15%)

Thoracopagus

• = united from upper thorax to umbilicus

• common sternum, diaphragm, upper abdominal wall

• common pericardial sac (90%) + some degree of cardiac fusion

• fusion of liver (invariably):

  • shared biliary system (in 25%)

  • absent / anomalous hepatic venous drainage

• common small intestine (in 50%): joins at duodenum + separates at distal ileum

Prognosis:

cardiac fusion precludes successful surgical separation in 75%

Cord Prolapse

• = prolapse of cord into endocervical canal

• Incidence: 0.5% at delivery

• Predisposing factors:

  • nonvertex fetal lie, polyhydramnios, cephalopelvic disproportion, multiple gestation, increased length of umbilical cord

• Cx: cord compression with high perinatal mortality N.B.: MEDICAL EMERGENCY! Alert obstetrician immediately!

• OB management:

  • Patient immediately placed into Trendelenburg / knee-elbow position in radiology department

  • Cesarean section for term infants

  • Expectant management for preterm infants

• DDx: Cord presentation (= umbilical cord between fetus and internal os)

Cystadenofibroma

= variant of serous cystadenoma, rarely malignant

Prevalence:	nearly 50% of all benign ovarian cystic serous tumors; bilateral in 6%
Age:	15 65 (mean 31) years

• may produce estrogen excess

• small multilocular cystic tumor

• clusters of short rounded papillary processes

Dermoid

• = DERMOID CYST = MATURE CYSTIC TERATOMA

• = congenital benign germ cell tumor containing mature tissues from all 3 germ cell layers with predominance of ectodermal component

P.1035

Incidence:	5 11 25% of all ovarian neoplasms; 20% of ovarian tumors in adults; 66 80% of pediatric ovarian tumors; Most common ovarian neoplasm!
Origin:	self-fertilization of a single germ cell after the first meiotic division (= random error in meiosis)
Path:	unilocular thin-walled cyst lined by an opaque gray-white wrinkled epidermis from which hair shafts protrude; lumen of cyst filled with sebaceous secretions mixed with hair strands
Histo:	mature epithelial elements (skin, hair, teeth, desquamated epithelium); cartilage; bone; muscle; bronchus; fat; salivary gland; neuronal tissue; pancreas; retina; may contain struma ovarii, carcinoid tumor
Age:	reproductive life (80%); age peak 20 40 years

• relatively soft pelvic mass (2/3) difficult to palpate

• pelvic pressure / pain due to torsion or hemorrhage

• Location: bilateral in 8 15 25%

• cystic mass with average diameter of 10 cm

• fat-fluid / hair-fluid level

• dermoid plug = Rokitansky nodule / protuberance = oval / round mural solid tissue mass (sebaceous material) of 10 65 mm projecting into cyst lumen

• Plain film (diagnostic in 40%):

  • tooth / bone

  • fat density (SPECIFIC)

• CT:

  • round mass of fat floating in interface between two water-density components (93%)

  • Rokitansky nodule = dermoid plug (81%) of adipose tissue, usually single, may be multiple

  • sebum-rich fat-fluid level in cyst cavity (12%)

  • globular calcifications (tooth) / rim of calcification (56%)

• US (77 87% sensitive):

  • complex mass containing echogenic components (66%):

    • tip of the iceberg sign = echogenic mass with dirty acoustic shadowing (= mixture of sebum + hair strands creates multiple tissue interfaces) in a predominantly cystic mass (25 44%) (DDx: stool-filled rectosigmoid)

    • fat-fluid level

  • predominantly solid mass (10 31%)

  • purely cystic tumor (9 15%)

  • echogenic focus with acoustic shadowing (due to calcification)

• MR:

  • cyst with fluid-fluid level:

    • hyperintense lipid-laden nondependent cyst fluid (above fluid of low signal intensity) on T1WI + of intermediate intensity on T2WI

    • in-phase GRE image:

      • dependent fluid hypointense compared with muscle

      • nondependent fluid slightly hypointense compared with surrounding fat

    • opposed-phase GRE image:

      • dependent fluid still hypointense compared with muscle

      • slightly hypointense compared with surrounding fat

      • thin black outline at interface between water and fat (phase cancellation of water + lipid in same voxel)

  • hyperintense mass (fat + serous fluid both with high signal intensity) on T2WI

  • chemical shift artifact of bright/ dark bands (along frequency-encoding gradient)

    Cx:	(1) Malignant degeneration in 1 3% (usually within dermoid plug of tumors >10 cm in diameter in postmenopausal women) into squamous cell carcinoma (most common)
    	(2) Torsion (4 16%)
    	(3) Rupture with chemical peritonitis (rare)
    	(4) Hydronephrosis
    Rx:	surgery (to avoid torsion / rupture)
    DDx:	tuboovarian abscess, acute hemorrhagic cyst, atypical endometrioma, bowel gas

Rupture of Ovarian Cystic Teratoma

Cause:

torsion, infarction, trauma, infection, malignant change, prolonged pressure during labor, idiopathic

• acute abdomen (due to severe chemical peritonitis)

• spilled sebaceous material / hair ball on T1WI + fat-suppressed T1WI (DDx to fluid)

• thickened peritoneum / intraperitoneal adhesions on contrast-enhanced fat-suppressed T1WI

  DDx:

  tubercular peritonitis, carcinomatosis

Diethylstilbestrol (Des) Exposure

= first reported transplacental carcinogen

@	Vagina:	adenosis, septa, ridges, clear-cell adenocarcinoma (in 1:1,000 women exposed in utero to DES, by age 35)
@	Cervix:	hypoplasia, stenosis, mucosal displacement, pseudopolyps, hooded / cockscomb appearance
@	Uterus:	hypoplasia, bands, contour irregularity, T- shaped uterus
@	Tubes:	deformity, irregularity, obstruction

Dysgerminoma

= malignant germ cell tumor of ovary homologous to testicular seminoma

Incidence:	0.5 2% of all malignant ovarian tumors
Peak age:	2nd 3rd decade

• no elevation of AFP / hCG (in 5% syncytiotrophoblastic giant cells present, which can elevate hCG levels)

Location:

usually unilateral; bilateral in 15 17% multilobulated solid mass divided by fibrovascular septa speckled pattern of calcifications (rare)

• MR:

  • hypo- / isointense septa on T2WI with contrast-enhancement on T1WI

• US:

  • hyperechoic solid mass, may have areas of hemorrhage + necrosis

  • prominent arterial color Doppler flow within septa

Rx:

highly radiosensitive

Eclampsia

= occurrence of coma pre-, intra-, or postpartum convulsions not related to a coincidental neurologic disorder in a preeclamptic patient

P.1036

• Pathophysiology:

  • VASOSPASM THEORY

    • overregulation of cerebral vasoconstrictive response to acute + severe hypertension progresses to vasospasm; prolonged vasospasm causes local ischemia, increased brain capillary permeability, disruption of blood-brain barrier, arteriolar necrosis, leading to cerebral edema + hemorrhage

  • FORCED-DILATATION THEORY

    • with severe arterial hypertension upper limit of cerebral autoregulation is reached + cerebral vasodilatation starts disrupting the blood-brain barrier and resulting in cerebral edema

  Time of onset:

  2nd half of pregnancy in primigravida; <20th week GA with trophoblastic disease

  • severe throbbing frontal headache

  • visual disturbance: scotomata, amaurosis, blurred vision

  • retinal / cortical blindness

  • hyperreflexia, hemi- / quadriparesis, confusion, coma

  • seizures: usually tonic-clonic

  • CT (positive in up to 50%):

    • bilateral rather symmetric white matter hypodensities without contrast enhancement

    • cerebral edema with compression of lateral ventricles

    • usually transient + completely reversible cerebral-cortical + basal ganglia hypodensities (= reversible ischemic lesions)

    • cerebral infarction in prolonged ischemia

    • intracerebral hemorrhage (major cause of mortality in 10 60%)

  • MR:

    • transiently increased T2-signal intensity in cerebral cortex + subcortical white matter frequently in watershed areas of posterior hemispheres

Ectopia Cordis

= fusion defect of anterior thoracic wall / sternum / septum transversum prior to 9th week of gestation

• THORACIC TYPE (60%)

  • = heart outside thoracic cavity protruding through defect in sternum

• ABDOMINAL TYPE (30%)

  • = heart protruding into abdomen through gap in diaphragm

• THORACOABDOMINAL TYPE (7%)

  • = in pentalogy of Cantrell

• CERVICAL TYPE (3%)

  • = displacement of heart into cervical region

• Associated with:

  • Facial deformities

  • Skeletal deformities

  • Ventral wall defects

  • CNS malformations: meningocele, encephalocele

  • Intracardiac anomalies: tetralogy of Fallot, TGA

  • Amniotic band syndrome

Prognosis:

stillbirth / death within first hours / death within first days of life in most case

Ectopic Pregnancy

= implantation outside the endometrial cavity

Incidence:	2% of all pregnancies in United States (1992); 9.9:10,000 women annually; 73,700 cases in 1986 in United States
Risk of recurrence:	10 15%

• Cause: delayed transit of the fertilized zygote (formed on day 14 MA) secondary to

  • abnormal angulation of oviduct

  • adhesions or scarring from inflammation

  • slowed tubal transit from ciliary abnormalities

• Risk factors:

  • Previous tubal surgery (tubal ligation / tuboplasty)

  • Previous PID (30 50%): esp. Chlamydia

  • In vitro fertilization / gamete intrafallopian tube transfer

  • Endometriosis

  • Previous ectopic pregnancy (prevalence up to 1.1%, 10-fold increase in risk, 25% chance of recurrence)

  • Current use of IUD

  • Advanced maternal age

  • In utero diethylstilbestrol exposure

  • Documented tubal anomaly

    • If the pregnancy cannot be documented as intrauterine, the patient should be considered at risk!

• Time of manifestation: usually by 7th week of MA

• CLASSIC CLINICAL TRIAD (<50%):

  • abnormal vaginal bleeding (75 86%)

  • pelvic pain (97%)

  • palpable adnexal mass (23 41%)

• secondary amenorrhea (61%)

• cervical motion tenderness

• positive urinary pregnancy test (50%)

• progesterone level <25 mg/mL

• -hCG does not rise >66% within 48 hours (lower levels + slower rise and decline compared with IUP)

  • Most ectopic pregnancies do not exhibit a -hCG of >6,500 mIU/mL (1st IRP) prior to symptomatology!

  • A -hCG level above the discriminatory zone with absence of IUP suggests ectopic pregnancy!

• Discriminatory zone of -hCG (at which a normal IUP should be visualized):

  • by transabdominal scan:

    • 6,500 mIU/mL (IRP) with 100% sensitivity + 96% specificity

  • by endovaginal scan:

    • 2,000 3,000 mIU/mL (IRP)

  Caveats:	technical quality of exam, multiple gestations, distortion by uterine cavity (leiomyoma), lab error, assay variation
  Dx:	diagnostic laparoscopy (3 4% false negative, 5% false positive)

  • Location:

    (a) tubal (95%):	(1) Ampullary ectopic (75 80%)
    	(2) Isthmic ectopic (10 15%)
    	(3) Fimbrial ectopic (5%)
    	(4) Interstitial ectopic (2 4%)
    (b) other (5%):	(1) Abdominal ectopic
    	(2) Ovarian ectopic (0.5 1%)
    	(3) Interligamentary ectopic
    	(4) Cervical ectopic (0.15%)

  • Spectrum:

    P.1037

    
    

    Type 1:	unruptured live ectopic + heartbeat
    Type 2:	early embryonic demise without rupture / embryonic structures / heartbeat
    Type 3:	ruptured ectopic with blood in pelvis
    Type 4:	no sonographic signs of ectopic

    

    Intrauterine Signs of an Ectopic Pregnancy

    Transvaginal US (6 20% false-negative rate):

    • Detected 1 week sooner than by transvesical US!

    • @ Uterus

      • absence of intrauterine pregnancy (beyond 6 weeks MA / with -hCG level >1,000 mIU/mL [2nd IRP])

        • No IUP by transvesical US = ectopic pregnancy in 43 46%

        • No IUP by endovaginal US = ectopic pregnancy in 67%

      • thickening of endometrium:

        • hyperechoic endometrial thickening (50%) due to hormonal stimulation from ectopic pregnancy

        • sloughing of endometrium = decidual cast (21%)

        • decidual endometrium lacks low-impedance blood flow

      • decidual cyst = 1 5-mm cyst at junction of endometrium and myometrium (14%)

      • pseudogestational sac (10 20%) = single parietal decidual layer surrounding an anechoic fluid collection in uterine cavity secondary to bleeding

    • @ Adnexa

      • tubal ring = extrauterine hypoechoic saclike structure (40 68%) 1 3 cm in diameter + surrounded by a 2 4-mm concentric ring

      • extrauterine mass of any type (84%):

        • solid / complex adnexal mass = clotted blood free in peritoneal cavity / hematosalpinx (36%)

        • extrauterine gestational sac without live embryo / yolk sac (35%)

        • embryonic heartbeat (6 28%) = PATHOGNOMONIC

      • echogenic tubal mass (89 100%)

        Probability of Ectopic Pregnancy without IUP or clinical symptoms of an ectopic pregnancy coupled with

        normal scan / simple cyst in adnexa	5%
        complex adnexal mass	92%
        tubal ring	95%
        live embryo outside uterus	100%

      • varying flow pattern depending on viability

      • corpus luteum within ovary in >50% on side of ectopic pregnancy (DDx: ectopic pregnancy)

    • @ Cul-de-sac

      • free fluid (40 83%): echogenic / particulate fluid

      • (= hemoperitoneum) has 93% positive predictive value for ectopic pregnancy

      • DDx: anechoic fluid in 10 27% of IUP

    • Doppler-US (low diagnostic impact):

      • high-velocity low-impedance flow around extrauterine gestation in 54% (up to 4 kHz shift with 3 MHz transducer, 0.38 0.2 Pourcelot index, RI = 0.18 0.58)

      • absence of peritrophoblastic flow after 36 days (<0.8 kHz shift with 3 MHz transducer or <1.3 kHz shift with 5 MHz transducer)

    • DDx of low-impedance flow:

      • corpus luteum cyst, tuboovarian abscess, fibroid

    • MR:

      • hematosalpinx slightly hyperintense relative to urine on fat-suppressed T1WI

      • bloody ascites

      • heterogeneous adnexal mass of mixed signal intensity on fat-suppressed T1WI + T2WI

      • extravasation of contrast material (= bleeding site) on contrast-enhanced dynamic subtraction MR

    Prognosis:	(1) 3.8:10,000 mortality rate (4% of all maternal deaths)
    	(2) Infertility (in 40%)
    Dx:	(1) Laparoscopy (almost 100% accurate)
    	(2) Culdocentesis (high probability for ectopic with aspiration of nonclotting blood with a hematocrit >15)
    Cx:	maternal death in 1:1,000; tubal rupture (10 15%)
    Rx:	(1) Surgery
    	(2) Methotrexate (>90% success rate)

    • Contraindications to methotrexate therapy:

      • -hCG level of > 5000 IU/L

      • Documented fetal cardiac activity on US

      • Ectopic mass of 3.5 cm

      • Hemodynamic instability

      • Unreliable patient who may not comply with serial -hCG testing

    • Complications of methotrexate therapy:

      • Selflimiting posttreatment pain

      • Treatment failure (severe pain, hemodynamic instability, tubal rupture, hemoperitoneum)

      • Increase in size of gestational mass (in up to 56%) up to 8 cm in diameter

      • Slow resolution of ectopic (up to 108 days)

    • DDx:

      • Hemorrhagic corpus luteum / hematoma

      • Adnexal mass: hydrosalpinx, endometrioma, ovarian cyst

      • Fluid-containing small bowel loop

      • Eccentrically placed GS in bicornuate / retroflexed / fibroid uterus

Abdominal Ectopic

• Frequency: 1:6,000 ectopic pregnancies

• >25% may be missed sonographically!

• bloating, abdominal pain (fetal movement / peritoneal irritation due to adhesions)

P.1038




• bleeding, hypotension, shock

• extrauterine location of fetus + placenta

• uterus compressed with visible endometrial cavity line

• absence of uterine wall between gestation + bladder / abdominal wall

• anhydramnios

Cx:

bowel obstruction / perforation; erosion of pregnancy through abdominal wall

Lithopedion

• = stone child = very rare obstetric complication consisting of a dehydrated + calcified demised fetus in an extrauterine pregnancy existing for >3 months without infection

• Types:

  • Lithokelyphosis = fetal membranes calcified

  • Lithokelyphopedion = fetus + membranes calcified

  • True lithopedion = only fetus calcified

Maternal age at discovery:	23 100 years of age; within 4 20 years of fetal demise
Location:	most common in adnexae

• large densely calcified mass in lower abdomen / upper pelvis

• CT scan reveals fetal skeleton

DDx:

uterine fibroid, calcified ovarian malignancy / cyst, sarcoma

Heterotopic Pregnancy

• = ectopic + coexistent intrauterine pregnancy

• Incidence: 1:6,800 30,000 pregnancies (higher number of coexisting ectopic with ovulation induction)

• An IUP does not preclude a complete pelvic ultrasound evaluation, although depiction of an IUP virtually excludes the diagnosis of an ectopic pregnancy!

Interstitial (Cornual) Ectopic (2 4%)

• = ectopic pregnancy with eccentric location in relation to endometrium + close to uterine serosa

• Often rupture late because of greater myometrial distensibility compared with other parts of tube!

• High likelihood of catastrophic hemorrhage + death due to abundant blood supply by both ovarian + uterine arteries!

Increased risk:

previous ipsilateral salpingectomy

• Baart de la Faille sign = broad-based palpable mass extending outward from uterine angle

• Ruge-Simon syndrome = fundus displaced to contralateral side with rotation of uterus + elevation of affected cornu

• eccentric heterogeneous mass in cornual region (66%)

• eccentrically placed gestational sac (25%)

• thinning of myometrial mantle to <5 mm (33%)

• interstitial line sign = thin echogenic line extending directly up to the center of ectopic pregnancy (= endometrial canal / interstitial portion of fallopian tube) in 92%

• myometrium between sac and uterine cavity

• large vascular channels + peritrophoblastic blood flow

• absence of double decidual sign

  Prognosis:	massive bleeding from erosion of uterine arteries + veins (pregnancy survives only 12 16 weeks GA); 2-fold mortality compared with other tubal ectopics
  DDx:	pregnancy within horn of bicornuate uterus; hydatidiform mole; degenerating uterine fibroid

Embryonic Demise

Incidence:

20 71% loss rate of one twin <10 weeks

Early Embryonic Demise / Failing Pregnancy

• -hCG level <2 3 standard deviations below the mean for given MA / GS size / CRL

• on endovaginal scan:

  • DEFINITE DEMISE

    • absence of cardiac activity with CRL of 5 mm / 6.5 weeks GA (repeat scan in 3 days for confirmation)

  • PROBABLY FAILING PREGNANCY

    • mean sac diameter of 16 mm without embryo

    • mean sac size of 8 mm without yolk sac (repeat scan in 3 days for confirmation)

    • >1,000 mIU/mL (1st IRP) without gestational sac

    • >7,200 mIU/mL (1st IRP) without yolk sac

    • >10,800 mIU/mL (1st IRP) without embryo

  • HIGH RISK OF SUBSEQUENT DEMISE

    • severe bradycardia <80 bpm

    • small mean gestational sac size (difference between mean sac size and CRL <5 mm is predictive of miscarriage in 94%)

  • MODERATELY HIGH RISK OF DEMISE

    • bradycardia of 80 90 bpm

    • large subchorionic hematoma lifting much of placenta

    • yolk sac >6 mm / abnormal shape

• mean gestational sac size too small for good clinical dates

• gestational sac growth 0.7 mm/day (normal growth rate of 1.13 mm/day determines appropriate time interval for follow-up scan, ie, when sac is expected to be 27 mm)

• sac position in lower uterine segment / cervix

• stringlike / granular debris / fluid-fluid level within gestational sac (= intrasac bleeding)

Late Embryonic Demise

• on endovaginal scan:

  • wrinkled collapsing amniotic membrane

  • irregular distorted shape of gestational sac (DDx: compression by bladder, myoma, contraction)

  • absence of double decidual sac = thin (<2 mm) weakly hyperechoic / irregular choriodecidual reaction

Endodermal Sinus Tumor of Ovary

• = YOLK SAC TUMOR

• = rare but highly malignant tumor

• Histo: resembles endodermal sinuses of the rat yolk sac

  • papillary pattern (most common): contains glomerular structures with central vessel + peripheral mantling of epithelial cells (= Schiller-Duval bodies)

  • others: reticular, solid, polyvesicular vitelline

    • periodic acid-Schiff reaction

    • -fetoprotein positive hyaline globules

P.1039

Incidence:	<1% of all ovarian carcinomas
Age:	usually adolescence
May be associated with:	teratoma, dermoid cyst, choriocarcinoma

• frequently abdominal enlargement + pain

• elevated serum AFP (common)

• predominantly echogenic solid tumor

• cystic areas (epithelial-lined cysts / cysts of coexisting mature teratoma / hemorrhage / necrosis)

• bilateral in 1%

Rx:	surgery + combination chemotherapy
Prognosis:	poor

Endometrial Cancer

Most common invasive gynecologic malignancy; 4th most prevalent female cancer in USA women

Incidence:	34,000 new cases per year with 3,000 deaths
Histo:	adenocarcinoma (90 95%), sarcoma (1 3%)
Peak age:	55 62 years; 74% > age 50
Risk factors:	nulliparity, late menopause, exposure to unopposed estrogen therapy, polycystic ovaries, obesity, hypertension, diabetes mellitus

• Histo:

  • endometrioid carcinoma (75% of all cancers)

  • serous, mucinous, clear cell carcinoma (less common): similar to ovarian counterpart

  • squamous (rare): associated with cervical stenosis, pyometra, chronic inflammation

  • mixed mesodermal tumor: contains elements of epithelial + mesenchymal differentiation

Lymph node metastases:

3% with superficial invasion; 40% with deep invasion

• postmenopausal bleeding without hormonal therapy

Location:

predominantly in uterine fundus; 24% in isthmic portion)

• US:

  • normal-sized / enlarged uterus

  • focal / diffuse endometrial thickening (mean AP bilayer thickness of 18.2 mm)

    Endometrial Cancer

    FIGO stage	Description
    0	In situ
    I a	Tumor limited to endometrium
    I b	Superficial invasion to <50% of myometrium
    I c	Deep invasion to more than half of myometrium
    II a	Endocervical glandular involvement only
    II b	Cervical stromal invasion
    III a	Invasion of serosa / adnexa / peritoneal metastases
    III b	Vaginal metastases
    III c	Metastases to pelvic / paraaortic lymph nodes
    IV a	Invasion of bladder / bowel mucosa
    IV b	Distant metastases (lung, brain, bone) including intraabdominal / inguinal lymph nodes
    Clinical staging with dilatation & curettage inaccurate in up to 51%!

