33 - Video-Assisted Thoracic Surgery for Wedge Resection, Lobectomy, and Pneumonectomy
Editors: Shields, Thomas W.; LoCicero, Joseph; Ponn, Ronald B.; Rusch, Valerie W.
Title: General Thoracic Surgery, 6th Edition
Copyright 2005 Lippincott Williams & Wilkins
> Table of Contents > Volume I - The Lung, Pleura, Diaphragm, and Chest Wall > Section IX - The Chest Wall > Chapter 41 - Chest Wall Deformities
function show_scrollbar() {}
Chapter 41
Chest Wall Deformities
Robert C. Shamberger
A broad spectrum of congenital chest wall deformities occurs. The severe life-threatening deformities, ectopia cordis and asphyxiating thoracic dystrophy, are rare in comparison with the more frequent and milder pectus excavatum and carinatum. Congenital anterior thoracic deformities can be conveniently considered in five categories: (a) pectus excavatum; (b) pectus carinatum; (c) Poland's syndrome; (d) sternal defects, including ectopia cordis; and (e) miscellaneous conditions, including vertebral and rib anomalies, asphyxiating thoracic dystrophy (Jeune's disease), and rib dysplasia.
PECTUS EXCAVATUM
Posterior depression of the sternum and costal cartilages produces the characteristic findings of pectus excavatum: funnel chest, or trichterbrust. The first and second ribs and the manubrium are usually in their normal position (Fig. 41-1), but the lower costal cartilages and the body of the sternum are depressed. In older adolescents and adults, the most anterior portion of the osseous ribs may also be curved posteriorly. The extent of sternal and cartilaginous deformity is quite variable. Numerous methods of grading and defining these deformities have been proposed by H mmer and Willital (1984), Oelsnitz (1981), Welch (1980), and Haller and associates (1987), as well as others, but none has been universally accepted. Asymmetry of the depression is present frequently. Often the right side is more depressed than the left, and the sternum may be rotated as well. Many children with pectus excavatum have a characteristic physique with a broad thin chest, dorsal lordosis, hook shoulder deformity, costal flaring, and poor posture.
Pectus excavatum is present at birth or within the first year of life in the majority of affected children (86%), as shown in Fig. 41-2. The deformity rarely resolves with increasing age, and it may worsen during the period of rapid adolescent growth. Waters and associates (1989) identified scoliosis in 26% of 508 patients with pectus excavatum. Hence, all patients with pectus deformities should be evaluated clinically for scoliosis. Asymmetric pectus excavatum with a deep right gutter and sternal rotation often is accompanied by scoliosis. Correction of the associated pectus excavatum may stabilize the curve in conjunction with exercises or bracing, thereby avoiding spinal fusion.
Congenital heart disease has been identified by Shamberger and Welch (1988b) in 1.5% of infants and children undergoing chest wall correction at the Children's Hospital in Boston (Table 41-1). The frequency of chest wall deformities among all patients with congenital heart disease evaluated at this institution was only 0.17%.
Asthma may be identified in patients with pectus excavatum and carinatum. In a review of 694 consecutive cases, Shamberger and Welch (1988b) found a subgroup of 35 patients with asthma (5.2%), which is comparable with the occurrence of asthma in the general pediatric population.
Etiology and Incidence
Ravitch (1977) reported that pectus excavatum may occur as frequently as 1 in 300 to 400 live births and that it is rare in blacks. It occurs more frequently in boys than girls, by almost a 4:1 ratio. Although the sternal depression appears to be caused by overgrowth of costal cartilages, the etiology of pectus deformities is unknown. Early investigators, such as Lester (1957), attributed its development to an abnormality of the diaphragm. Little evidence has supported this theory other than the occurrence reported by Greig and Azmy (1990) of pectus excavatum in children after repair of agenesis of the diaphragm. Vanamo and associates (1996) also demonstrated a frequent association between congenital diaphragmatic hernia and pectus excavatum. Hecker and associates (1981) described histopathologic changes in the costal cartilages similar to those seen in scoliosis, aseptic osteonecrosis, and inflammatory processes, but the etiology of these findings and their significance are unknown. An increased familial incidence exists. In a review by this author and colleagues (1988), 37% of 704 patients had a family history of chest wall deformity.
P.654
Three of four siblings were involved in one family. Scherer and colleagues (1988) reported a high incidence of chest wall deformities in children with Marfan's syndrome that are often severe and usually accompanied by scoliosis. Patients with abdominal musculature deficiency syndrome (prune-belly syndrome) commonly have pectus excavatum [8 of 43 patients in the experience of Welch and Kearney (1974)]. Pectus excavatum also occurs with other myopathies and chromosomal defects, such as Turner's syndrome. A summary of the associated musculoskeletal abnormalities is shown in Table 41-2.
 |
Fig. 41-1. An 8-year-old boy with a symmetric pectus excavatum deformity. Note that the depression extends to the sternal notch. |
 |
Fig. 41-2. Age at appearance of pectus excavatum deformity in 704 infants and children. Note the large proportion identified at birth or within the first year of life and the predominance of males with deformity. From Shamberger RC, Welch, KJ: Surgical repair of pectus excavatum. J Pediatr Surg 23:615, 1988b. With permission. |
Table 41-1. Cases of Congenital Heart Disease Associated with Pectus Excavatum and Carinatum | ||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||||||||||||||||
Symptoms
Pectus excavatum is well tolerated in infancy and childhood. The anterior depression in an infant with a flexible chest may be magnified by upper airway obstruction from tonsillar and adenoidal hypertrophy, but they do not primarily produce the pectus deformity. Older children may complain of pain in the area of the deformed cartilages or of precordial pain after sustained exercise. A few patients have palpitations, presumably due to transient atrial arrhythmias. These patients may have mitral valve prolapse and associated atrial arrhythmias.
Pathophysiology
Some researchers, including Haller and associates (1970), contend that no cardiovascular or pulmonary impairment
P.655
results from pectus excavatum. This contrasts, however, with the clinical impression that many patients have increased stamina after surgical repair. These findings date back to the surgical repair performed by Sauerbruch in 1913 (1920). The patient was an 18-year-old boy who developed dyspnea and palpitations with very limited exercise. Three years after his operation, he could work 12 to 14 hours a day without tiring and without palpitations. Anecdotal reports during the next three decades confirmed this observation. Since then, investigators have sought to identify the physiologic abnormality or combination of abnormalities that could explain this symptomatic improvement after surgery. Early physiologic measurements of cardiac and pulmonary function were crude and did not yield convincing evidence of a cardiopulmonary deficit. In many early studies, summarized by Shamberger and Welch (1988a), the results fell within the broad range of normal values, if often at the lower limit.
Table 41-2. Musculoskeletal Abnormalities Identified in 130 of 704 Cases of Pectus Excavatum | ||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||||
A systolic ejection murmur is frequently identified in patients with pectus excavatum and is magnified by a short interval of exercise. It is attributed to the close proximity between the sternum and the pulmonary artery, which results in transmission of a flow murmur.
Electrocardiographic abnormalities are common, and Schaub and Wegmann (1954) attributed them to the abnormal configuration of the chest wall and the displacement and rotation of the heart into the left thoracic cavity. Preoperative electrocardiographic findings reported by Welch (1980) in 32 patients with pectus excavatum are