    • any endometrial thickness >5 mm is suspicious (100% negative predictive value, not very specific):

      • 10% cancer rate with endometrial thickness of 6 15 mm

      • 50% cancer rate with endometrial thickness of >15 mm

  • irregular heterogeneous echogenic texture with hypoechoic areas:

    • irregular poorly defined endometrial-myometrial interface (= invasive endometrial cancer)

    • increased echogenicity in myometrium (= invasive endometrial cancer)

  • intrauterine fluid collection (DDx: cervical stenosis)

• Transvaginal US:

  • apparent distension of endometrial lumen with extrinsic thinning of the myometrium (polypoid tumor)

  • Doppler pulsatility index of <1.5 or resistive index <0.7 suggest malignancy (DDx: endometritis, benign endometrial polyp)

  • areas of venous flow (DDx: endometrial hyperplasia)

• MR (82 92% accuracy for staging, 74 87% accuracy for depth of invasion):

  • endometrial cancer has slightly lower signal intensity than endometrium but higher than myometrium on T2WI

  • endometrial thickness abnormal if >3 mm (postmenopausal woman) / >10 mm (under estrogen replacement)

    DDx:

    blood clot, uterine secretions, adenomatous hyperplasia, submucosal leiomyoma

  • disruption / absence of junctional zone (myometrial invasion)

  • hyperintense areas penetrating into myometrium (deep muscle invasion; 74 87% accuracy)

Endometrioid Carcinoma of Ovary

Incidence:	8 15% of all ovarian cancers; 2nd most common malignant ovarian neoplasm (after serous adenocarcinoma)
Associated with:	hyperplasia / carcinoma of the uterine endometrium in 20 33%
Path:	malignant mixed mesodermal tumor = carcinoma-sarcoma is grouped with endometrioid cancer
Histo:	tubular glandular pattern with a pseudostratified epithelium resembling endometrial adenocarcinoma / metastatic colon carcinoma; ~100% malignant

• solid / complex (= cystic + solid) tumor

• bilateral in 15% of stage I cases

Prognosis:

better than serous / mucinous carcinomas

Endometriosis

• = ENDOMETRIOSIS EXTERNA

• = encysted functional endometrial epithelium + stroma in an ectopic site outside the uterine cavity / myometrium (internal endometriosis within uterus = adenomyosis)

  Prevalence:

  5 10% of menstruating women; in 5% of postmenopausal women on estrogen replacement therapy

• Etiology:

  • Metastatic theory:

    • peritoneal implantation of endometrial cells via retrograde menstruation through fallopian tubes

      • Up to 90% of women have bloody peritoneal fluid during perimenstrual period

      P.1040

      
      

      • Obstructive m llerian duct anomalies are the most common cause in girls <17 years of age

    • vascular + lymphatic spread

    • intraoperative implantation (uterine surgery, amniocentesis, needle biopsy)

  • Metaplastic theory: transformation of peritoneal epithelium into functioning endometrial tissue

  • Induction theory: combination of first two

    Mean age:

    25 29 years

• Path:

  • punctate small foci / stellate patches of <2 cm initiating inflammatory response (as organizing hemorrhage, fibrosis, adhesions)

  • endometrioma (in up to 10%) = endometriotic cyst in ovary containing thick dark degenerated blood products = chocolate cyst (due to repeated cyclic hemorrhage), in up to 50% bilateral

    Histo:

    endometrial glands, stroma, rare smooth muscle fibers; secretory changes during 2nd half of menstrual cycle; stromal decidualization during pregnancy

• infertility:

  • 20% of infertile women have endometriosis

  • 30 50% of women with endometriosis are infertile

    Cause:

    involvement of tubes + ovaries (peritubal adhesions causing anatomic distortion, impaired tubal mobility to capture ovum, tubal destruction / occlusion)

• pelvic pain:

  • 24 33% of women with pelvic pain have endometriosis

  • dysmenorrhea, dyspareunia, back pain, rectal discomfort

  • chronic pelvic pain (peritoneal adhesions, bleeding)

• localized tenderness along uterosacral ligaments + cul-de-sac + adnexa

• thickened nodular ligaments + rectovaginal masses

• fixed pelvic organs during bimanual exam

Location:

ovaries (80%) > uterosacral ligaments > pouch of Douglas > uterine serosal surface > fallopian tube > rectosigmoid

• Morphologic types:

  • Diffuse form (70%)

    • often no detectable abnormality (when lesions small + scattered)

    • frequently multiple cysts bilaterally

    • thickened wall + loss of definition of borders of pelvic organs

    • any combination of signal intensities

  • Discrete pelvic mass

    Histo:

    obliterated mostly endometrial gland lining; initially thin wall that becomes fibrotic + thickened with irregular external border

    • Multiplicity favors the diagnosis of endometrioma

    • US:

      • unilocular endometrioma = cyst of up to 20 cm in diameter (usually 2 5 cm):

        • acoustic enhancement

        • diffuse homogeneous low-level internal echoes (= hemorrhagic debris) in 95% CLASSIC

        • anechoic cyst (rare)

        • may show fluid-fluid / fluid-debris level (due to layering of debris)

        • echogenic wall foci (= cholesterol deposits) in 35% CLASSIC

        • wall nodularity in 20%

        • may contain echogenic material (= blood clot) appearing as a solid tumor floating dependently within cyst cavity

      • multilocular endometrioma = multiple separate cysts:

        • thin / thick septations between loculi

      • hematosalpinx (in 28%)

      • DDx:

        • Hemorrhagic ovarian cyst (acute symptoms, more complex cyst with clot retraction, thin fibrin stranding, resolution in 4 6 weeks)

        • Dermoid cyst (calcification, fat-fluid level, hyperechoic areas)

        • Cystic neoplasm

        • Tuboovarian abscess

      • CT:

        • varied findings including solid appearance

      • MR (91 96% accurate, 90 92% sensitive, 91 98% specific with fat suppression):

        • MR is MORE SPECIFIC than US or CT!

        • typically homogeneously hyperintense cyst(s) on T1WI similar to fat (intra- and extracellular methemoglobin shortens T1 of fluids)

        • hyperintense on fat-suppressed T2WI (effectively excluding a dermoid cyst)

          DDx:

          hemorrhagic adnexal cyst (solitary, thin-walled, brighter than endometrioma)

        • shading sign = faint / complete loss of signal of entire cyst / in dependent fluid-debris layer on T2WI (high concentration of cross-linked proteins + iron from recurrent hemorrhage results in a decrease in T2-relaxation time)

        • atypically hypointense on all pulse sequences in 27%

        • hypointense thick fibrous wall (DDx: PID)

        • multilocularity + multiplicity

        • adhesion to surrounding organs, eg, bowel tethering and sharp angulations

    • Cx:

      • Adhesions

        • fixed pelvic organs (during bimanual US)

        • obscuration of organ interfaces

        • posterior displacement of uterus (retroverted uterus) and ovaries

        • angulation of bowel loops

        • elevation of posterior vaginal fornix

        • loculated fluid collection

        • hydrosalpinx

      • Malignant transformation (<1%)

        • endometrioid carcinoma > clear cell carcinoma

          Dx:	laparoscopy / surgery
          Rx:	(1) Expectant
          	(2) Hormonal therapy (for pelvic pain / dyspareunia) to create a state of pseudopregnancy /pseudo-menopause / chronic anovulation: danocrine (Danazol ), GRH agonist (Lupron ), oral contraceptive pills
          	(3) Surgery (for infertility / intractable pain): implant recurrence in 28% by 18 months + in 40% by 9 years; adhesion recurrence in 40 50%

P.1041

Atypical Sites of Endometrial Implantation

• @ GI tract (12 37%)

  • catamenial diarrhea, constipation

  • rectal pain / bleeding

  Path:	initially serosal endometriotic deposits that erode into bowel wall causing hypertrophy + fibrosis of muscularis propria
  Location:	inferior margin of sigmoid colon + anterior wall of rectosigmoid (72%); rectovaginal septum (14%); distal ileum (7%); cecum (4%); appendix (3%); occasionally multiple lesions

  • single extramucosal mass with crenulated / spiculated mucosal pattern (DDx: drop metastasis)

  • polypoid intraluminal mass / annular constricting lesion (rare appearance)

    Cx:

    adhesion, bowel stricture, GI obstruction

• @ GU tract (20%)

  • urgency, frequency, hematuria

  • urinary obstruction, flank pain

  Path:

  initially serosal endometriotic deposits that may infiltrate into bladder / ureteral wall

  • mass projecting into bladder lumen typically at dome of bladder (DDx: bladder cancer)

  • smooth / tapered / angulated short to medium-length ureteral stricture near inferior aspect of sacroiliac joint

• @ Chest

  • = THORACIC ENDOMETRIOSIS SYNDROME

  • pleuritic chest pain, cyclic hemoptysis

    Cause:	microembolization (via lymphatics or vascular channels), peritoneal-pleural migration (through diaphragmatic defects)
    Time of onset:	5 years after pelvic endometriosis

  • presenting symptoms:

    • pneumothorax (73%), hemothorax (14%)

    • hemoptysis (7%), lung nodules (6%)

  • almost exclusively right-sided pleural lesions

  • bilateral lung nodules

  • catamenial pneumothorax

  • pleural effusion

• @ Cutaneous Tissue

  • palpable mass catamenial bleeding

  • focal pain / tenderness associated with menses

  Location:

  laparotomy scar, cervical biopsy / electrocautery, episiotomy scar, umbilicus

  • well-defined hypoechoic / cystic / solid mass

  • hyperechoic border / tissue stranding (due to inflammatory reaction)

  DDx:

  abscess, hematoma, hernia, sebaceous cyst, lipoma, hemangioma, malignant tumor

• @ CNS

  • cyclic headaches, seizures

  • subarachnoid hemorrhage

Ruptured Ovarian Endometrioma

= uncommon acute complication

• acute abdomen (due to chemical peritonitis)

  • distorted shape of endometrioma

  • thinned irregular wall component = rupture site

  • markedly hyperintense fluid in free intraperitoneal space on fat-suppressed T1WI

    Rx:

    emergency surgery

Facial Clefting

Incidence:	0.5:1,000 in Blacks; 1:1,000 live births in white population; 1.5:1,000 in Asians; 3.6:1,000 in American Indians; 13% of all congenital anomalies; second most common congenital malformation; most common craniofacial malformation
Normal embryology:	1st branchial arch develops into maxillary + mandibular prominences; by 5th week the stomadeum is surrounded by 5 prominences: frontal-nasal, paired maxillary, paired mandibular prominences; nasal pits are formed by invagination of nasal placodes on each side of frontal-nasal prominence; the 2 maxillary prominences grow medially to fuse with the 2 medial nasal prominences forming the upper lip; the lateral nasal prominences form the nasal alae
Risk of recurrence:	4% with one affected sibling, 17% with one affected sibling + parent

Median Facial Cleft

• = failure of fusion of the 2 medial nasal prominences

• Incidence: rare

• Cause:

  • median cleft face syndrome = frontonasal dysplasia

    • brain anomalies rare

  • Holoprosencephaly

  • Majewski syndrome (short rib, polydactyly, median cleft)

Lateral Facial Cleft

Cleft Lip (25%)

Cause:	lack of fusion of maxillary prominence with medial nasal prominence (= intermaxillary segment) around 7th week MA
Associated with:	anomalies in 20% (most frequently clubfoot); NO chromosomal anomalies
Site:	isolated in 8%, bilateral in 20%

• linear echo-poor region extending from one side of fetal upper lip into nostril

Prognosis:

excellent

Cleft Lip & Palate (50%)

Cause:	incomplete fusion of lip + primary palate with secondary palate
Associated with:	72 abnormalities in 56 80%: most frequently polydactyly; chromosomal anomalies in 20 33%
Location:	L > R
Site:	unilateral in 23%, bilateral in 30%

• linear defect extending through alveolar ridge + hard palate reaching the floor of the nasal cavity / orbit (often deeper + longer cleft than in isolated cleft lip)

P.1042




• paranasal echogenic mass inferior to nose (= premaxillary protrusion of soft tissue + alveolar process + dental structures) in bilateral cleft lip + palate

  

  Facial Clefting

Cleft Palate (25%)

= lack of fusion of mesenchymal masses of lateral palatine processes around 8th 9th weeks MA

Associated with:

anomalies in 50% (most frequently clubfoot + polydactyly)

• often missed on prenatal sonograms

• small fetal stomach + polyhydramnios (due to impaired fetal swallowing)

Fetal Cardiac Dysrhythmias

Normal heart rate:

120 160 bpm

Premature Atrial Contractions

= PAC = most common benign rhythm abnormality

• transient tachycardia

• transient bradycardia (due to atrial bigeminy if every other beat is nonconducted)

Cx:	supraventricular tachycardia (unusual)
Rx:	discontinue smoking, alcohol, caffeine
Follow-up:	biweekly auscultation until arrhythmia resolves

Supraventricular Tachyarrhythmia

Incidence:	1:25,000; most frequent tachyarrhythmia in children
Etiology:	viral infection, hypoplasia of sinoatrial tract

• Pathogenesis:

  • Automaticity = irritable ectopic focus discharges at high frequency

  • Reentry = electric pulse reentering the atria inciting new discharges

• Types:

  • Supraventricular tachyarrhythmia (SVT)

    • paroxysmal supraventricular tachycardia

    • paroxysmal atrial tachycardia

      • atrial rate of 180 300 bpm + ventricular response of 1:1

  • Atrial flutter

    • atrial rate of 300 460 bpm + ventricular rate of 60 200 bpm

  • Atrial fibrillation

    • atrial rate of 400 700 bpm + ventricular rate of 120 200 bpm

• Hemodynamics:

  • fast ventricular rate results in suboptimal filling of heart chambers + decreased cardiac output, overload of RA, CHF

• Associated with:

  • cardiac anomalies (5 10%): ASD, congenital mitral valve disease, cardiac tumors, WPW syndrome, cardiomyopathy

  • thyrotoxicosis

• OB-US:

  • M-mode echocardiography with simultaneous visualization of atrial + ventricular contractions allows inference of atrioventricular activation sequence

Cx:	congestive heart failure + nonimmune hydrops
Rx:	Intrauterine pharmacologic cardioversion (digoxin, verapamil, propranolol, procainamide, quinidine)

Atrioventricular Block

Incidence:	1:20,000 live births; in 4 9% of all infants with CHD
Etiology:	(1) Immaturity of conduction system
	(2) Absent connection to AV node
	(3) Abnormal anatomic position of AV node

• Associated with:

  • Cardiac structural anomalies (45 50%): corrected transposition, univentricular heart, cardiac tumor, cardiomyopathy

  • Maternal connective tissue disease: lupus erythematosus

• Types:

  • First-degree heart block = simple conduction delay

    • normal heart rate + rhythm (not reportedly diagnosed in utero)

  • Second-degree heart block

    • Mobitz type I

      • = progressive prolongation of PR interval finally leading to the block of one atrial impulse (Luciani-Wenckebach phenomenon)

      • a few atrial contractions are not followed by a ventricular contraction

    • Mobitz type II

      • = intermittent conduction with a ventricular rate as a submultiple of the atrial rate (eg, 2:1 / 3:1 block)

      • atrial contraction not followed by ventricular contraction in a constant relationship

  • Third-degree heart block = complete heart block

    • = complete dissociation of atria + ventricles

    • slow atrial + ventricular contractions independent from each other

Cx:

decreased cardiac output + CHF

Fetal Death in Utero

• = INTRAUTERINE DEMISE

• = fetal death during 2nd + 3rd trimesters

P.1043




• Specific signs:

  • absent cardiac / somatic motion

• Nonspecific signs seen not before 48 hours after death:

  • same / decreased BPD measurement compared with prior exam

  • development of dolichocephaly

  • Spalding sign = overlapping fetal skull bones

  • distorted fetus without recognizable structures

  • skin edema (epidermolysis) = fetal maceration

  • increased amount of echoes in amniotic fluid (= fetal tissue fragments)

  • gas in fetal vascular system

Vanishing Twin

= disappearance of one twin in utero due to complete resorption / anembryonic pregnancy

Incidence:	13 78% (mean 21%) before 14 weeks GA
Time:	<13 weeks MA

• NO sonographic evidence of twin pregnancy later in pregnancy

Fetus Papyraceus

• = compression + mummification of fetus

  Time:	in 2nd trimester
  Path:	resorption of fluid resulting in paperlike fetal body + compression into adjacent membranes

• compressed mummified fetus plastered against uterine wall

• Risk to surviving twin:

  • Dichorionic gestation (minimal risk)

    • Premature labor

    • Obstruction of labor by macerated fetus

  • Monochorionic gestation

    • DIC in response to release of thromboplastin from degenerating fetus

      • into maternal circulation

      • into twin fetus through shared circulation (= twin embolization syndrome)

Fetal Hydrops

Nonimmune Hydrops

• = excess of total body water evident as extracellular accumulation of fluid in tissues + serous cavities without antibodies against RBC

• Incidence: 1:1,500 to 1:4,000 deliveries

• Cause:

  • Cardiac anomalies (40%):

    • structural heart disease (25%): AV septal defect, hypoplastic left heart, rhabdomyoma

    • tachyarrhythmia (15%)

  • Hematologic causes: thalassemia, hemolysis, fetal blood loss

  • Idiopathic (25 44%)

  • Twin-twin transfusion (20%)

  • Chromosomal abnormalities (6%): Turner syndrome

  • Skeletal dysplasias: achondroplasia, achondrogenesis, osteogenesis imperfecta, thanatophoric dwarfism, asphyxiating thoracic dysplasia

  • Renal disease (4%): congenital nephrotic syndrome

  • Infections: toxoplasmosis, CMV, syphilis, Coxsackie virus, parvovirus

  • Cervical tumors: teratoma

  • Chest masses: cystic adenomatoid malformation, extralobar sequestration, mediastinal tumor, rhabdomyoma of heart, diaphragmatic hernia

  • Abdominal masses: neuroblastoma, hemangioendothelioma of liver

  • Placental tumors: chorioangioma

Prognosis:

46% death in utero; 17% neonatal death

Immune Hydrops

• = ERYTHROBLASTOSIS FETALIS

• = lysis of fetal RBCs by maternal IgG antibodies

  Prevalence:

  35:10,000 live births at risk

• Pathophysiology:

  • rh-negative women (= no D antigen) may become isoimmunized (= alloimmunization) if exposed to paternally derived fetal Rh-positive blood inherited from the father (= D allotype present); maternal IgM antibodies develop initially, later IgG antibodies with ability to cross placenta (= transplacental passage)

• Cause of isoimmunization:

  • fetomaternal hemorrhage during pregnancy / delivery / spontaneous or elective abortion if fetus is D-positive; fetus has a 50% chance of being rh-negative as 56% of RhD-positive fathers are heterozygous for D antigen

• At risk:

  • Caucasians (15%), Blacks (6%), Orientals (1%); absence of D antigen originates in Basques

• Determination of extent of disease by:

  • Optical density shift at 450 nm (= delta OD 450) reflects amount of bilirubin in amniotic fluid; reasonably reliable only >25 weeks MA; unreliable in alloimmunization due to Kell antibodies

  • Percutaneous umbilical cord sampling (PUBS) with direct determination of Hct and Hb

• hemolysis + anemia

• anasarca (= skin edema)

• fetal ascites in 2nd trimester (indicates severe anemia with Hct <15%, Hb <4 g/dL; present in only 66%)

• pleural effusion

• increased diameter of umbilical vein

• subcutaneous edema (skin thickness >5 mm)

• polyhydramnios (75%)

• placentomegaly >6 cm

• pericardial effusion

• hepatosplenomegaly

• increased blood flow in middle cerebral artery (due to increased cardiac output + decline in blood viscosity)

• Prophylaxis:

  • Rh immune globulin (RhoGAM = antibody against D antigen) blocks antigen sites on Rh-positive cells in maternal circulation to prevent initiation of maternal antibody production; Rh immune globulin given at 28 weeks to all rh-negative women

• OB management:

  • regular monitoring from 18 weeks on when maternal anti-D concentration exceeds 4 IU/mL (severe anemia unlikely if maternal antibodies <15 IU/mL)

P.1044

Prognosis:	(if untreated) 45 50% mild anemia, 25 30% moderate anemia (with neonatal problems only), 20 25% develop hydrops (death in utero / neonatally)
Rx:	umbilical vein transfusion during PUBS (necessary in only 10% before 34 weeks GA)

Fetal Trauma

Incidence:	7% of pregnant patients sustain accidental injury (greatest frequency during 3rd trimester); 0.3 0.4% are admitted to a hospital
Cause:	motor vehicle accident (66%), physical abuse (10%)

• Type of injury to pregnancy:

  • Uterine rupture (0.6%)

  • Complete (6 66%) / incomplete (30 80%) placental separation

• US:

  • evaluate fetal motion, breathing, heart rate, placenta

• The major cause of fetal death is maternal death!

Gartner Duct Cyst

Frequency:	1 2%
Origin:	remnant of vaginal portion of mesonephric / wolffian duct with incomplete involution + persistent glandular secretion
Histo:	lined by flat cuboidal / columnar epithelium
May be associated with:	complex renal + urogenital malformations

• Herlyn-Werner-Wunderlich syndrome = ipsilateral renal agenesis + ipsilateral blind vagina

• Ectopic ureter inserting into Gartner duct cyst

• usually asymptomatic

Location:

anterolateral aspect of proximal third of vaginal wall extending into ischiorectal fossa

• well-defined round lesion with fluid contents

• large cysts may displace ureter upward / protrude through introitus

Cx:

dyspareunia; interference with vaginal delivery

Gastroschisis

= paramedian full-thickness abdominal fusion defect usually on right side of umbilical cord; may involve thorax; bowel is nonrotated and lacks secondary fixation to dorsal abdominal wall

Incidence:

1 2:10,000 live births (same as omphalocele), sporadic

• Cause:

  • abnormal involution of right umbilical vein resulting in rupture of anterior abdominal wall at area of weakness

  • premature interruption of right omphalo-mesenteric artery (normally persists proximally as superior mesenteric artery) resulting in ischemic damage to abdominal wall

Age of occurrence:	37 days (5 weeks) of embryonic life
Age of detection:	difficult <20 weeks GA due to small size of defect (1 3 cm) + lack of bowel dilatation

• Associated anomalies (5%):

  • intestinal atresia / stenosis (25%; small size of opening leads to compression or torsion of vessels); ectopia cordis (rare)

• MS-AFP 2.5 MoM in 77 100%

• exteriorized bowel = thick-walled edematous freely floating loops outside fetal abdomen (due to lack of peritoneal covering)

• dilated intra- / extraperitoneal bowel

• <2 5 cm paraumbilical defect, usually on right side of cord insertion

• normal insertion of umbilical cord

• no fetal ascites

• polyhydramnios may be present

• liver / spleen may herniate infrequently

• malrotation / nonrotation of bowel

• Cx before birth:

  • Bowel obstruction

  • Peritonitis (exposure of bowel to fetal urine / meconium)

  • Perforation (from peritonitis)

  • Fetal growth restriction (38 77%) secondary to nutritional loss from exposed bowel

• Cx after birth:

  • malrotation, jejunal / ileal atresia (18%), bowel necrosis, necrotizing enterocolitis, hyperalimentation hepatitis, prolonged intestinal motility dysfunction, chronic short-gut syndrome

Mortality rate:	17%
Survival rate:	87 100% after surgical treatment (during 1st day of life, not influenced by mode of delivery); death from premature delivery / sepsis / bowel ischemia

Germ Cell Tumor of Ovary

= malignant (except for mature teratoma) ovarian tumors of varying histology

Age:

14 years on average

• pelvic / abdominal pain + mass

• elevated alpha-fetoprotein (60% in immature teratoma; 100% in endodermal sinus tumor)

• elevated -hCG (30% of endodermal sinus tumors)

• average diameter of 15 cm

• unilateral, rarely bilateral

• calcifications (40%)

• homogeneously solid (3%), predominantly solid (85%), predominantly cystic (12%)

Gestational Trophoblastic Disease

• = group of disorders as a result of an aberrant fertilization event arising from trophoblastic elements of the developing blastocyst with invasive tendency

• Components of trophoblast:

  • Cytotrophoblast = stem cell with high mitotic activity

  • Syncytiotrophoblast = synthesis of -hCG

  • Intermediate trophoblast = responsible for endometrial invasion + implantation

• increased levels of -hCG

Incidence:	<1% of all gynecologic malignancies
Associated with:	molar pregnancy (most), post abortion, ectopic pregnancy, term pregnancy
Spectrum:	1. Benign hydatidiform mole (80 90%)
	2. Invasive mole (5 8 10%)
	3. Choriocarcinoma (1 2 5%)
	4. Placental site trophoblastic tumor (rare)

P.1045

• Cytogenesis:

  • = fertilization of one egg by two sperm = chromosomes completely / predominantly of paternal origin

  • Diploid karyotype

    • 46, XX = from fertilization of ovum by two 23, X sperm after loss of maternal haploid chromosomes

    • 46, XY = from fertilization of a chromosomally empty ovum by two different sperm:

      • in complete hydatidiform mole (almost 100%), invasive mole (almost 100%), choriocarcinoma (50%)

  • Triploid karyotype (69, XXX; 69 XXY; 69, XYY)

    • = fertilization of a normal ovum (23, X) by 2 different sperm thus containing 2/3 paternal chromosomes

      • occurs in partial hydatidiform mole

        At risk:

        maternal age >35 years and <20 years, previous molar gestation, previous spontaneous abortions

Granulosa Cell Tumor

= most common hormone-active estrogenic tumor of ovary

Incidence:	1 2 3% of all ovarian neoplasms
Origin:	from cells surrounding developing follicles
Age:	puberty (5%), reproductive age (45%), postmenopausal (50%)

• Path:

  • large encapsulated smooth / lobulated multicystic mass with thick irregular septa + solid components; multiple blood-filled cysts (due to hemorrhage / cystic degeneration when tumor gets larger)

  • unilocular cyst (rare) apt to manifest with virilization

• abdominal pain (acute hemorrhage into tumor / rupture into peritoneal cavity)

• palpable adnexal mass

Location:

unilateral in 90 95%; bilateral in 5%

• multilocular mass containing cystic spaces with fluid / blood (most frequently) + solid components:

  • thick irregular septations but NO intracystic papillary projections

  • usually large with sizes up to 40 cm in diameter

• predominantly solid small hypoechoic mass simulating fibroid (less common)

• calcifications (rare)

• MR:

  • hyperintense on T1WI (hemorrhage within tumor cysts)

  • spongelike appearance of mostly solid intermediate signal intensity with innumerable cystic spaces

• Estrogenic effects:

  • uterine enlargement

  • endometrial thickening / hemorrhage

  • endometrial hyperplasia / polyps / carcinoma

• Pattern of dissemination:

  • local extension

  • peritoneum-based metastases similar to epithelial neoplasm (rare)

  • cystic liver metastases

Rx:	uni- / bilateral salpingo-oophorectomy postoperative chemotherapy
DDx:	serous / mucinous ovarian tumor (intracystic papillary projections)

Adult Granulosa Cell Tumor

• Most common estrogenic ovarian tumor! (occasionally produces androgen)

Incidence:	95% of all GCT; 5 10% of solid ovarian tumors
Age:	in peri- and postmenopausal women; peak prevalence at 50 55 years
Histo:	macrofollicular (multiple cysts resembling follicles), microfollicular (with Call-Exner bodies), insular, trabecular, cylindromatous, watered silk, diffuse type; frequently accompanied by theca cells + fibroblasts

• irregular menstruation cycles, menorrhagia

• postmenopausal bleeding

• amenorrhea

• 90% present at stage I (confined to ovary)

Cx:	(1) Malignant transformation (5 25%)
	(2) Low-grade endometrial carcinoma (3 25%)
	(3) Recurrence (raised serum aromatase + estradiol levels)
Recurrence:	common even decades after resection with slow growth

Juvenile Granulosa Cell Tumor

Incidence:	5% of all GCT; more common than adult GCT in patients <30 years old
Mean age:	13 years; 3% in women >30 years of age
Histo:	larger cells with hyperchromatic nuclei + lack of characteristic nuclear groves (compared with adult GCT)
Associated with:	Ollier disease, Maffucci syndrome

• sexual pseudoprecocity (estrogen effect without ovulation)

• GCT accounts for 10% of cases of precocious puberty

Cx:	malignant degeneration (rare)
Prognosis:	80 93% cure rate after surgery
Recurrence:	unusual after simple resection for stage Ia / Ib tumors

Hellp Syndrome

= Hemolysis, Elevated Liver enzymes, Low Platelets

Prevalence:	4 12% of patients with severe preeclampsia / eclampsia; higher in White women (24%), with delayed diagnosis of preeclampsia / delayed delivery (57%), in multiparous patients (14%)
Time of onset:	before / immediately after birth
Histo:	portal areas surrounded by deposited fibrin + hemorrhage + hepatocellular necrosis

• epigastric / RUQ pain (90%)

• nausea + vomiting (45%), occasionally jaundice

• headache (50%)

• demonstrable edema (55%)

• tender hepatomegaly

• fatty infiltration of liver (peak at 35th week)

• intraparenchymal hemorrhage of liver leading to subcapsular hematoma / rupture into peritoneal cavity

• hepatic necrosis

• subcapsular hematoma of kidney

• ascites + pleural effusions

• vitreous hemorrhage

P.1046

Cx:	(1) Perinatal mortality (8 60%)
	(2) Maternal death (3 24%) from hepatic necrosis, hemorrhagic liver infarction, liver rupture, DIC, abruptio placentae, acute renal failure, sepsis

Hydatidiform Mole

= MOLAR PREGNANCY

Frequency:	1:1,200 to 1:2,000 pregnancies; <5% of abortions
Prognosis:	noninvasive in 85%, locally invasive in 13%, metastasizing in 2%

Complete / Classic Mole

= fertilization of ovum by two haploid 23, X sperm after loss of maternal haploid chromosomes (46, XX) or one sperm which duplicates its genes within egg or occasionally fertilization of an empty egg (= ovum with no active chromosomal material) by 2 different sperm (46, XY)

Histo:

generalized hydropic swelling of all chorionic villi with prominent acellular space centrally; pronounced trophoblastic proliferation of syncytio- and cytotrophoblast

• severe eclampsia prior to 24 weeks

• uterus too large for dates (in 50%)

• 1st trimester bleeding

• marked elevation of -hCG with hyperemesis

• passing of grapelike vesicles per vagina

• hyperthyroidism (due to thyroid-stimulating properties of -hCG)

• anemia (secondary to plasma volume expansion + vaginal bleeding)

• diploid karyotype, almost always paternal XX chromosomes

• hyperechoic to moderately echogenic central uterine mass interspersed with punctate hypoechoic areas

• numerous discrete cystic spaces (= hydropic villi) within a central area of heterogeneous echotexture

• in 25% atypical appearance:

  • large hyperechoic areas (blood clot) + areas of cystic degeneration resembling incomplete abortion

  • single large central fluid collection with hyperechoic rim mimicking an anembryonic gestation / abortion

• no fetal parts / no chorionic membrane

• bilateral theca lutein cysts (18 37 50%), which may take 4 months to regress after evacuation of a molar pregnancy

• ascites

Prognosis:	in 80 85% benign, in 15 20% invasive mole / choriocarcinoma
Rx:	dilatation + suction curettage (curative in 85%)
DDx:	(1) Hydropic degeneration of the placenta (associated with incomplete / missed abortions)
	(2) Degenerated uterine leiomyoma
	(3) Incomplete abortion = retained products with hemorrhage
	(4) Choriocarcinoma
	(5) Loculated abruptio placentae
	(6) Hydropic changes of the placenta

Complete Mole with Coexistent Fetus (1 2%)

= molar degeneration of one conceptus of a dizygotic twin pregnancy with same risk of malignant degeneration as in classic mole

• vaginal bleeding in 2nd trimester

• uterus large for dates

• abnormally elevated serum -hCG

• amniocentesis with normal diploid karyotype excludes diagnosis of partial mole

• normal gestation with placenta + separate typical echogenic material of a hydatidiform mole

• ovarian theca lutein cysts

Prognosis:

fetal survival unlikely due to maternal complications from coexistent mole

Invasive Mole

= chorioadenoma destruens

Histo:	excessive trophoblastic proliferation with presence of villous structure + invasion of myometrium
Preexisting condition:	complete / partial hydatidiform mole

• history of previous molar gestation / missed abortion (75%)

• continued uterine bleeding

• persistently elevated -hCG levels (with failure of -hCG to return to undetectable levels after treatment of a complete hydatidiform mole)

• hyperechoic tissue with punctate lucencies

• irregular focal hyperechoic region within myometrium

• bilateral theca lutein cysts, 4 8 cm in size

• myometrial invasion occasionally demonstrable

Rx:

chemotherapy, hysterectomy (if at risk for uterine perforation)

Partial Mole

= areas of molar change alternating with normal villi + nonviable fetus with significant congenital anomalies

Histo:

moderate focal proliferations of syncytiotrophoblast; normal villi interspersed with hydropic villi

• triploid karyotype (66% 69, XXY; 33% 69, XXX) due to fertilization of single ovum by 2 sperm

• early onset of preeclampsia

• nearly always coexistent fetus with severe abnormalities

• placenta with numerous cystic spaces

• Prognosis:

  • frequently spontaneous abortion (unrecognized as mole for lack of karyotyping of the abortus)

  • no survival of triploid fetus

  • 3% risk of persistent gestational trophoblastic neoplasia

Hydro- / Hematometrocolpos

= accumulation of sterile fluid (hydro~) / blood (hemato~) / pus (pyo~) within uterus (~metria) + vagina (~colpos);

• premenarcheally = secretions + mucus

• postmenarcheally = blood

  Incidence:

  1:16,000 female births

• vague pelvic discomfort

• pain during defecation / urination

• asymptomatic

• smooth symmetric enlargement resulting in pear-shaped uterus distended vagina

• varying amounts of low-level internal echoes centrally within uterus continuous with vaginal canal

• hematosalpinx endometriosis

P.1047




• OB-US:

  • cystic / midlevel echogenic retrovesical mass (mucous secretions secondary to stimulation by maternal estrogens during fetal life)

  • cystic mass fluid-debris level (distended vagina)

  • bladder often not identified (compression by distended vagina)

DDx:	ovarian cyst, duplication cyst, meconium cyst, mesenteric cyst, rectovesical fistula, anterior meningocele, cystic tumor, trophoblastic disease, degenerating leiomyoma / leiomyosarcoma
Cx:	endometritis, myometritis, parametritis (= pelvic lymphangitis), pelvic abscess, septic pelvic thrombophlebitis, urinary tract infection

Acquired Hydro- / Hematometra

• Cause:

• neoplastic obstruction of endocervical canal / vagina, postpartum infection, attempted abortion, cervical stenosis after radiotherapy, postsurgical scarring (eg, dilatation & curettage, traumatic delivery), senile contraction

Congenital Hematometra / Hematometrocolpos

Age:

puberty

• Cause:

  • persistent urogenital sinus = single exit chamber for bladder + vagina; separate orifice for anus; caused by virilization of female fetus / intersex anomaly / arrest of normal vaginal development

    Frequently associated with:	ambiguous genitalia
    Age:	newborn period

  • cloacal malformation = single perineal orifice for bladder + vagina + rectum; caused by early embryologic arrest

    Frequently associated with:	duplex genital tract
    Age:	newborn period

  • imperforate hymen, transverse vaginal septum, segmental vaginal atresia, imperforate cervix, blind horn of bicornuate uterus, Mayer-Rokitansky-K ster-Hauser syndrome (= agenesis of uterus + vagina with active uterine anlage)

  • Hematometrocolpos / hematocolpos are due to imperforate hymen / transverse vaginal septum

  • Hematometra is due to cervical dysgenesis + vaginal agenesis / Mayer-Rokitansky-K ster-Hauser syndrome / obstructed uterine horn

• primary amenorrhea = delayed menarche

• cyclical abdominal pain

• interlabial mass

• May be associated with:

  • imperforate anus, hydronephrosis, renal agenesis / dysplasia, polycystic kidneys, duplication of vagina + uterus, sacral hypoplasia, esophageal atresia

Immature Teratoma of Ovary

= EMBRYONAL TERATOMA = MALIGNANT TERATOMA

= SOLID TERATOMA

Histo:	immature tissue resembling those of the embryo; grade 0 3 reflect amount of immature neuroectodermal tissue
May be associated with:	gliomatosis peritonei = multiple peritoneal implants of mature glial tissue

• elevated AFP levels (50%)

• no elevation of serum hCG levels

• predominantly solid tumor with numerous cysts of varying size

• scattered calcifications (due to invariable association with mature teratoma)

Incompetent Cervix

= gaping cervix usually develops during 2nd trimester /early 3rd trimester

Predisposed:

cervical trauma (D & C, cauterization), DES exposure in utero with cervical hypoplasia, estrogen medication

• physical examination tends to underestimate the true length of the cervix

• Appearance of cervix may change during course of sonographic examination!

  Cause:

  uterine contraction / manual pressure on fundus / patient erect (stress test of cervix) / degree of bladder distention

• dilatation of cervical canal beginning at internal os + extending toward external os:

  • beaking / funneling of cervical canal

  • bulging of membranes through external os (= amniotic fluid within dilated endocervical canal)

  • visualization of fetal parts within dilated endocervical canal

• shortening of cervix to <25 mm

Prognosis:

14th 18th week best time for Rx prior to significant cervical dilatation

Infertility

= failure to conceive after 1 year of unprotected intercourse

Incidence:

affects 10 15% of couples

• Etiology:

  • female factors (55%):

    • Tubal disease (10 20 40%): congenital anomalies, DES exposure, pelvic inflammatory disease, salpingitis isthmica nodosa, endometriosis, postoperative factors, polyp, neoplasm, ectopic pregnancy

    • Uterine factors (2 5%): bicornuate uterus, septate uterus, DES exposure, intrauterine adhesions, endometrial inflammation / infection, uterine neoplasm, complications after pregnancy, leiomyoma

    • Ovulatory disorder (10 20%)

    • Pelvic factors (20 25%)

    • Cervical factors (5 10%)

      Normal Cervical Length

      	Transabdominal	Transvaginal
      1st trimester (<14 wks)	53 17 mm	40 8 mm
      2nd trimester (14 28 wks)	44 14 mm	42 10 mm
      3rd trimester ( 28 wks)	40 10 mm	32 12 mm
      Distended bladder improves visualization but increases cervical length on transabdominal US!
      Difference between nulli- and multiparous women 10%!

  P.1048

  
  

  • male factors (40%)

  • combination of factors (15 25%)

  • unknown cause (5 10%)

• Tests:

  • history + physical examination

  • laboratory tests (mainly hormonal)

  • basal body temperature measurement

  • postcoital test

  • cervical culture

  • endometrial biopsy

  • sonographic monitoring of ovaries

  • sperm agglutination studies

  • in vitro mucus penetration test

  • laparoscopy + hysteroscopy

  • hysterosalpingography

Intrauterine Contraceptive Device

• double echogenic line with plastic IUD

• reverberation echoes with metal IUD

• Types of IUD:

  • Lippes loop

    • 4 5 echogenic dots on SAG view

    • horizontal line / dot on TRV view

  • Saf-T-coil

    • echogenic solid line on SAG view

    • series of echoes / dot on TRV view

  • Copper 7 / Copper T / Progestasert

    • dot in fundus + solid line in corpus on SAG view

    • solid line in fundus + dot in corpus on TRV view

  • Dalkon shield (no longer produced)

Cx:

pelvic inflammatory disease (2 3-fold risk compared with that of non-IUD users) in 35%; actinomycosis with IUD in place for >6 years

Lost IUD

= locator device not palpated

Cause:	1. expulsion of IUD
	2. migration of thread
	3. detachment of thread
	4. uterine perforation of IUD

• Abdominal plain film is indicated if IUD not identified by US!

IUD & Pregnancy

• IUD may not be visualized after 1st trimester (as uterus grows IUD is drawn into cavity)

Prognosis:	high risk of septic abortion
Rx:	early removal of IUD if string remained in vagina

Intrauterine Growth Restriction

• = FETAL GROWTH RETARDATION

• = perinate with a weight at / below the 10th percentile for gestational age occurring as a result of a pathologic process inhibiting expression of normal intrinsic growth potential

for twin pregnancy:

discordant weight >25%

• Fetal weight at / below 10th percentile for age will classify 7% of normal fetuses as growth retarded!

Prevalence:

3 7% of all deliveries; in 12 47% of all twin pregnancies; in 25% of fetuses following birth of a growth-retarded sibling / stillborn

• fundal height as screening test (37 60% true positive, 40 55% false negative; 26 60% false positive)

• IUGR is primarily an ultrasound diagnosis!

• Sequence of events in fetal hypoxia:

  • nonreactive CST > absence of fetal breathing > nonreactive NST > diminished fetal movements > absence of fetal movements > absence of fetal tone

• Etiology:

  • UTEROPLACENTAL INSUFFICIENCY (80%)

    • = injury during period of cell hypertrophy resulting in decreased cell size with features of intrauterine starvation + protective cardiac output redistribution reflex

    • absence of body fat

    • diminished liver and muscle glycogen

    • Maternal causes:

      • asymmetric IUGR / symmetric IUGR (in severe cases)

      • deficient supply of nutrients:

        • cyanotic heart disease, severe anemia (in 10 25% of sickle cell anemia), maternal starvation, life in high altitudes, drugs (anticonvulsants, methotrexate, warfarin), alcohol abuse (dose related), illicit drugs (up to 50% with heroine addiction, 30% with cocaine abuse), uterine anomaly, multiple gestation (in 15 20%)

      • maternal vascular disease resulting in inadequate placental perfusion:

        • nicotine-induced release of catecholamines, preconceptual diabetes, preeclampsia, chronic renal disease collagen vascular disease (SLE)

      • maternal demographics:

        • maternal age (adolescence / advanced), nulliparous mother, small short habitus, racial influence (Asians)

    • Primary placental causes:

      • extensive placental infarctions, chronic partial separation (abruption), partial mole, Breus mole, chorioangioma, placenta previa, low implantation, placental metastases (breast, melanoma), placentitis (luetic, malaria)

        Histo:

        reduction in placental villous surface area + in number of capillary vessels

        • asymmetric growth failure

  • PRIMARY FETAL CAUSES (20%)

    • = injury during the period of cell hyperplasia (= embryogenesis) producing profound reduction in cell number across all cell lines

    • symmetric IUGR (globally decreased intrinsic growth)

    • normal / increased amniotic fluid volume

    • Chromosomal abnormalities (in 2 6%):

      • triploidy, tetraploidy, trisomy 13 + 18 + 21, aneuploidy (Turner syndrome), partial deletion(4p, 5p [cri du chat], 13q), partial trisomy (4p, 18p, 10q, 18q), unbalanced translocation (chromosomes 4 + 15), balanced translocation (chromosomes 5 + 11)

    • Structural anomalies:

      • congenital heart disease, genitourinary anomalies, CNS anomalies, dwarfism

    • Viral infection:

      • rubella (in 40 60%), CMV, varicella (in 40%)

    P.1049

    
    

    • All fetuses with IUGR need to have a detailed and often repeated search for structural anomalies!

Diagnostic Ultrasound Methods in IUGR

• An accurate fix on fetal age dictates accuracy of diagnosis of IUGR (early US exam, clinical dates, early physical exam, pregnancy test)!

• Every effort needs to be made to determine the underlying cause for growth failure as it effects management + perinatal morbidity and mortality!

• Fetal morphometric indices

  • The three key parameters for diagnosing IUGR are

    • low estimated fetal weight (EFW),

    • low amniotic fluid volume (AFV),

    • maternal hypertension (HBP)!

  • intrafetal proportions

    • elevated HC AC ratio for dysmature IUGR (overall 36% sensitive, 90% specific, 67% PPV, 72% NPV; 93% sensitive in fetus >28 weeks MA with severe dysmature IUGR)

    • Early-onset dysmature IUGR not detectable!

    • May not be used in anomalous fetuses!

  • rate of growth = growth velocity

    • HC, AC, FL measurements allow DDx between erroneous dates + normal small fetus + fetus with intrinsic abnormality

    • plot growth curves

    • Minimum time interval of 2 weeks necessary!

• Amniotic fluid volume

  • Screening for decreased amniotic fluid is of value in the fetus with dysmature IUGR (60 84% sensitive, 79 100% accurate)!

  • normal amniotic fluid does not exclude IUGR

  • oligohydramnios means dysmature IUGR in a fetus with normal GU tract until proven otherwise (DDx: trisomy 13 + 18)

• Fetal morphologic assessment + fat distribution

  • diminished thigh circumference

  • absent paraspinal fat pad (posterior neck)

  • reduced / absent malar fat pads

    

    Effect of Birth Weight on Perinatal Morbidity and Mortality

  • disproportionately small liver size

  • increased small bowel echogenicity (= absent omental fat)

• Placental assessment

  • increased placental calcium deposition

• Biophysical profile

  Accuracy:

  false-negative fetal death rate of 0.645/1000 fetuses within 1 week of the last normal BPP; 33% sensitivity, 17% positive predictive value

• Invasive fetal testing:

  • fetal blood analysis for karyotyping, hypoxemia, hypercapnia, acidemia, hypoglycemia, hypertriglyceridemia

• Nonstress test (NST)

• Contraction stress test (CST)

Diagnostic Doppler Methods in IUGR

• PLACENTAL PERFUSION

  • Umbilical artery velocimetry (UA)

    • Not useful with unknown dates / for screening!

    • Better predictor of adverse perinatal outcome than MCA or RA pulsatility index

      Pathophysiology:

      fewer terminal villi due to developmental defect / villous infarction

    • elevated systolic:diastolic ratio (S/D ratio >3.0 beyond 30 34 weeks GA) indicates an increase in vascular resistance within placental circulation

    • absent diastolic flow = 50 90% mortality rate

    • reverse diastolic flow = impending fetal collapse (due to severe fetal hypoxia)

      Note:

      S/D ratio increases with sampling site closer to fetus + increasing fetal heart rate; S/D ratio decreases with advancing gestational age; S/D ratio may decrease in lateral recumbent position

  • Uterine artery waveform

    Method:

    sample volume at point of overlap of uterine artery with external iliac artery

    • S/D ratio >2.6 after 26 weeks GA

    • persistence of early diastolic notch

    Pathophysiology:

    lack of trophoblastic invasion of spiral arteries

    • Rules:

      • Trophoblastic invasion may not occur until 20 22 weeks GA in some patients

      • An abnormal waveform at 24 weeks GA will never become normal

      • A normal waveform will never revert to a high resistive waveform

      • Both / one uterine artery may be abnormal

• FETAL CIRCULATION

  Pathophysiology:

  preferential shunting of blood to brain ( brain sparing ) in response to hypoxemia

  • Fetal middle cerebral artery (MCA)

    • Significant correlation between hypoxemia at cordocentesis and MCA pulsatility index

    • If the MCA pulsatility index is normal the fetus is unlikely to have a major adverse outcome

    P.1050

    
    

    Method:

    sample volume in MCA of near field 1 cm distal to its origin

    • reduction in S/D ratio 2.5 to 3.0 (normally about 6.0)

  • Renal artery waveform (RA)

    Method:

    sample volume in proximal renal artery on coronal image that visualizes the entire renal artery

    • increase of pulsatility index

  • Fetal aortic flow volume (no proven usefulness)

    • decrease in blood flow to <185 246 mL/kg/min

Cx:	increased risk for perinatal asphyxia, meconium aspiration, electrolyte imbalance from metabolic acidosis, polycythemia
Neonatal Cx:	pulmonary hemorrhage + vasoconstriction, persistent fetal circulation, intracranial hemorrhage, bowel ischemia, necrotizing enterocolitis, acute renal failure
Prognosis:	6 8-fold increase in risk for intrapartum death + neonatal death 20% of all stillborn fetuses are growth retarded!

• DDx of fetus small for gestational age (SGA):

  • Small normal fetus (80 85%)

  • Small abnormal fetus (5 10%)

  • Dysmature fetus (10 15%)

Pure Symmetric IUGR

• = decreased cell-number IUGR = early-insult IUGR

• = low-profile IUGR

• = proportionate reduction of all fetal measurements due to

  • intrinsic alteration in growth potential (usually due to chromosomal abnormalities)

  • severe nutritional deprivation overwhelming protective brain-sparing mechanism occurring prior to 26 weeks MA + persisting until delivery

• proportionate decrease in HC and AC maintaining normal HC AC ratios

• estimated fetal weight <10th percentile for age by middle of 2nd trimester

Mixed IUGR

• = onset of IUGR during period of mixed hyperplasia / hypertrophy with near normal inherent fetal growth potential but decreased size + impaired function of placenta

  Sonographic Criteria for IUGR

  Sonographic Finding	PPV [%]	NPV [%]
  Advanced placental grade	16	94
  Elevated FL AC ratio	18 20	92 93
  Abnormal UA waveform	17 37
  Low total intrauterine volume	21 24	92 97
  Small BPD	21 44	92 98
  Slow BPD growth rate	35	97
  Low EFW	45	99
  Oligohydramnios	55	92
  Elevated HC AC ratio	62	98

• impaired fetal growth asymmetry

• abnormal Doppler umbilical artery flow velocity (due to increased placental vascular resistance)

• progressive oligohydramnios

Asymmetric IUGR

• = decreased cell-size IUGR

• = late-onset IUGR = late-flattening IUGR (75%)

• = disproportionate reduction of fetal measurements due to uteroplacental insufficiency with preferential shunting of blood to fetal brain occurring after 26 weeks GA

• IUGR usually not detectable before 32 34 weeks GA (time of maximal fetal growth)!

Effective time for screening:	34 weeks MA
Routine surveillance:	every 4 weeks beginning at 26 weeks MA

• AC >2 SD below the mean for age = highly suspicious; AC >3 SD below mean for age

  • = diagnostic (AC single most effective fetal parameter for detection of asymmetric IUGR)

• high HC AC and FL AC ratios (head size + femur length less affected)

• fetal weight percentile useful for follow-up

• accelerated placental maturity

• decreased amniotic fluid volume

• elevated umbilical artery S/D ratio

• FL AC ratio + umbilical artery S/D ratio are the only effective techniques to screen for IUGR on a single exam with late prenatal care in 3rd trimester!

Krukenberg Tumor

• = ovarian tumors from GI tract cancer (colon:stomach = 2:1) now including pancreatic + biliary primaries; 2% of females with gastric cancer develop Krukenberg tumor

• Krukenberg tumors antedate the discovery of the primary lesion in up to 20%!

  Age:

  any age, most common in 5th 6th decade

• in 80% bilateral hypo- / hyperechoic mass cystic degeneration

Limb-Body Wall Complex

Prevalence:	1:10,000 live births
Cause:	? severe form of amniotic band syndrome; ? early vascular disruption; ? embryonic dysplasia due to malformation of ectodermal placodes

• EXTERNAL DEFECTS

  • Ventral wall anomaly

    • large eccentric defect

    Location:

    L:R = 3:1 (DDx: gastroschisis)

  • Craniofacial defects:

    • anencephaly, cephalocele, facial cleft

  • Limb reductions

  • Spinal defects: dysraphism, scoliosis

• INTERNAL DEFECTS (in 95%)

  • Cardiac defects

  • Diaphragmatic absence

  • Bowel atresia

  • Renal abnormalities:

    • agenesis, hydronephrosis, dysplasia

• persistence of extraembryonic coelom (= separation of amnion + chorion)

  P.1051

  
  

  Prognosis:

  invariably fatal shortly after birth

Macrosomia

• = FETAL GROWTH ACCELERATION

• = fetus large for gestational age (LGA) with EFW >90th percentile for age / >4,000 g at term

• AC >3 SD above the mean for age (most reliable + largest measurement)

• estimated fetal weight (EFW) including fetal head, abdomen, femur length >90th percentile ( 15% accuracy)

• low FL AC ratio

• low HC AC ratio

• enlarged thigh circumference

• low FL thigh circumference ratio

• greater than expected interval growth

• polyhydramnios

  Risk:

  shoulder dystocia, prolonged labor, meconium aspiration

Massive Ovarian Edema

• = tumorlike condition with marked enlargement of one / (occasionally) both ovaries due to accumulation of edema fluid in stroma

  Age:

  6 33 (average 21) years

• Cause:

  • partial / intermittent torsion (obstruction to ovarian lymphatic + venous drainage)

  • ovarian stromal proliferation with enlargement of ovary susceptible to torsion

Histo:

edematous ovarian stroma + extensive fibromatosis surrounding primordial follicles, luteinized cells

• acute / intermittent lower abdominal pain for months

• masculinization (in chronic phase)

• solid / multicystic adnexal mass

• ovarian diameter of 5 40 (mean 11.5) cm

  Rx:

  oophorectomy / salpingo-oophorectomy / wedge resection with ovarian suspension

Mucinous Ovarian Tumor

Incidence:	20% of all ovarian tumors; 2nd most common benign epithelial neoplasm of ovary (after serous ovarian neoplasm)
Histo:	single layer of nonciliated tall columnar epithelium with clear cytoplasm of high mucin content (similar to endocervix + intestinal epithelium)
Age:	middle adult life, rare before puberty + after menopause
Cx:	rupture may lead to pseudomyxoma peritonei
DDx:	serous ovarian tumor (smaller, unilocular)

Mucinous Cystadenoma (80%)

Prevalence:	20% of all benign ovarian neoplasms
Age:	3rd 5th decade of life

• multilocular cyst with numerous thin septa

• cysts frequently have high protein content:

  • low-level echoes in cysts

  • high attenuation on CT

  • hyperintense on T1WI

• usually unilateral, bilateral in 5%

Borderline Malignant Mucinous Cystadenoma (10%)

Mucinous Cystadenocarcinoma (10%)

Histo:

mucoid material in cysts, sometimes accompanied by hemorrhagic / cellular debris; difficult to differentiate from benign variety + metastasis from intestinal primary

• multilocular with numerous smooth thin-walled cysts

• solid tissue areas: thick septa + other soft-tissue elements within septated cyst

• usually unilateral; bilateral in 5 10% of stage I cases

• capsular infiltration with loss of definition + fixation

• CT:

  • multiseptated tumor of low attenuation

  • high-attenuation proteinaceous material (20 30 HU) in some loculi

• MR:

  • variable signal intensity in different loculi (proteinaceous / mucinous content, hemorrhage)

Nuchal Cord

= umbilical cord encircling fetal neck: single loop > two loops (2 3%) > 3 or more loops (<1%)

Incidence:	25% of pregnancies; frequently transient
Associated with:	increased cord length, small fetus, vertex presentation, polyhydramnios

• generally not of clinical significance: no difference in 5-minute Apgar score, no increase in infant mortality

• two adjacent cross sections of cord on longitudinal view of neck (diagnosis facilitated by color Doppler flow)

• indentation of skin by nuchal cord suggests tight loop

  Risk:

  signs of fetal distress (fetal bradycardia, variable decelerations, depressed 1-minute Apgar score)

• OB management:

  • Assess fetal well-being (biophysical profile biweekly, NST, fetal growth)

  • Vaginal delivery permissible if without evidence of fetal compromise

  • Intervention only for signs of fetal distress

Omphalocele

• = midline defect of anterior abdominal wall due to failure to form the umbilical ring during 3rd to 4th week of gestation with herniation of intraabdominal contents into base of umbilical cord

  Prevalence:

  1:4,000 to 1:5,500 pregnancies

• Cause:

  • migration failure of lateral mesodermal body folds:

    • omphalocele contains liver

  • persistence of primitive body stalk beyond 12th week MA:

    • omphalocele contains primarily bowel

      Age:

      earliest detection at 12 weeks menstrual age

  • High incidence of ASSOCIATED ANOMALIES (45 88%):

    • Chromosomal (10 30 58%): trisomy 13, 18, 21, Turner syndrome (13% with liver in omphalocele, 77% with bowel in omphalocele), triploidy

    • Genitourinary (40%): bladder exstrophy

      • OEIS complex = Omphalocele + bladder Exstrophy + Imperforate anus + Spinal anomalies

    • Cardiac (16 30 47%): VSD, ASD, tetralogy of Fallot, ectopia cordis in pentalogy of Cantrell, DORV

    P.1052

    
    

    • Neural tube defects (4 39%): holoprosencephaly, encephalocele, cerebellar hypoplasia

    • IUGR (20%)

    • Beckwith-Wiedemann syndrome (5 10%)

    • GI tract:

      • intestinal atresia (vascular compromise); malrotation; abnormal fixation of liver, esophageal atresia, facial cleft, diaphragmatic hernia

    • Limb-body wall deficiency; cystic hygroma

  • MS-AFP 2.5 in 40 70%

  • midline central defect at base of umbilical cord insertion:

    • defect over entire ventral abdominal wall (mean size 2.5 5 cm)

    • widened cord where it joins the skin of the abdomen

  • cord inserting at apex of defect

  • herniation of abdominal viscera at base of umbilical cord:

    • liver (27%) stomach bowel

  • covering amnioperitoneal membrane (inner layer = peritoneum; outer layer = amnion); may rupture in up 15% of cases

  • hypoechoic loose mesenchymal tissue (= Wharton jelly) between layers of membrane

  • ascites within herniated sac (common)

  • polyhydramnios (occasionally oligohydramnios)

    mnemonic:

    OMPHALOCele

    • Other anomalies (common)

    • Membrane surrounding viscera

    • Perfectly midline

    • Heart anomalies

    • Ascites

    • Liver commonly herniated

    • O for zero bowel complications

    • Chromosomal abnormalities (common)

      Cx:	(1) Infection, inanition
      	(2) Immaturity (23%)
      	(3) Rupture of hernia sac
      	(4) Intestinal obstruction
      Mortality rate:	10% mortality if isolated abnormality; 80% with one / more concurrent malformations; nearly 100% with chromosomal + cardiovascular abnormalities
      DDx:	(1) Gastroschisis (usually right-sided defect)
      	(2) Limb-body wall complex (usually left-sided defect)

Pseudo-omphalocele

• Deformation of fetal abdomen by transducer pressure coupled with an oblique scan orientation may give the appearance of an omphalocele

  • obtuse angle between pseudomass and fetal abdominal wall

• Physiologic herniation of midgut into umbilical cord between 8th and 12th week of gestation

  • herniated sac never contains liver

  • herniated sac usually <7 mm

  • disappears by 12th week GA

Omphalomesenteric Duct Cyst

Etiology:	persistence + dilatation of a segment of the omphalomesenteric / vitelline duct joining the embryonic midgut and the primary yolk sac, which is formed during the 3rd week and closed by the 16th week of gestation
Histo:	cyst lined by columnar mucin-secreting gastrointestinal epithelium

• M:F = 3:5

Location:

usually in close proximity to fetus

• umbilical cord cyst up to 6 cm in diameter

• beneath amniotic surface of cord (= eccentric)

Cx:	(1) Compression of umbilical vessels by expanding cyst
	(2) Erosion of umbilical vein from acid-producing gastric mucosal lining
DDx:	allantoic cyst, umbilical cord hematoma

Ovarian Cancer

• 8th leading cause of cancer in women (4% of all cancers among women); 2nd most common gynecologic malignancy; 5th leading cause of cancer deaths in women after lung, breast, colon, pancreas (leading cause of cancer deaths of all female cancers); accounts for 50% of cancer deaths of female genital tract

• 1:70 women will develop ovarian cancer (lifetime risk)

• 1:100 women will die of ovarian cancer

  Etiology:	ovarian surface epithelium proliferates temporarily to repair defect after rupture of ovum, which may result in an inclusion body / cystoma ; an error in DNA replication within inclusion body may occur resulting in inactivation / loss of a tumor-suppressor gene
  Incidence:	affects 1:2,000 women; 50 cases per year per 100,000 women (33 cases per year per 100,000 women > age 50); 26,700 new cases + 14,500 deaths in 1996
  Age:	increasing with age; peaking at 55 59 years (80% of cases in women >50 years)

• Histo:

  • The proportion of malignant tumors increases with age:

    <20 years of age	4%
    >50 years of age	40%

  • MALIGNANT EPITHELIAL TUMORS (85%)

    • = arising from surface epithelium / mesothelium

      Age peak:

      6th 7th decade; rare before puberty

      • serous tumor resembling ciliated columnar cells of the fallopian tubes (60 80%)

      • mucinous tumor similar to endocervical canal epithelium (15%)

      • endometrioid tumor similar to endometrial adenocarcinoma (8 15%)

      • clear cell carcinoma = mesonephroid tumor (5%)

      • undifferentiated tumor (4%) = cellular dedifferentiation does not allow categorization

      • Brenner tumor (2.5%)

  • MALIGNANT GERM CELL TUMORS (7%)

    Frequency:	2/3 of ovarian malignancies in females <20 years of age
    Age:	4 27 years

    • mature teratoma (10%) = the only benign variety

    • dysgerminoma (1.9%)

    • immature teratoma (1.3%)

    • endodermal sinus tumor (1%)

    • malignant mixed germ cell tumor (0.7%)

    • choriocarcinoma (0.1%)

    P.1053

    
    

    • embryonal carcinoma (0.1%)

  • METASTASES (5 10%)

  • SEX CORD STROMAL TUMORS (7%)

    Size versus risk of malignancy:	<5 cm in 3%
    	5 10 cm in 10%
    	>10 cm in 65%

• Epidemiology:

  • sporadic: ovulation hypothesis = risk of ovarian cancer is a direct function of the number of ovulatory cycles during a woman's life span

  • hereditary (1 5%) = defined as ovarian cancer occurring in at least two 1st-degree relatives

    • 50% lifetime probability to develop ovarian cancer

      • breast cancer-ovarian cancer syndrome

      • ovarian cancer only syndrome

      • Lynch type II cancer family syndrome

        • = inheritance of nonpolyposis colorectal cancer, endometrial cancer and (rarer) ovarian cancer

• Increased risk:

  • nulliparity, early menarche, late menopause, Caucasian race, higher socioeconomic group, positive family history for ovarian cancer (risk factor of 3 with one close relative, risk factor of 30 with two close relatives affected with ovarian cancer), history of breast cancer (risk factor of 2) / early colorectal cancer (risk factor of 3.5

• Decreased risk = protective effect:

  • multiparity, use of oral contraceptives, breast-feeding

• Staging laparotomy:

  • = abdominal hysterectomy + bilateral salpingo-oophorectomy + omentectomy + random peritoneal biopsy + lymph node biopsy

• Spread:

  • direct extension through subperitoneal space (sigmoid mesocolon on left, cecum + distal ileum on right), uterus, fallopian tubes, broad ligament

  • intraperitoneal implantation = exfoliation of tumor cells from ovarian capsule into peritoneal space (often microscopic)

    • with frequent seeding to:

      • posterior cul-de-sac in pouch of Douglas

      • infundibulopelvic ligaments

      • omentum

      • superior aspect of sigmoid

      • right paracolic gutter

      • termination of small bowel mesentery

      • undersurface of right hemidiaphragm

        Ovarian Cancer

        Type	Benign	Borderline	Malignant
        [all types combined]	[75%]	[4%]	[21%]
        Serous	60%	15%	25%
        Mucinous	80%	10%	10%
        Endometrioid	~0%	~0%	~100%
        Clear cell	~0%	~0%	~100%
        Undifferentiated	0%	0%	100%

      • with less frequent seeding to:

        • Morison pouch

        • liver surface

        • porta hepatis

        • intrahepatic fissure

    • lymphatic spread

      • craniad parallel to gonadal veins in infundibulo-pelvic ligament terminating at level of renal vessels

      • laterally through broad ligament terminating at pelvic side wall: external iliac, obturator, hypogastric chains

      • lymph node short-axis diameter of >1 cm (in 88% positive for ovarian cancer)

      • hydronephrosis (2nd most common form of tumor-related morbidity after bowel obstruction)

      • thoracic adenopathy (paracardiac lymph nodes >5 mm in 28% of stage II/III disease + in 29% mediastinal lymphadenopathy at autopsy)

    • hematogenous dissemination (% at autopsy):

      • liver (45 48%), lung (34 39%), adrenal gland (15 21%), pancreas (11 21%), spleen (15 20%), bone (11%), kidney (7 10%), skin (5%), brain (3 6%)

      • malignant pleural effusion (pleural metastases in 28 60%, lung parenchymal metastases in 7%, pericardial metastases in 5%)

  • often silent without obvious signs / symptoms

  • occasional pelvic-abdominal pain

  • constipation, urinary frequency

  • early satiety

  • ascites

  • paraneoplastic hypercalcemia

  • elevated CA-125 levels (= cancer-associated marker = high-molecular-weight glycoprotein with normal level of <35 units/mL):

    • >35 U/mL in 29 50% of stage I disease

    • >65 U/mL in 21% of stage I disease

      Staging of Ovarian Cancer

      Stage	(FIGO system) based on staging laparotomy
      I	Limited to ovary
      I a	limited to one ovary
      I b	limited to both ovaries
      I c	+ positive peritoneal lavage / ascites
      II	Limited to pelvis
      II a	involvement of uterus / fallopian tubes
      II b	extension to other pelvic tissues
      II c	+ positive peritoneal lavage / ascites
      III	Limited to abdomen = intraabdominal extension outside pelvis / retroperitoneal or inguinal nodes / extension to small bowel / omentum
      III a	microscopic abdominal peritoneal seeding
      III b	2 cm implants of abdominal peritoneum
      III c	>2 cm implants of abdominal peritoneum
      IV	Hematogenous disease (liver parenchyma) / spread beyond abdomen
      50 70 75% of patients have stage III / IV disease at time of diagnosis!

      P.1054

      
      

      • CA-125 levels elevated in 80% of ovarian cancers (60% of mucinous + 20% of nonmucinous tumors) and in 40% of patients with advanced intraabdominal nonovarian malignancy!

      • CA-125 levels elevated in 30% of benign processes (in 1% of healthy individuals, fibroid, first-trimester pregnancy, menstruation, endometriosis, PID, benign ovarian tumors, liver cirrhosis, pancreatitis)!

  • Limitations of imaging:

    • Microscopic peritoneal disease not detectable!

  • Benefit of imaging:

    • Second-look surgery can be avoided if there is evidence for residual / recurrent tumor!

  • US:

    • Screening finds adnexal cysts in 1 15% of postmenopausal women; only 3% of ovarian cysts <5 cm are malignant!

    • @ Morphologic tumor criteria (85 97% sensitive, 56 95% specific, 99% NPV):

      • thick irregular walls and thick septations (DDx: endometrioma, abscess, peritoneal cyst, cystadenofibroma, mucinous cystadenoma)

      • papillary projections 3 mm (in 67%, 38%, 9% of borderline, malignant, benign neoplasm)

      • solid / moderately echogenic loculi

      • postmenopausal ovarian volume >9 cm³

    • @ Doppler tumor criteria (50 100% sensitive, 46 100% specific, 49% PPV; more sensitive + specific in postmenopausal women):

      • presence of color flow (in 93% of malignant + 35% of benign tumors) usually within thick wall, septa, papillary projections, solid inhomogeneous areas

      • low-resistance Doppler waveform (due to lack of muscular layer of arterial wall + presence of arteriovenous shunts in neoplasms):

        • RI <0.40, PI <1.0 (37 47% PPV)

        • false positive: physiologic alteration of ovarian blood flow during menstrual cycle, benign tumor, acute inflammatory disease, endometriosis

    • @ Metastatic disease

      • omental / peritoneal masses ( omental cake )

      • pseudomyxoma peritonei (with tumor rupture)

      • liver metastases

      • ascites

  • CT (70 90% preoperative staging accuracy):

    • @ Primary tumor:

      • lesion diameter >4 cm

      • enhancing papillary projections

      • septa and walls >3 mm thick

      • partially solid, partially cystic mass

      • lobulated solid mass

      • tumor vessels on contrast-enhanced images

    • @ Local extension:

      • localized distortion of uterine contour

      • irregular interface between tumor and myometrium

      • loss of tissue plane between tumor and wall of sigmoid colon / bladder

      • encasement of sigmoid colon

      • tumor distance from pelvic side wall <3 mm

      • iliac vessels surrounded / displaced by tumor

    • @ Secondary findings:

      • ascites:

        • often lesser sac ascites with displacement of fundus and posterior wall of stomach anteriorly (DDx to benign ascites) + gastrosplenic ligament laterally

        • loculated ascites due to adhesions

      • >10-mm nodular / plaquelike peritoneal implants:

        • indentation of hepatic / splenic surface

        • calcifications

      • lymphadenopathy, may be calcified

      • omental implants:

        • small nodules / strands of hyperdense soft tissue increasing the attenuation of omental fat / marked omental thickening ( omental cake )

        • fat plane obscured between anterior abdominal wall + intestinal wall

      • mesenteric deposits:

        • round / irregular ill-defined masses / stellate lesions of small bowel mesentery

        • tethering of small bowel loops

      • invasion of bowel:

        • bowel obstruction (most common form of ovarian-cancer associated morbidity)

        • nodular / plaquelike lesions along / projecting from peritoneal surfaces

        • bowel wall thickening

      • pseudomyxoma peritonei

  • MR (combines best features of US and CT)

  • BE:

    • serosal spiculation / tethering

    • annular constriction / complete obstruction

  • Rx:

    stage I:	total abdominal hysterectomy (TAH) + bilateral salpingo-oophorectomy (BSO) melphalan / intraperitoneal P-32
    stage >I:	TAH-BSO + surgical cytoreduction (debulking) + 6 cycles of chemotherapy (cyclophosphamide + cisplatin)

  • Prognosis (without change in past 60 years):

    • 46% overall 5-year survival rate, 5 8% for stage IV, 14 30% for stage III, 50% for stage II, 80 90% for stage I

      DDx:

      tuboovarian abscess, dermoid cyst, endometrioma

Ovarian Cyst

Functional / Retention Cyst

• Cause:

  • failure of involution of follicle / corpus luteum with changes in the menstrual cycle

  • excessive hormonal stimulation of follicles preventing normal follicular regression (eg, theca-lutein cysts)

    Prognosis:	spontaneous regression is common but unpredictable; typically resolve within 2 menstrual cycles (less likely if cyst >5 cm)
    Cx:	torsion
    Rx:	(1) Hormonal manipulation
    	(2) Surgery (absolutely indicated if cyst enlarges)
    	(3) Percutaneous aspiration (if chance of malignancy is nil as in infants)
    DDx:	cystic teratoma, simple benign epithelial neoplasm, endometrioma in resolution, paraovarian cyst, quiescent hydrosalpinx

P.1055

Follicular Cyst (from preovulatory follicle)

• Cause:

  • unruptured Graafian follicle from failure to ovulate

  • Graafian follicle with failure to regress / involute

  • ruptured Graafian follicle that sealed immediately (after continued stimulation)

• may elaborate estrogen, extremely common

• sign of anovulatory cycle

Predisposed:

patients during puberty + menopause; S/P salpingectomy

• thin-walled, unilocular cyst

• size usually >2.5 cm / occasionally up to 10 cm

• usually multiple / may be single

• low-level internal echoes / fluid-debris level / septations / predominantly hyperechoic

  • = hemorrhagic cyst (DDx: teratoma, abscess, torsion, malignancy, ectopic pregnancy)

Prognosis:

usually disappears after 1 2 menstrual cycles

Corpus Luteum Cyst (from postovulatory follicle)

• = hemorrhage into mature corpus luteum

• Types:

  • Corpus luteum of menstruation

    • = formed after rupture of follicle + increasing in size until 22nd day of menstrual cycle

    • usually >12 17 mm in size

    • elaborates progesterone causing delayed menstruation / persistent bleeding

      Prognosis:

      resolves within 1 2 menstrual cycles

  • Corpus luteum of pregnancy

    • = caused by hCG stimulation during pregnancy

    • may be temporarily painful

    • usual size 30 40 mm, may grow up to 15 cm in diameter

      • Excessively large cysts with thin wall suggest poor function (= low progesterone levels)

    • reaches maximum size after 8 10 weeks

    • occurs on same side as ectopics in 85%

  Prognosis:

  resolves by 12 16 weeks, occasionally persists past 1st trimester

  • high diastolic flow component

  • thin-walled usually unilateral cyst

  • echogenic (organized clot) / sonolucent (resorbed blood)

  • low-level internal echoes frequent (= hemorrhage)

  • Cx:

    • Enlarging hemorrhagic corpus luteum with

      • severe pelvic adhesions preventing ovulation of luteinized follicles

      • NSAIDs which may cause luteinized unruptured follicle syndrome

      • excessive anticoagulation

      • endometriosis

    • Rupture with intraperitoneal life-threatening hemorrhage at ovulation

      DDx:

      endometrioma, ovarian tumor, organized clot in any enclosed space

Corpus Albicans Cyst

= from corpus luteum following regression of luteal tissue; no hormone production

Theca Lutein Cyst

= multiple bilateral corpus luteum cysts

• in hyperstimulated ovary from ovary-stimulating drugs, twins, trophoblastic disease

• elaborates estrogen

Surface Epithelial Inclusion Cyst

• common in postmenopausal women

  Age:	any; in newborns (influence of maternal estrogen)
  Incidence:	3 5 17% in postmenopausal women

• usually asymptomatic

• acute unilateral pelvic pain (from hemorrhage / pressure)

• up to 8 10 cm in diameter

Imaging Classification of Ovarian Cyst

• SIMPLE CYST

  • unilocular smooth-walled cyst + thin sharply defined wall of <3 mm

  • contents anechoic = NO internal septations / mural nodules

  • posterior acoustic enhancement

  • Doppler flow in cyst wall (detected in 19 61%) with pulsatility index >1.0 / RI >0.4 (unreliable!)

  • isointense to urine on T1WI + T2WI

    DDx:

    serous cystadenoma

• HEMORRHAGIC CYST

  • = functional cysts that developed internal hemorrhage

  • US:

    • echogenic mass (= solid clot)

    • whirled pattern of mixed echogenicity

    • ground-glass pattern = diffuse low-level echoes

    • fishnet weave pattern = fine interdigitating septations / lacelike reticular echoes

    • NO color Doppler signals inside cyst

  • MR:

    • intermediate / high intensity on T1WI

    • intermediate / high intensity with distinct central area of hypointensity on T2WI

      Cx:

      rupture into intraperitoneal space

• COMPLEX CYST

  • = does not satisfy criteria for hemorrhagic cysts / endometrioma

  • internal septations / mural nodules / internal echoes

  • mixed signal intensity, hyperintense on T2WI

Management of Ovarian Cyst

• NEONATAL

  • change in position between exams suggests pedunculation with potential for torsion

  • fluid debris level / low-level echoes / retracting clot suggest torsion

• PREMENOPAUSAL

  • Unilocular cyst 2.5 cm hemorrhage

    Rx:

    no follow-up unless on birth control pills

  • Unilocular thin-walled cyst 2.5 6 cm without hemorrhage

    P.1056

    
    

    Rx:

    clinical / sonographic follow-up in 1 2 months addition of hormones

  • Unilocular cyst 2.5 6 cm with hemorrhage

    Rx:

    sonographic follow-up in 1 month addition of hormones

  • Unilocular cyst >6 cm

    Rx:	surgery
    N.B.:	All follow-up scans should take place in the immediate postmenstrual period, when follicular cysts should not be present!

• POSTMENOPAUSAL

  • Screening of 1,300 symptomatic women:

    • in 2.5% abnormalities on US

    • in 1.9% benign ovarian tumors

    • in 0.15% ovarian cancers

  • Unilocular nonseptated thin-walled cyst <3 cm

    Incidence: 15 17%

    • high resistive index (RI) of >0.7 (resistive index <0.40 is suspect for malignancy!)

      Prognosis:	56% decrease in size / disappear; 28% remain unchanged for up to 2 years
      DDx:	serous ovarian cyst, peritubal cyst, hydrosalpinx
      Rx:	serial follow-up

  • Septated cyst / cyst >3 cm / cyst with low RI

    • 18% of complex cysts are malignant!

      Rx:

      CA-125 determination + surgical exploration

Ovarian Fibroma

Incidence:	3 4% of all ovarian tumors; bilateral in <10%
	Most common of the sex cord-stromal tumors!
Age:	primarily >40 years
Path:	solid firm white mass
Histo:	pure mesenchymal tumor consisting of intersecting whorled bundles of spindle-shaped fibroblasts + collagen; varying degrees of edema often separate cells
Association:	fibromas occur in 17% of patients with the basal cell nevus syndrome (Gorlin) syndrome (commonly bilateral calcified tumors + mean age of 30 years)

• usually asymptomatic (pure fibromas are not estrogenic, admixture of theca cells causes estrogenic effect)

• Meigs syndrome (in only 1%):

  • ascites (in 10 40% of tumors >10 cm)

  • pleural effusion (rare)

  • ascites + pleural effusion resolve after removal of tumor

    Location:

    bilateral in 4 8%

• cystic degeneration and edema in larger lesions

• US:

  • solid hypoechoic mass with marked sound attenuation

  • occasionally hyperechoic / with increased through-transmission

• MR:

  • well-circumscribed low-signal-intensity mass on T1WI + T2WI less than or equal to myometrium due to abundant collagen content (FAIRLY DIAGNOSTIC)

  • scattered high-signal-intensity areas (edema / cystic degeneration)

• CT:

  • well-defined solid homogeneous / slightly heterogeneous slightly hypoattenuating mass

  • poor delayed contrast enhancement

    DDx:

    pedunculated uterine leiomyoma, Brenner tumor, adenofibroma, malignant ovarian neoplasm

Ovarian Hyperstimulation Syndrome

Incidence:

severe OHSS in 1.5 6% under Pergonal therapy

• Etiology:

  • Induced by hCG therapy with human menopausal gonadotropin (Pergonal ), occasionally with clomiphene (Clomid )

  • Hydatidiform mole

  • Chorioepithelioma

  • Multiple pregnancies

Path:

enlarged ovaries with multiple follicular + theca lutein cysts, edematous stroma (fluid shift secondary to increased capillary permeability)

• abdominal pain (100%) + distension (100%)

• nausea (100%), vomiting (36%)

• acute abdomen (17%)

• dyspnea (16%)

• thrombophlebitis (11%)

• marked hemoconcentration

• fainting (11%)

• blurred vision (5%)

• anasarca (5%)

• hydrothorax

• enhanced fertility

• ovary >5 cm in longest dimension containing large geometrically packed follicles

• ovarian cyst >10 cm (100%): usually disappear after 20 40 days; may persist for 12 16 weeks during pregnancy

• ascites (33%)

• pleural effusion (5%)

• hydroureter (11%)

  Cx:	(related to volume depletion)
  	(1) Hypovolemia + hemoconcentration
  	(2) Oliguria, electrolyte imbalance, azotemia
  	(3) Death from intraabdominal hemorrhage / thromboembolic event

Ovarian Vein Thrombosis

• Etiology:

  • Bacterial seeding from puerperal endometritis with secondary thrombosis (pregnancy + puerperium are hypercoagulable states) = puerperal ovarian vein thrombophlebitis

  • Pelvic inflammatory disease

  • Gynecologic surgery

  • Malignant tumors

  • Chemotherapy

Incidence:

1:600 1:2,000 deliveries

• presents on 2nd / 3rd postpartum day

• lower abdominal / flank pain (>90%)

• palpable ropelike tender abdominal mass (50%)

• fever if diagnosis delayed

Location:

right ovarian vein (80%), bilateral (14%), left ovarian vein (6%)

P.1057

• CT:

  • tubular structure in location of ovarian vein with low-density center + peripheral enhancement

Cx:	IVC thrombosis; pulmonary embolism (25%); septicemia; metastatic abscess formation
Mortality:	5%
Rx:	IV antibiotics + heparin; ligation of involved vessel at most proximal point of thrombosis after failure to improve after 3 5 days
DDx:	appendicitis, broad-ligament phlegmon / hematoma, torsion of ovarian cyst, urolithiasis, pyelonephritis, degenerated pedunculated leiomyoma, pelvic cellulitis, pelvic / abdominal abscess

Paraovarian Cyst

= vestigial remnant of wolffian duct in mesosalpinx

Frequency:

10% of all pelvic masses

• Embryology:

  • wolffian body (= mesonephros) consists of

    • mesonephric duct (= wolffian duct) in female degenerates into vestigial structures of epithelial-lined cysts (= canals / duct of Gartner)

      Location:

      at lateral edge of uterus and vagina extending from broad ligament to vestibule of vagina

    • mesonephric tubules in female degenerate into vestigial structures of

      • Epo phoron (at lateral part of fallopian tube)

      • Paro phoron: (at medial part of fallopian tube)

        Location:

        between the tube and hilum of the ovary within the two peritoneal layers of broad ligament

• Gartner duct cyst:

  • inclusion cyst lateral to vagina + uterine wall

• Paro phoron:

  • medial location between tube + hilum of ovary

• Epo phoron:

  • lateral location between tube + hilum of ovary

• Hydatids of Morgagni (= appendices vesiculosae): most lateral + outer end of Gartner duct

  • 1 vesicle(s) attached to fringes of tube + filled with clear serous fluid

• thin-walled unilocular cyst, up to 18 cm in diameter

• may arise out of pelvis (if pedunculated + mobile)

• low-level internal echoes (from hemorrhage)

  DDx:

  functional cyst, cystic teratoma, benign epithelial neoplasm

Paraovarian Cystadenoma

May be associated with:	von Hippel-Lindau disease
Location:	typically unilateral

• simple cyst

• one / more small nodules along a smooth inner wall (86%)

• septations

  Cx:	malignant degeneration in 2 3%
  DDx:	(1) Hydrosalpinx (tubular shape, folds / short echogenic lines protruding into lumen)
  	(2) Peritoneal inclusion cyst (surrounding much of the ovary, history of surgery / PID)
  	(3) Cystic neoplasm of fallopian tube (more solid components)
  	(4) Paraovarian cyst with blood clot (resolution of clot on follow-up sonogram)
  	(5) Exophytic complex ovarian mass

Pelvic Inflammatory Disease

• = PID

• = acute clinical syndrome associated with ascending spread of microorganisms ( canalicular spread ) from vagina / cervix to uterus, fallopian tubes, and adjacent pelvic structures, not related to surgery / pregnancy

  Incidence:	10% of women in reproductive age (17% in Blacks); 1 million American women/year
  Risk factors:	early age at sexual debut, multiple sexual partners, history of sexual transmitted disease, douching
  Predisposed:	formerly married > married > never married; intrauterine contraceptive device (1.5 4-fold increase in risk)
  Etiology:	(a) bilateral: venereal disease, IUD, S/P abortion
  	(b) unilateral = nongynecologic: rupture of appendix, diverticulum, S/P pelvic surgery

• Organism:

  • Chlamydia trachomatis + Neisseria gonorrhoeae (>50% with high prevalence of coinfection) damage protective barrier of endocervical canal with spread to tubes (30 50%) producing fibrosis + adhesions

  • Aerobes: Streptococcus, Escherichia coli, Haemophilus influenzae

  • Anaerobes: Bacteroides, Peptostreptococcus, Peptococcus

  • Mycobacterium tuberculosis (hematogenous)

  • Actinomycosis in IUD users

    • = chronic suppurative infection characterized by multiple abscesses, abundant granulation tissue, fibrosis

  • Herpesvirus hominis type 2, Mycoplasma

May be associated with:

Fitz-Hugh-Curtis syndrome (= gonorrheal perihepatitis)

• usually bilateral lower abdominal pain (due to peritoneal irritation)

• abnormal vaginal discharge / uterine bleeding

• dysuria, dyspareunia, nausea, vomiting

• lower abdominal + adnexal + cervical motion tenderness

• fever, leukocytosis, elevated ESR

• elevated blood level of C-reactive protein

• MR:

  • ill-defined hyperintense area on fat-suppressed T2WI + intense enhancement on contrast-enhanced fat-suppressed MR (= extent of inflammation)

    Dx:	clinically, laparoscopically
    	Imaging employed only to differentiate between medical + surgical condition!
    Cx:	(1) Infertility due to tubal occlusion (25%): 8% after single episode, 20% after 2 episodes, 40% after 3 episodes of PID
    	(2) Ectopic pregnancy (6 as frequent)
    	(3) Chronic pelvic pain (from pelvic adhesions)
    Prognosis:	infertility, ectopic pregnancy, chronic pelvic pain
    DDx:	acute appendicitis, endometriosis, hematoma of corpus luteum, ectopic pregnancy, paraovarian cyst

P.1058

Endometritis

• endometrial prominence

• small amount of fluid within uterine lumen

• gas reflection within uterine cavity (most specific)

• pain over uterus

Postpartum Endometritis

Incidence:	2 3% of vaginal deliveries; up to 85% of cesarean sections
Associated with:	prolonged labor, premature rupture of membranes, retained clots, retained products of conception

• fever (most common cause of postpartum fever)

• normal ultrasound

• thickened heterogeneous endometrium

• intracavitary fluid

• intrauterine air

Salpingitis

• NOT depicted by imaging techniques

• often beginning during / immediately after menstruation (due to less effective barrier of mucus at cervix)

• MR:

  • thickened wall of dilated fallopian tube + tubal contents of low signal intensity on T2WI

  • debris / hemorrhage in fluid component most conspicuously hypointense to urine on heavily T2WI

Salpingitis Isthmica Nodosa

Etiology:	unknown; commonly associated with pelvic inflammatory disease, infertility, ectopic pregnancy
Location:	uni- / bilateral

• nodular thickening of isthmic portion of tube

• irregularity + multiple diverticula / obstruction of isthmic portion of fallopian tube on HSG

Hydro- / hemato- / pyosalpinx

• = continued secretion of tubal epithelium into lumen of a fallopian tube obstructed at two sites

  Cause:	infection, endometriosis, adhesions, microtubal surgery, ectopic pregnancy
  Location:	ampullary / infundibular portion of tube

• undulating / folded tubular structure in extraovarian location filled with sterile fluid / debris / pus

• short linear echoes protruding into lumen (= tall ramified mucosal plicae)

• longitudinal folds in ampullary portion

• US (sensitivity of 34%):

  • thickened fluid-filled tubes

• HSG:

  • absence of peritoneal spill

  • dilated ampullary portion of tube (visualization of mucosal folds confirm intratubal contrast accumulation) (DDx: contrast collection adjacent to tubes due to peritubal adhesions)

• MR:

  • high-signal intensity of fluid on T1WI due to hematosalpinx

  • well-enhancing wall, thicker than in hydrosalpinx

• Cx: tubal torsion

Sexually Transmitted Diseases (STD)

Chlamydia	33%	Human papillomavirus (warts)	6.0%
Trichomoniasis	25%	Genital herpes simplex	4.0%
Nonspecific urethritis	10%	Hepatitis B virus	1.2%
Gonorrhea	9%	Syphilis	1.0%
Mucopurulent cervicitis	8%	HIV	0.3%

DDx:

dilated uterine / ovarian vein, TOA, neoplasm, endometrioma, developing follicle

Tuboovarian abscess (TOA)

Cause:	sexually transmitted disease, IUD (20%), diverticulitis, appendicitis, pelvic surgery, gynecologic malignancy
Organism:	anaerobic bacteria become dominant
Location:	usually in posterior cul-de-sac extending bilaterally

• multilocular complex mass often with debris, septations, irregular thick wall

• may contain fluid-fluid levels or gas

• intense contrast enhancement of abscess wall

  DDx:

  endometrioma, ovarian tumor, infected cyst, abscess from other sources (eg, Crohn disease, appendicitis)

Pena-Shokeir Phenotype

= autosomal recessive syndrome (45% sporadic, 55% familial) characterized by fetal akinesia

Cause:	decreased / absent fetal motion secondary to abnormalities of fetal muscle / nerves / connective tissue ( fetal akinesia deformation sequence )
Time of first detection:	16 18 weeks MA

@ Spine:	scoliosis, kyphosis, lordosis
@ Thorax:	pulmonary hypoplasia, cardiac anomalies
@ Kidney:	renal dysplasia
@ Limbs:	limited movement, knee + hip ankylosis (arthrogryposis), abnormal shape + position, demineralization, camptodactyly, clubfeet

• craniofacial anomalies

• polyhydramnios

• IUGR

• short umbilical cord

  Prognosis:	still birth
  DDx:	multiple pterygium syndrome, Neu-Laxova syndrome, restrictive dermopathy, Larsen syndrome, trisomies 13 + 18

Pentalogy of Cantrell

= sporadic very rare abnormality

Cause:

failure of lateral body folds to fuse in the thoracic region with variable extension inferiorly

• Omphalocele + defect of lower sternum

• Ectopia cordis

• Deficiency of anterior diaphragm (herniation of intraabdominal organs into thoracic cavity is rare)

• Deficiency of diaphragmatic pericardium

• Cardiovascular malformation: atrioventricular septal defect (50%), VSD (18%), tetralogy of Fallot (11%)

Associated with:

trisomies

• exteriorization of heart

  Prognosis:

  death within a few days after birth

P.1059

Peritoneal Inclusion Cyst

• = PERITONEAL PSEUDOCYST = ENTRAPPED OVARIAN CYST

  Cause:	from previous abdominal surgery (time delay of 6 months to 20 years) / trauma / pelvic inflammatory disease / endometriosis
  Pathogenesis:	extensive pelvic adhesions result in impaired peritoneal clearing of fluid normally produced by an active ovary
  Path:	cyst adherent to surface of ovary
  Histo:	cyst lined by hyperplastic mesothelial cells + fibroglandular tissue with chronic inflammation

• single / multiloculated cyst contiguous with ovary

  Cx:	infertility
  Rx:	surgery (30 50% risk of recurrence)
  DDx:	paraovarian cyst (ovoid cyst outside ovary), hydrosalpinx (visible folds, located outside ovary), ovarian neoplasm, lymphangioma

Perlman Syndrome

= rare fetal overgrowth disorder characterized by visceromegaly, renal lesions, distinctive facial features, high neonatal mortality

Cause:

? autosomal recessive with reported abnormalities of chromosome 11

• generalized muscle hypotonia

• pancreatic islet cell hyperplasia

• hypertrophy of islets of Langerhans

• cystic hygroma

• fetal macrosomia

• macrocephaly

• agenesis of corpus callosum

• choroid plexus cyst

• nephromegaly + hydronephrosis

• hepatomegaly

• cryptorchidism

• polyhydramnios

  Prognosis:

  death within first few days of life

Placental Abruption

• = PLACENTAL HEMORRHAGE

• = premature separation of placenta from the myometrium secondary to maternal hemorrhage into decidua basalis between 20th week and birth

  Incidence:	0.5 1.3% of gestations
  Risk factors:	mnemonic: VASCULAR
  	Vascular disease + hypertension
  	Abruption (previous history)
  	Smoking
  	Cocaine
  	Unknown (idiopathic)
  	Leiomyoma
  	Anomaly (fetal malformation)
  	Reckless driving (trauma)
  Associated with:	intraplacental infarction / hematoma

• vaginal bleeding (80%): bright red (acute), brownish red (chronic)

• abdominal pain (50%)

• consumptive coagulopathy = DIC (30%)

• uterine rigidity (15%)

• Echogenicity of hemorrhage:

  • hyperechoic / isoechoic hematoma (initially difficult to distinguish from placenta):

    • abnormally thick + heterogeneous placenta (if blood isoechoic)

  • hypoechoic / complex collection between uterine wall + placenta in 50% within 1 week (hematoma / placental infarction)

  • anechoic collection within 2 weeks

• A normal ultrasound does not rule out abruption if

  • separation occurs WITHOUT hematoma

  • hematoma isoechoic to placenta

• Prognosis:

  • Only large hematomas (occupying >30 40% of the maternal surface) result in fetal hypoxia

  • Abruptions with contained hematoma have worse prognosis

  • Responsible for up to 15 25% of all perinatal deaths

  • Normal term deliveries in 27% of hematomas detected >20 weeks GA

  • Normal delivery in 80% of intrauterine hematomas detected <20 weeks GA

Cx:	(1) Perinatal mortality (20 60%), up to 15 25% of all perinatal deaths
	(2) Fetal distress / demise (15 27%)
	(3) Premature labor + premature delivery (23 52%) (3-fold increase)
	(4) Threatened abortion during first 20 weeks
	(5) Infant small-for-gestational age (6 7%)
DDx:	(1) Normal draining basal veins
	(2) Normal uterine tissue
	(3) Retroplacental myoma
	(4) Focal contraction
	(5) Chorioangioma
	(6) Coexistent mole

Retroplacental Hemorrhage (16%)

• = ABRUPTIO PLACENTAE

• = accumulation of blood beneath placenta

  Pathophysiology:	high-pressure bleed due to rupture of spiral arteries; hemorrhage may dissect into placenta / myometrium; associated with hypertension + vascular disease
  Incidence:	4.5%; 16% of all placental abruptions

• external bleeding

• thickened heterogeneous appearing placenta (hematoma of similar echogenicity as placenta)

• rounded placental margins + intraplacental sonolucencies

Cx:	(1) Precipitous delivery
	(2) Coagulopathy
	(3) Fetal demise (accounts for 15 25% of all perinatal deaths); risk for fetal demise with hematomas >60 mL: 6% before 20 weeks GA; 29% after 20 weeks GA

Subchorionic Hemorrhage (79%)

• = MARGINAL PLACENTAL HEMORRHAGE

• = submembranous PLACENTAL HEMORRHAGE

P.1060




• = separation of chorionic membrane from decidua with accumulation of blood in subchorionic space (placental membranes are more easily stripped from myometrium than from placenta)

  Pathophysiology:	low-pressure bleed due to tears of marginal veins; associated with cigarette smoking
  Incidence:	79% of all placental abruptions; in 91% before 20 weeks MA

• may lead to vaginal hemorrhage after dissection through decidua (18% of all causes of 1st-trimester bleeding)

• placental margin detached from adjacent myometrium (60%):

  • separation / rounding of placental margin

  • elevation of chorioamnionic membrane

    • (DDx: incomplete chorioamnionic fusion during 2nd trimester, blighted twin)

• hematoma contiguous with placental margin (100%)

• predominant hemorrhage often separate from placenta, even on opposite side of placenta

  Prognosis:

  worsens with (1) increased maternal age, (2) earlier gestational age, (3) size of hematoma; 9% overall miscarriage rate; risk of fetal demise doubles once hematoma reaches 2/3 of circumference of chorion

Preplacental Hemorrhage

• = BREUS MOLE = SUBCHORIAL HEMORRHAGE

• = variant of placental abruption with progressive slow intracotyledonary bleeding

  Incidence:	in 4% of all placental abruptions
  Etiology:	massive pooling + stasis due to extensive venous obstruction
  Time of onset:	18 weeks MA

• total loss of normal placental architecture

• gelatinous character of placenta elicited by fetal movement / abdominal jostling

• severe symmetric IUGR

  Risk for fetal demise:

  67% overall; 100% for hematomas >60 mL

Placenta Accreta

• = underdeveloped decidualization with chorionic villi growing into myometrium

  Incidence:

  1:2,500 7,000 deliveries; in 5% of placenta previa patients

• Risk of placenta accreta vs. cesarean section:

  • in 10% of placenta previa; in 24% of placenta previa + 1 cesarean section; in 48% of placenta previa + 2 cesarean sections; in 67% of placenta previa + 4 cesarean sections

    Predisposed:	areas of uterine scarring with deficient decidua: previous dilatation + curettage, endometritis, submucosal leiomyoma, Asherman syndrome, manual removal of placenta, adenomyosis, increasing parity
    Associated with:	placenta previa (20%)

• Types:

  • PLACENTA ACCRETA (76%)

    • = chorionic villi in direct contact with myometrium

  • PLACENTA increta (18%)

    • = villi invade myometrium

  • PLACENTA percreta (6%)

    • = villi penetrate through uterine serosa

• US (78 86% sensitive, 92 94% specific):

  • thinning to <1 mm / absence of hypoechoic myometrial zone between placenta + echodense uterine serosa / posterior bladder wall (retroplacental hypoechoic zone of decidua + myometrium + dilated periuterine venous channels measures 9.5 mm thick >18 weeks GA)

  • thinning / irregularity / focal disruption of linear hyperechoic boundary echo (= uterine serosa-bladder wall interface)

  • focal masslike elevations / extensions of echogenic placental tissue beyond uterine serosa

  • >6 irregular intraplacental lacunae (= vascular spaces)

• MR:

  • heterogeneous hyperintense placenta on T2WI

  • interruption of junctional zone

  • focal thinning of myometrium

Cx:	(1) Retention of placental tissue
	(2) Life-threatening hemorrhage in 3rd stage of labor necessitating emergent hysterectomy
	(3) Persistent postpartum bleeding
	(4) Maternal death
Rx:	(1) Hysterectomy
	(2) Conservative measures: curettage, oversewing of placental bed, ligation of uterine arteries

Placenta extrachorialis

= chorionic plate smaller than basal plate; ie, the transition of membranous to villous chorion occurs at a distance from the placental edge that is smaller than the basal plate radius

Circummarginate Placenta

Incidence:

up to 20% of placentas

• No clinical significance

• placental margin not deformed

Circumvallate Placenta

• = attachment of fetal membranes form a folded thickened ring with underlying fibrin + often hemorrhage

  Incidence:	1 2% of pregnancies
  Cx:	premature labor, threatened abortion, increased perinatal mortality, marginal hemorrhage

Placenta Membranacea

= presence of well-vascularized placental villi in the peripheral membranes

Cause:

? endometritis, endometrial hyperplasia, extensive vascularization of decidua capsularis, previous endometrial damage by curettage

• repeated vaginal bleeding extending into 2nd trimester + abortion at 20 30 weeks

• postpartum hemorrhage

• thickened outline over whole gestational sac (0.2 3.0 cm)

• may show additional distinct disk of placenta

Placenta Previa

= abnormally low implantation of ovum with the placenta covering all / part of internal cervical os

P.1061

Incidence:

0.5% of all deliveries; 3 5% of all pregnancies are complicated by 3rd trimester bleeding; of these 7 11% are due to placenta previa; in 0.26% with unscarred uterus

• Risk for placenta previa vs. cesarean section:

  • 0.65% after 1 section, 1.8% after 2 sections, 3% after 3 sections, 10% after 4 sections

    Cause:

    defective decidual vascularization in areas of endometrial scarring causing compensatory placental thinning; placenta occupies a greater surface of the uterus with increased probability for encroachment upon internal os

• Predisposed:

  • Previous uterine incision (cesarean section, myomectomy)

  • Older women

  • Multiparous women

• painless vaginal bleeding in 93% (usually 3rd trimester / as early as 20 weeks)

• Types on clinical examination:

  • Central / total previa (1/3) = complete covering of internal os

  • Partial previa = internal os partially covered by placenta

  • Low-lying placenta = low placental edge without extension over internal os; palpable by examining finger

• US - False Positives (5 7%):

  • Placental migration / rotation

    • = differential growth rates between lower uterine segment + placenta

    • 63 93% will have normal implantation at term!

    • conversion to normal position: anterior wall > posterior wall of uterus

    • NO conversion if placenta attaches to both posterior + anterior walls

  • Overfilled urinary bladder

    • bladder-induced compression leads to apposition of the lower anterior + posterior uterine walls (cervical length >3.5 4 cm) simulating a placenta previa

  • Focal myometrial contraction (myometrial thickness

    • >1.5 cm) in the region of the lower uterine segment

      mnemonic:

      ABCD and F

      • Abruption (may mimic placenta previa)

      • Bladder (must be empty)

      • Contraction (may have to wait 15 20 minutes)

      • Dates (be wary in 1st half of pregnancy)

      • Fibroid

• US - False Negatives (2%):

  • Obscuring fetal head

    • remedied by Trendelenburg position / gentle upward traction on fetal head

  • Lateral position of placenta previa

    • remedied by obtaining oblique scans

  • Blood in region of internal os

    • mistaken for amniotic fluid

Cx:	(secondary to premature detachment of placenta from lower uterine segment)
	(1) Maternal hemorrhage (blood from intervillous space)
	(2) Premature delivery
	(3) IUGR
	(4) Perinatal death (5%)
Rx:	precludes vaginal delivery + pelvic examination

Placental site trophoblastic disease

= very rare neoplasm (? type of choriocarcinoma)

Path:	microscopic tumor / diffuse nodular replacement of myometrium
Histo:	proliferation of predominantly intermediate trophoblasts but no syncytio- or cytotrophoblasts

• abnormal bleeding / amenorrhea

• low -hCG levels (due to lack of syncytiotrophoblastic proliferation)

• cystic / solid lesions central component

• myometrium usually invaded

Prognosis:	benign / highly malignant course
Rx:	hysterectomy

Postmaturity Syndrome

= inability of aging placenta to support demands of fetus

Incidence:

in 15% of all postterm gravidas

• meconium-stained amniotic fluid

• grade 3 placenta (in 85%), grade 2 (in 15%), grade 1 (in 0%)

• decreased subcutaneous fat + wrinkling of skin

• long fingernails

• decreased vernix

Cx:

meconium aspiration, perinatal asphyxia, thermal instability

Postterm Fetus

= fetus undelivered by 42nd week MA

Incidence:	7 12% of all pregnancies
Risk of perinatal mortality:	2-fold at 43 weeks MA, 4 6-fold at 44 weeks MA

Preeclampsia

= TOXEMIA OF PREGNANCY

Incidence:

5% of pregnancies, typically during 3rd trimester

• Clinical triad:

  • pregnancy-induced / -aggravated hypertension

  • proteinuria

  • peripheral edema + weight gain

Histo:

blunted invasion of vasa media of spiral arterioles + focal vasculitis + atheromatous degeneration + fibrin deposits in intima of maternal placental arterioles

• heavy calcium deposition (in areas of placental degeneration)

• IUGR (6% with late-onset preeclampsia, 18% with early-onset preeclampsia)

• Cx:

  • @ CNS

  • @ Liver: hematoma, infarction

  • @ Kidney

Eclampsia

• convulsions + coma

Premature Rupture of Membranes

• = spontaneous rupture of chorioamnionic membranes before the onset of labor

• Types:

  • Preterm premature rupture of membranes (PPROM) <37 weeks GA

  • Term premature rupture of membranes (TPROM) >37 weeks GA

    P.1062

    
    

    Incidence:	overall 2.1 17.1%; PPROM 0.9 4.4%; in 29% of all preterm deliveries; in 18% of all term deliveries
    Risk of recurrence:	21% of women with PPROM
    Cause:	? infection of membranes

• Cx:

  • TPROM:

    • >24 hours may result in intrapartum fever

    • >72 hours may result in chorioamnionitis + still-birth

  • PPROM: respiratory distress syndrome (9 43%), neonatal sepsis (2 19%)

Primary Ovarian Choriocarcinoma

= nongestational CHORIOCARCINOMA

Incidence:	extremely rare; 50 cases in world literature
Age:	<20 years

• elevated serum hCG

• predominantly solid tumor with areas of hemorrhage + necrosis

  DDx:

  metastasis to ovary from gestational choriocarcinoma (reproductive age)

Seckel syndrome

• = BIRD-HEADED DWARFISM

• = rare autosomal recessive disorder (44 cases)

• proportionate postnatal short stature

• characteristic stance: slight flexion of hips and knees

• mental retardation

• simian crease

• cryptorchidism

• @ Skull

  • severe microcephaly

  • receding forehead, large beaked nose, micrognathia

• @ Skeleton

  • dislocation of radial head + hypoplasia of proximal end of radius

  • absence of phalangeal epiphysis

  • clinodactyly of 5th digit

  • gap between 1st and 2nd toe

  • hip dislocation

  • hypoplasia of proximal fibula

  • absence of patella

  • 11 pairs of ribs

• OB-US:

  • severe IUGR

  • oligohydramnios

  • decreased bone length (femur, tibia, fibula)

  • decreased AC, HC

Serous Ovarian Tumor

• Most common neoplasm in benign + malignant category

  Incidence:	30% of all ovarian tumors; 60 80% of all malignant ovarian neoplasms
  Path:	areas of solid tissue components + hemorrhage and necrosis (more common in malignant tumors)
  Histo:	lined by tall columnar epithelial cells (like fallopian tubes), filled with serous fluid, psammoma bodies (= microscopic calcifications in up to 30% of malignant tumors);
  Age:	20 50 years (malignant variety later)

Serous Cystadenoma (60%)

• Second most common benign tumor of the ovary (after dermoid cyst); 20% of all benign ovarian neoplasms

• usually unilocular (occasionally multilocular) thin-walled cyst up to 20 cm in diameter

• only small amount of solid tissue: occasional septum / mural nodule (papillary projections in 9%)

• bilateral in 7 20 30%

Borderline Malignant Serous Cystadenoma (15%)

• papillary projections within cyst (in 67%)

Serous Cystadenocarcinoma (25%)

• = 60 80% of all ovarian carcinomas

• multilocular cyst with large amount of solid tissue: papillomatous excrescences within cyst (= papillary serous carcinoma) in 38%

• may have calcifications

• bilateral in 50 70%

• loss of capsular definition + tumor fixation

• ascites secondary to peritoneal surface implantation

• lymph node enlargement (periaortic, mediastinal, supraclavicular)

• CT:

  • psammomatous calcifications (12%)

Sertoli Stromal Cell Tumor of Ovary

= androblastoma = ARRHENOBLASTOMA

Origin:	from hilar cells of ovary
Incidence:	<0.5%
Age:	any age; most common in 2nd 3rd decade
Histo:	components of Sertoli cells, Leydig cells, fibroblasts

• androgenic

• hypoechoic mass simulating fibroid

• may have cystic / hemorrhagic degeneration

Sertoli-Leydig Cell Tumor

• Most common virilizing tumor of ovary!

Incidence:	0.5% of all ovarian neoplasms
Age peak:	25 45 years (range 15 66 years); 75% occur in patients <30 years of age
Path:	solid cystic areas; hemorrhage is rare
Histo:	6 subtypes; tissues are so varied that it is frequently confused with other tumors

• virilization (30%): amenorrhea, male secondary sexual characteristics

• estrogenic (20%); no hormonal manifestations (50%)

• small mass often difficult to visualize by US / CT

• solid mass with cystic components (hemorrhage necrosis)

• well-defined hypoechoic mass intratumoral cysts

• calcifications are unusual

• contrast enhancement

• unilateral (95%), up to 27 cm in diameter

Cx:	malignant transformation in 10 18%
Prognosis:	good when detected as stage I (in 92%); tend to recur soon after initial diagnosis
DDx:	granulosa cell tumor (spongelike multicystic with areas of hemorrhage)

P.1063

Single Umbilical Artery

• = Two-vessel cord

• Etiology:

  • Primary agenesis of one umbilical artery (usually first appears in 5th menstrual week)

  • Secondary atrophy / atresia of one umbilical artery

  • Persistence of original single allantoic artery of the body stalk

    Incidence:

    0.2 1% of singleton births; 5% in dizygotic twins; 2.5% in abortuses; increased incidence in trisomy D / E, diabetic mothers, White patients, spontaneous abortions

• Associated with:

  • Congenital anomalies (21%):

    • CHD (most frequent): VSD, conotruncal anomalies

    • Abdomen: ventral wall defect, diaphragmatic hernia

    • CNS: hydrocephalus, holoprosencephaly, spina bifida

    • GU: hydronephrosis, dysplastic kidney

    • Esophageal atresia, cystic hygroma, cleft lip

    • Polydactyly, syndactyly

  • IUGR

  • Premature delivery

  • Perinatal mortality (20%): stillbirth (66%)

  • Marginal (18%) / velamentous (9%) insertion of umbilical cord

  • Chromosomal anomalies (67%): trisomy 18 > trisomy 13 > Turner syndrome > triploidy

• Site: left artery slightly more often absent than right

• axial view of cord shows 2 vessels

• single umbilical artery nearly as large as umbilical vein (umbilical vein-to-umbilical artery ratio < 2)

• incurvation of distal aorta toward common iliac artery on the side of patent umbilical artery

• ipsilateral hypoplastic common iliac artery

• absence of abdominal portion of umbilical artery on ipsilateral side of missing umbilical artery

• color flow imaging permits earlier (15 16 weeks) + more confident diagnosis

• Prognosis:

  • 4-fold increase in perinatal mortality (14%) with concurrent major abnormality

  • Isolated single umbilical artery does not affect clinical outcome

• DDx:

  • Normal variant = two arteries at fetal end may fuse near placental end into single umbilical artery (umbilical arteries normally unite with allantoic artery near placental insertion)

  • Arterial convergence of 2 into 1 umbilical a.

Stein-Leventhal Syndrome

= POLYCYSTIC OVARY SYNDROME

Incidence:	2.5% of all women
Etiology:	deficient aromatase activity (catalyst for conversion of androgen into estrogen) results in androgen excess; exaggerated pulsatile release of LH stimulates continued ovarian androgen secretion at the expense of estradiol; reduction of local estrogen impairs FSH activity; this results in accumulation of small- + medium-sized atretic follicles without final maturation into graafian follicles
Path:	pearly white ovaries with multiple cysts below the capsule, which are lined by a hyperplastic theca interna layer showing pronounced luteinization; granulosa cells are absent / degenerating; corpora lutea are absent
Age:	late 2nd decade
Associated with:	Cushing syndrome, basophilic pituitary adenoma, postpill amenorrhea, virilizing ovarian / adrenal tumor

• reduced infertility / sterility

• mild facial / severe generalized hirsutism

• obesity

• secondary amenorrhea (most common cause)

• menstrual irregularities / oligomenorrhea

• cystic acne

• cephalic hair loss

• periodic abdominal discomfort

• elevated LH levels without LH surge + normal / decreased FSH = increased LH/FSH ratio

• elevated androstenedione / testosterone levels

• elevated estrone / estradiol

• bilaterally enlarged ovaries >15 cm³ (70%)

• normal ovarian size (in 30%), polycystic ovaries have a volume of 6 30 cm³

• excessive number of developing follicles:

  • multiple (more than 5) small cysts of 5 8 mm in subcapsular location (40%)

  • hypoechoic ovaries (25%)

  • isoechoic ovaries (5%)

Cx:	endometrial cancer <40 years of age (due to unopposed chronic estrogen stimulation)
DDx:	ovaries in congenital adrenal hyperplasia, normal ovaries
Rx:	(1) Ovulation induction with clomiphene (Clomid ) / menotropins (Pergonal )
	(2) Wedge resection (transient effect only)

Steroid Cell Tumors

• = LIPID / LIPOID CELL TUMORS

• = characterized by cells resembling typical steroid-secreting cells (lutein cells, Leydig cells, adrenocortical cells)

Incidence:	0.1 0.2% of all ovarian tumors
Age:	wide range of ages; usually 5th 6th decade
Types:	stromal luteoma, Leydig / hilus cell tumor, steroid tumor not otherwise specified
Path:	usually <3 cm yellow nodule with rich vascularity; rarely cystic
Histo:	abundant clear cytoplasm + varying amounts of lipid resembling adrenocortical cells

• virilizing (majority): amenorrhea, hirsutism

• Cushing syndrome (rare)

• unilateral solid tumor

• cystic change / necrosis (rare)

• MR:

  • high-signal intensity on T1WI (lipid content)

  • intense contrast enhancement (high tumor vascularity)

Prognosis:

clinically malignant (33%)

Stuck Twin

• = one twin with IUGR residing within an oligo- / anhydramniotic sac of a diamniotic twin pregnancy

P.1064




• amnion invisible secondary to close contact with fetal parts

• fetus fixed relative to the uterine wall without change during shift in maternal position

• diminished / absent active fetal motion

• absence of intermingling of fetal parts between twins

Prognosis:

fetal death in utero

Succenturiate Lobe of Placenta

• = ACCESSORY LOBE

• = separate mass of chorionic villi connected to main placenta by vessels within membrane

Cause:	placental villi atrophy in area of inadequate blood supply + proliferate in two opposite directions (trophotropism) with fetal vessels remaining at the site of villous atrophy
Incidence:	0.14 3%
Cx:	(1) Retained in utero with postpartum hemorrhage
	(2) Placenta previa with intrapartum hemorrhage
	(3) Vasa previa = succenturiate vessels traversing internal os, which may rupture resulting in fetal blood loss

Teratoma of Neck

• = germ cell tumor of neck (oropharynx, tongue)

• polyhydramnios in 30% (from esophageal obstruction)

• complex mass in cervical region

Cx:	airway obstruction
DDx:	cystic hygroma, goiter, branchial cleft cyst, cervical meningocele, neuroblastoma of neck, hemangioma of neck

Teratoma of Ovary

= immature derivatives of all 3 germ cell layers

Incidence:	rare
Age:	childhood / adolescence

• cystic / complex mass (most frequently)

• usually large solid mass with internal echoes

Theca Cell Tumor of Ovary

• = THECOMA = FIBROSED THECOMA = FIBROTHECOMA

• = spectrum of tumors with varying amounts of theca cells (lipid-rich with estrogenic activity) + fibroblasts

Histo:	theca cells contain intracellular lipids
Incidence:	4 6% of all ovarian neoplasms; 50% of all gonadal-stromal tumors
Age:	mean age of 59 years; >30 years (30%); postmenopausal (70%)
May be associated with:	endometrial hyperplasia / ca.

• estrogenic with uterine bleeding

• hypoechoic mass with sound attenuation

• unilateral

• MR:

  • low signal intensity on T2WI for fibrotic component

  • high signal intensity on T2WI for components with little / no fibrosis

Luteinized Thecoma

= rare subtype of fibrothecomas associated with virilization rather than estrogenic activity

Theca Lutein Cyst

= multiple bilateral corpus luteum cysts as a form of ovarian hyperstimulation

• associated with abnormally high levels of -hCG secondary to

  • multiple gestations

  • gestational trophoblastic disease (in 40%)

  • fetal hydrops

  • pharmacologic stimulation with -hCG

  • normal pregnancy (uncommon)

• multiloculated cysts, often bilateral

• ovaries several cm in size

• involution within a few months after source of gonadotropin removed

Torsion of Ovary

• = result of rotation of ovary tube on its axis producing arterial, venous, and lymphatic stasis

• Cause:

  • Enlarged ovary (large cyst / tumor, paraovarian cyst)

    • Benign cystic teratoma is most common neoplasm

  • Hypermobility of adnexa (more frequent in younger children + during pregnancy), excessively long mesosalpinx, tubal spasm

• Associated with: ipsilateral ovarian lesion (in 50 81%)

• Age: usually affects prepubertal girls, may occur prenatally, increased risk during pregnancy

• Path: hemorrhagic necrosis

• Pathophysiology: circulatory stasis is initially venous; becomes arterial as torsion + edema progress; complete obstruction of arterial blood supply leads to gangrenous + hemorrhagic necrosis

• gradual / sudden onset of severe lower abdominal pain

• nausea, vomiting, fever

• palpable mass in 50%

• Location: R:L = 3:2 (? protective effect of sigmoid mesentery)

• deviation of uterus toward side of torsion (36%)

• engorgement of blood vessels on side of torsion

• small amount of clear (64%) / hemorrhagic (8%) ascites

• US:

  • markedly enlarged hypo- / hyperechoic midline mass

  • multiple peripheral cysts (= transudation of fluid into follicles) measuring 8 12 mm in diameter (64 74%)

  • good sound transmission (vascular engorgement + stromal edema)

  • free fluid in cul-de-sac (32%)

  • absence of arterial Doppler waveforms (not always reliable due to dual blood supply by ovarian + uterine artery

  • whirlpool sign = twisted ovarian pedicle

  • complex mass (if secondary to cyst / tumor)

• CT:

  • amorphous / tubular masslike structure (84%) between adnexal mass + uterus due to tube thickening >10 mm

    N.B.: the diameter of the normal tube measures up to 4 mm at isthmic, 8 mm at ampullary, 10 mm at infundibular portion

  • whorled structure abutting ovarian mass (= twisted vascular pedicle)

  P.1065

  
  

  • cystic ovarian mass with smooth wall thickening (76%):

    • >10 mm thickness suggests hemorrhagic infarction

  • obliteration of fat planes around torsed ovary

  • attenuation >50 HU means hemorrhagic infarction on NECT

  • lack of enhancement of solid component

• MR:

  • thickened cyst wall / mural nodule

  • high signal intensity on fat-suppressed T1WI (= hemorrhage / vascular congestion)

  • no enhancement of solid component on contrast-enhanced dynamic subtraction MR

  • tube thickening (SAG image improves detection)

• Prognosis:

  • Spontaneous detorsion is common (history of prior similar episodes = intermittent torsion)

  • Infection of torsed ovary with local peritonitis bowel obstruction

• Rx: immediate surgery (most ovaries not salvageable)

Triploidy

• = 69 chromosomes

• Incidence: 1% of conceptions; 0.04% of 20-week fetuses

• NO obvious pattern!

• early severe asymmetric IUGR (MOST PROMINENT FEATURE); cephalocorporal disproportion

• oligohydramnios

• large hydropic placenta with scattered vesicular spaces (partial hydatidiform mole)

• congenital heart disease: ASD, VSD

• brain anomalies: hydrocephalus, holoprosencephaly, neural tube defect

• cleft lip / palate

• syndactyly of fingers

• omphalocele

• renal abnormalities

• Prognosis: most ending in spontaneous abortion

Trisomy 13

• = Patau SYNDROME

• Incidence: 1:5,000 live births

• @ OB: severe IUGR, hydramnios

• @ CNS: alobar holoprosencephaly, posterior encephalocele, neural tube defect

• @ Face: midline labial cleft, proboscis, hypotelorism, cyclopia, anophthalmia

• @ Skeleton: postaxial polydactyly, rocker-bottom foot

• @ Heart: (CHD in 90%) VSD, echogenic chordae tendineae, hypoplastic ventricle, tetralogy of Fallot, transposition

• @ Kidney: polycystic kidney, horseshoe kidney

• @ GI: omphalocele (occasionally)

• Prognosis: few infants live more than a few days / hours; death by 3 months of age

• DDx: Meckel-Gruber syndrome

Trisomy 18

• = EDWARDS SYNDROME

• Incidence: 1:3,000 live births

• triple-marker screening test:

• decreased maternal alpha-fetoprotein

• decreased hCG (DDx: increased in Down syndrome)

• decreased estriol

• no anomalies (14%) on initial 2nd-trimester sonogram only due to incomplete / early gestational age

  @OB:	severe symmetric IUGR (28% <24 weeks MA), single umbilical artery (30%), polyhydramnios (occasionally)
  @Face:	micrognathia, hypotelorism, facial cleft (10 40%)
  @Head:	strawberry-shaped head (50%), cystic hygroma
  @CNS:	holoprosencephaly, choroid plexus cyst (30 51 75%), cerebellum 10th percentile with prominent cisterna magna (45%), myelomeningocele
  @Hand:	clenched hand with overlapping of index finger (33%, HIGHLY CHARACTERISTIC), abnormal wrist position (27%)
  @Arm:	shortened radial ray, clubbed forearm
  @Foot:	clubbed foot, rocker-bottom foot
  @Heart:	(CHD in 90%) VSD (82%), ASD (8%), complete AV canal, DORV
  @GI:	diaphragmatic hernia, omphalocele (30 40%), TE fistula
  @Kidney:	polycystic kidney, horseshoe kidney, UPJ obstruction

• Prognosis: usually delivered by emergency cesarean section due to IUGR + fetal distress, if not detected prenatally; death by 1 year of age

Twin Embolization Syndrome

• = rare complication of monochorionic pregnancy following the death of one twin whose blood pressure falls to zero

• Pathophysiology:

  • Acute reversal of transfusion to co-twin at time of intrauterine demise of one twin with ischemic changes in survivor

  • Embolization of thromboplastin-enriched blood / detritus from the dead to the living twin through vascular anastomoses in placenta

• Embolized organs: CNS (72%), GI tract (19%), kidneys (15%), lungs

• ventriculomegaly, cortical atrophy, porencephalic cyst, cystic encephalomalacia within 2 weeks of death of co-twin

Twin-Twin Transfusion Syndrome

• = fetofetal TRANSFUSION SYNDROME= monovular TWIN TRANSFUSION = INTRAUTERINE PARABIOTIC SYNDROME

• = complication of monozygotic twinning with one placenta or one fused placenta of mono- / dizygotic twins

  Incidence:	5 18% of twin pregnancies; 5 15% of monozygotic multiple pregnancies; 15 30% of monochorionic twin gestations
  Cause:	unbalanced intrauterine shunting of blood through shared placental vessels
  Time of onset:	2nd trimester with discordant amniotic fluid volumes
  Path:	large communication between arterial circulation of one twin and venous circulation of the other twin through arteriovenous shunt (= common villous district) deep within placenta

P.1066




• discrepant amniotic fluid volume (75%)

• discordant BPD by >5 mm (57%)

• discordant estimated fetal weight >25% (67 100%)

• DONOR TWIN

  • = twin that transfuses the recipient twin + remains itself underperfused

  • anemia + hypovolemia

  • high output cardiac failure + hydrops (rare)

  • oligohydramnios (75 80%) / stuck twin = severe oligohydramnios (60%) from oliguria

  • intrauterine growth restriction (common) diagnosed by discordant EFW of >25%

  • morphologically normal

• RECIPIENT TWIN

  • polycythemia (higher hemoglobin)

  • plethora = hypervolemia (volume overload)

  • polyhydramnios (70 75%) from increased fetal urination

  • fetal hydrops (10 25%): pericardial + pleural effusions, ascites, skin thickening

  • organomegaly

  • fetus papyraceus = macerated dead fetus

  • velamentous cord insertion (64%)

• Prognosis: 80 100% perinatal mortality if presenting <28 weeks MA and left untreated

• Cx: amniorrhexis, preterm labor

• Rx: elective termination, volume-reduction amniocentesis of polyhydramniotic sac (decreasing mortality rates to 34%), selective feticide, laser ablation of vascular anastomoses

• DDx: IUGR of one dizygotic twin (two separate placentas, two different sexes)

Uterine Anomalies

• = anomalies of fusion of paramesonephric duct (= m llerian duct) completed by 18th week of fetal life

• Incidence: 0.1 3%

• Uterine anomalies are found in 9% of women with infertility / repeated spontaneous abortions!

• 25% of women with uterine abnormalities have fertility problems!

• Associated with: urinary tract anomalies in 20 50%; possibly increased familial occurrence of limb reduction

• Embryology:

  • m llerian ducts develop at 5 6 weeks GA from coelomic epithelium and form uterovaginal canal by lateral fusion at 7 9 weeks GA

  • by 8 weeks the uterovaginal canal reaches the urogenital sinus at the m llerian tubercle while a vaginal plate develops distally resulting in vertical fusion (upper 2/3 to 4/5 of vagina are of m llerian duct origin, lower 1/3 or 1/5 of vagina originate from urogenital sinus)

• Classification:

  [classes in parenthesis refer to the classification of the American Fertility Society]

  • ARRESTED M LLERIAN DUCT DEVELOPMENT

    • bilateral: Uterine agenesis / hypoplasia (class I)

      • Incidence: 1:5,000

        Often associated with: vaginal agenesis / hypoplasia

        Age of detection: menarche

      • small uterus with small endometrial canal

      • poor zonal differentiation + abnormal T2-hypointense myometrium

    • unilateral: Unicornuate uterus = Uterus unicornis unicollis (class II)

      • with contralateral rudimentary horn

      • without rudimentary horn

      • Incidence: 3 6 13% of uterine anomalies

      • May be associated with: ipsilateral renal agenesis

      • infertility in 5 20%

      • ? pregnancy wastage

      • reduced uterine volume

      • asymmetric ellipsoidal uterine configuration

      • rudimentary horn may contain endometrium + may communicate with main uterine cavity

      • solitary fusiform banana-shaped uterine cavity with lateral deviation within pelvis terminating in a single fallopian tube on HSG

      • Cx: cryptomenorrhea within endometrium-containing rudimentary horn that does not communicate with endometrial cavity

    • Mayer-Rokitansky-K ster-Hauser syndrome

      • vaginal agenesis / hypoplasia of proximal + middle segments

      • intact normal ovaries + fallopian tubes

      • variable anomalies of uterus (agenesis / hypoplasia, uni- or bicornuate), urinary tract (renal agenesis, pelvic kidney in 40 50%), skeletal system (12%)

      • Frequency: 1:4,000 1:5,000

      • Cause: lack of m llerian development

      • normal external genitalia

      • shallow distal vaginal pouch (derived from urogenital sinus)

      • amenorrhea

      • cyclic pelvic pain (secondary to functioning endometrium within rudimentary uterine tissue) in 6 10%

      • 2nd most common cause of primary infertility

      • Rx: neovaginoplasty

  • TOTAL / PARTIAL FAILURE OF M LLERIAN DUCT FUSION = duplication defects = disorders of lateral fusion (75% of uterine anomalies)

    • Uterus didelphys (class III)

      • = complete duplication with 2 vaginas + 2 cervices + 2 uterine horns

      • May be associated with: renal agenesis

      • usually asymptomatic; frequently first diagnosed during routine obstetric sonography

      • two widely spaced uterine corpora, each with a single fallopian tube

      • separate divergent uterine horns

      • large fundal cleft

      • cervical duplication

      • transverse vaginal septum between upper one-third and lower two-third of vagina (occasionally with ipsilateral renal agenesis)

      • opacification of single deviated horn on HSG

        P.1067

        
        

        Cx: unilateral hydro- / hematocolpos (if transverse vaginal septum present) with reflux endometriosis

        Rx: surgery is rarely performed

    • Bicornuate uterus = uterus bicornis (class IV)

      • = lack of fusion of corpus

        • bicornis bicollis = complete with division down to internal os

        • bicornis unicollis = partial

      • external uterine fundal contour (MRI):

        • concave surface depression >2 cm deep

        • large fundal cleft

      • separation of uterine horns

      • intercornual angle of >75 105 (demonstrated on luteal-phase US in conjunction with HSG)

      • intercornual distance (= distance between maximum lateral extent of hyperintense endometrium on transaxial image) >4 cm

      • divider between cornua comprised of myometrium / fibrous tissue / both

      • fusiform shape of each uterine horn with lateral convex margins

      • discrepancy in size of the 2 uterine horns

      • elongation + widening of cervical canal + isthmus

      Laparoscopy:	typical external fundal indentation
      Cx:	repeated spontaneous abortions (frequently in 2nd 3rd trimester), premature rupture of membranes, premature labor, SGA infant, persistent malpresentations (transverse lie)
      Rx:	transabdominal surgery to fuse uterine horns (abdominal / open metroplasty)

• NONRESORPTION OF SAGITTAL UTERINE SEPTUM

  • Septate uterus (class V)

    • Most common anomaly (almost 50%) associated with reproductive failure in 67%

    • Path: septum may be composed of fibrous tissue (low-signal intensity), myometrium (intermediate-signal intensity), or both

    • convex / flat / minimally indented ( 1 cm) external fundal contour

    • distal portion of septum hypoechoic to myometrium (= fibrous tissue)

    • intercornual angle of 75 (MRI more accurate than HSG with its projectional problems)

    • duplication of uterine horns on HSG (DDx to bicornuate uterus unreliable)

    • endometrial canals completely separated by tissue isoechoic to myometrium extending into endocervical canal

    • Types:

      • Uterus septus

        • = complete septum extending to internal os

      • Uterus subseptus

        • = partial septum involving endometrial canal

        • Cx: 90% abortion rate (poor septal vascularity)

        • Rx: hysteroscopic metroplasty (= excision of septum)

    • Uterus arcuatus (class VI)

      

      Uterine Anomalies

      • Most common anomaly unassociated with reproductive failure

      • NO division of uterine horns

      • normal fundal contour

      • smooth indentation of fundal endometrial canal

      • increased transverse diameter of uterine cavity

      • single uterine canal with saddle-shaped fundus on HSG

  • INADEQUATE HORMONAL STIMULATION DURING FETAL DEVELOPMENT

    = DES (= diethylstilbestrol)-related abnormalities (class VII)

    • synthetic hormone used in 1950s + 1960s to prevent miscarriage

    • may cause abnormal uterine morphology (with decreased fertility)

    • increased risk of vaginal malignancy

    • Uterine hypoplasia

      • associated with DES exposure in utero

      • mean uterine volume = 50 cm³

    • T-shaped uterus

      • encountered in 15% of women exposed to DES in utero

      • low uterine volume

      • uterine fundus thinner than cervix

      • greater width than depth of corpus + fundus over cervix

      • T-shaped lumen on hysterosalpingogram

P.1068

Uterine Leiomyoma

• = FIBROID = MYOMA

• = benign overgrowth of smooth muscle + connective tissue

• Most common gynecologic neoplasm! Commonest cause for uterine enlargement after pregnancy!

  Incidence: in 20 25% of White women; in 50% of Black women; Black:White women = 3:1 9:1; 0.3 2.6% during pregnancy; account for 30% of all hysterectomies in USA

  Path: whorl-like trabeculated mesenchymal tumor surrounded by pseudocapsule; may outgrow its blood supply resulting in:

  • hyaline degeneration (>60%) = homogeneous eosinophilic bands / plaques of proteinaceous material in extracellular space

  • myxoid degeneration = gelatinous intratumoral foci of hyaluronic acid-rich mucopolysaccharides

  • cystic degeneration (4%) = extreme sequelae of edema

  • red / hemorrhagic / carneous degeneration

    = massive hemorrhagic infarction due to venous thrombosis / rupture of intratumoral arteries; often during pregnancy / during use of oral contraceptives

  • calcification (4%) = dense amorphous calcifications within hyalinized tissue

• Specific types:

  • Lipoleiomyoma (0.8%) = substantial amount of fat due to fatty metamorphosis

  • Myxoid leiomyoma = rare soft translucent mass due to abundant myxoid material between smooth muscle cells, may be clinically malignant

  • Intravenous leiomyomatosis = wormlike masses growing within pelvic veins

  • Benign metastasizing leiomyoma

  • Diffuse leiomyomatosis = development of innumerable small leiomyomas

    • symmetric enlargement of uterus

  • Peritoneal disseminated leiomyomatosis

    • frequently associated with pregnancy

    • multiple nodules on peritoneal surface

  • Histo: monoclonal proliferation of smooth muscle cells (NOT myometrial hyperplasia) separated by variable amounts of fibrous connective tissue

  • Hormonal dependency:

    • Higher estrogen:progesteron receptor ratio than in normal myometrium!

      • Growth during pregnancy in 15 32% by a mean volume of 12 6% within the 1st trimester (NOT during remainder of pregnancy)

        • The larger the myoma, the greater the likelihood of growth!

      • Growth under estrogen therapy

      • Shrinkage in puerperium + after menopause

  • Age: usually >30 years; rare in girls <18 years + in postmenopausal women

  • asymptomatic in 70 75%

  • palpable abdominopelvic mass

  • pelvic pressure:

    • urinary frequency (due to compression of bladder)

    • constipation (due to impingement on rectosigmoid)

  • pain (30%) due to acute hemorrhagic infarction + necrosis, torsion of pedunculated subserosal fibroid, prolapse of pedunculated submucosal fibroid

  • dysmenorrhea (= colicky pelvic pain with menstruation)

  • abnormal uterine bleeding:

    • menorrhagia / hypermenorrhea (= heavy + prolonged menstrual flow)

    • metrorrhagia (= uterine bleeding outside time of menstruation)

  • infertility (infrequent)

  • Location: mostly in fundus + corpus; cervix (3 8%); fallopian tube; broad ligament; ovary

  • Classification by location in uterus:

    • Intramural fibroid (within confines of uterine outline) in 95%

      • asymptomatic (mostly)

      • occasionally menorrhagia (due to interference with normal uterine contractility)

      • occasionally infertility (due to compression of interstitial portion of fallopian tube / distortion of endometrial cavity)

    • Subserosal / exophytic fibroid

      • usually asymptomatic

      • pain from infarction due to torsion

      • intraligamentous fibroid (lateral growth between folds of broad ligament)

        • simulates ovarian mass

        • occasionally infertility (due to compression of isthmic / ampullary portion of fallopian tube)

        • Cx: hydroureteronephrosis (due to compression of ureter)

      • parasitic fibroid = subserosal fibroid, which has become detached secondary to circulatory occlusion of vessels in pedicle; revitalized through omental / mesenteric blood supply

    • Submucosal fibroid (4 5 18%) = projecting into endometrial canal

      • Most frequently symptomatic type of fibroid:

        • dysmenorrhea, menorrhagia, infertility

        • increased prevalence of early abortion

        • Cx: hemorrhage, ulceration

      • (a) fibroid polyp (2.5%) = partial / complete extrusion of pedunculated submucosal fibroid into cervical canal / vagina

  • uterine enlargement

  • lobulated / nodular distortion of uterine outline (subserosal leiomyoma) + indentation of urinary bladder

  • distortion / obliteration of the contour of the uterine cavity (submucosal leiomyoma)

  • intramural soft-tissue mass (most frequent), usually multiple, solitary in 2%

  • speckled / ringlike / popcorn calcification

  • US (60% sensitivity, 99% specificity, 87% accuracy):

    • hypoechoic solid concentric mass (<33%) (= muscle component prevails)

    • echogenic attenuating mass (= dense fibrosis prevails)

    • sharp discrete refractory shadows (from borders between fibrous tissue and smooth muscle, margins of leiomyoma with normal myometrium, edges of whorls, bundles of smooth muscle)

    P.1069

    
    

    • anechoic features (secondary to internal degeneration: atrophic, hyaline, cystic, myxomatous, lipomatous, calcareous, carneous, necrobiotic, hemorrhagic, proteolytic degeneration)

    • acoustic shadowing (= calcifications)

    • Presence of a prominent artery by color Doppler suggests potential for growth during pregnancy

  • CT:

    • hypo- / iso- (usually) / hyperdense mass

    • deformed uterine contour

    • calcifications (common)

  • MR (86 92% sensitive, 100% specific, 97% accurate; most accurate + desirable for planning myomectomy):

    N.B.:

    subserosal, intramural and submucosal classification enabled by uterine zonal anatomy

    • @ Nondegenerated leiomyoma

      • well-circumscribed mass

        • of homogeneously low signal intensity on T2WI compared with myometrium (for leiomyoma with variable amounts of collagen)

        • isointense to surrounding uterus on T1WI

      • slightly higher signal intensity + contrast enhancement on T2WI (for cellular leiomyoma with little / no collagen)

    • @ Degenerated leiomyoma

      • Histo: hyaline fibrosis (60%), edema (50%), hemorrhage (10%), cystic degeneration (4%), microcalcifications (4%)

      • low signal intensity on T2WI (for hyaline / calcific degeneration)

      • high signal intensity on T2WI (for cystic degeneration) without enhancement of cystic areas

      • very high signal intensity on T2WI (for myxoid degeneration) with minimal enhancement

    • variable signal intensity on T1WI + low signal intensity on T2WI (for hyaline / coagulative necrosis)

    • peripheral / diffuse high signal intensity on T1WI (due to proteinaceous content of blood) + variable signal intensity on T2WI (for red degeneration)

  • hyperintense rim on T2WI in 33% (= pseudocapsule of dilated lymphatics / veins / edema)

  • enhancement pattern (usually later than myometrium): 65% hypointense, 23% isointense, 12% hyperintense to myometrium

• Hysterosalpingography (9% sensitive, 97% specific, 76% accurate)

• Cx:

  • Infertility in 35%

    • narrowing of isthmic portion of tube

    • impingement on endometrium interfering with implantation; infertility rates highest for submucosal leiomyomas

  • Complications during pregnancy

    • Significantly increased for myomas >6 cm in size / multiple in number / myomas >200 cm³ and when fibroid is retroplacental

    • increased frequency of spontaneous abortions

    • increased frequency of ectopic pregnancies

    • increased frequency of IUGR

    • preterm labor in 7% + premature rupture of membranes

    • placental abruption

    • uterine dyskinesia, uterine inertia during labor

    • dystocia, obstruction of birth canal during vaginal delivery (if near internal os)

    • abnormal presentation

    • postpartum hemorrhage

    • retained products of conception

  • Hydroureteronephrosis

  • Leg edema (due to compression of pelvic vessels)

  • Malignant transformation (in 0.2%)

• Rx: (1) Hysterectomy for pain, menorrhagia, visceral compression (after childbearing completed)

• (2) Myomectomy for 2nd trimester fetal loss / anemia due to hypermenorrhea / pelvic pain

• Submucosal leiomyomas may be treated with hysteroscopic myomectomy

• (3) Uterine artery embolization for symptomatic fibroids

• DDx of necrotic leiomyoma:

  • Ovarian mass (ovarian cyst, hemorrhagic cyst, endometrioma, cystic dermoid, cystadenoma, malignancy)

  • Ectopic interstitial pregnancy

  • Intrauterine gestational sac

  • Intrauterine fluid collection

  • Hydatidiform mole

  • Myometrial contraction (lasts for 15 30 minutes)

  • Cervical tumor

  • Hematoma of broad ligament

• DDx of pedunculated subserosal leiomyoma:

  • Ovary: use transvaginal US / MR to identify follicles!

• DDx of leiomyoma by MR:

  • Adenomyosis

  • Solid adnexal mass (Brenner tumor, fibroma)

  • Focal myometrial contraction (transient)

  • Uterine leiomyosarcoma

Uterine Lipoleimyoma

• = uncommon benign uterine neoplasm

• Cause: fatty metamorphosis of smooth muscle cells

• Histo: smooth muscle + fat + connective tissue

• Associated with: leiomyomas

• Location: corpus; predominantly intramurally, endophytic or exophytic

• US:

  • well-defined hyperechoic mass surrounded by a hypoechoic ring of myometrium

• CT:

  • predominantly fatty mass + areas of nonfat soft-tissue density tissue arising from uterus

• MR:

  • hyperintense fat on T1WI + T2WI with chemical shift artifact

Benign Metastasizing Leiomyoma

• = smooth muscle tumors in lung, lymph nodes, abdomen

• Incidence: <60 cases

• Classification:

  • Benign metastasizing leiomyoma

    Origin:

    uterus in mature women; progression with estrogen + regression with progesterone

    • benign uterine leiomyoma removed many years earlier

      P.1070

      
      

      Rx:

      hysterectomy, bilateral oophorectomy, long-term hormone therapy; good prognosis

  • Metastatic leiomyoma

    Origin:	extrauterine primary in men + children (? slow-growing sarcoma)
    Rx:	surgical resection with mixed success

  • Multiple fibroleiomyomatous hamartoma

    Origin:	lung; totally benign behavior
    Spread:	lymph nodes outside the pelvis, peritoneal surface, venous channels, lung, heart

• asymptomatic in most cases

• fever, mild nonproductive cough

Histo:

well-differentiated benign-appearing smooth muscle cells

• multiple pulmonary nodules

• miliary pattern

• pedunculated pulmonary leiomyoma with cyst formation

• giant cyst

Uterine Leiomyosarcoma

Incidence:	0.67:100,000; 25% of all uterine sarcomas
Cause:	(a) de novo growth independent of leiomyoma!
	(b) sarcomatous transformation of preexisting leiomyoma (in <1% of leiomyomas)
Histo:	infiltrative margins, nuclear atypia, increased mitotic figures

• rapidly enlarging uterus (<3%)

• mass with irregular margin

• rapid change in size + appearance

• extensive degeneration

• loss of well-defined outer capsule

  Dx:

  often first established by pathologist

Uterine Rupture in Pregnancy

• = disruption of all layers surrounding the fetus (membranes, decidua, myometrium, serosa)

• Prevalence: 3 5% for classic cesarean sections; 1 2% for lower segment operations

• Classification:

  • Spontaneous rupture during labor

  • Traumatic rupture during delivery

  • Rupture due to myometrial scars / disease

• Predisposed: previous uterine surgery, previously excessively long / difficult labor

• Location:

  • corpus with rupture before onset of labor

  • lower uterine segment during labor, L > R

• Cx: hypofibrinogenemia (triggered by excessive blood loss, trauma, amniotic fluid embolism)

• Mortality: 2 20% maternal mortality; 10 25% fetal mortality

• DDx: Uterine dehiscence = rupture of only myometrium

Uterine Trauma During Pregnancy

Incidence:	6 7%
Cause:	motor vehicle accident (70%), physical abuse (10%)

• Placental abruption: complete (6 66%) / incomplete (30 80%)

• Uterine rupture (0.6%)

• Fetal injury (eg, cerebral injury)

• Fetal death

• The major cause for fetal death is maternal death

US:

evaluate fetal motion, breathing, heart rate, placenta

Vaginal Agenesis

• 2nd most common cause of primary amenorrhea

Incidence:

1:4,000 5,000 women

• cyclic abdominal pain

• May be associated with:

  • Uterine + partial tubal agenesis (90%)

  • Unilateral renal agenesis / ectopia (34%)

  • Skeletal malformations (12%)

  • McKusick-Kaufman syndrome (hydrometrocolpos + polydactyly + heart defects)

  • Ellis-van Creveld syndrome

Vasa Previa

= rare type of velamentous cord insertion in which umbilical vessels cross the internal os

• vessels connecting separate succenturiate lobe to main portion of placenta

• cord vessels of velamentous (membranous) cord insertion from low-lying placenta

• aberrant chorionic vessels in association with marginal cord insertion from low lying placenta

Cx:	(1) Bleeding from torn fetal vessels
	(2) Cord compression by presenting part during labor
	(3) Cord prolapse
Risk:	50 100% fetal mortality

Velamentous Cord Insertion

• = umbilical cord insertion into membranes before entering placenta = attachment of cord to chorion laeve

• Incidence: 0.09 1.8%

• Associated with:

  • multiple gestation, uterine anomaly, IUD

  • congenital anomalies (in 5.9 8.5%):

    • asymmetric head shape, spina bifida, esophageal atresia, obstructive uropathy, VSD, cleft palate

Cx:	(1) IUGR
	(2) Preterm labor
Risk:	(1) Cord compression
	(2) Rupture of cord with traction during delivery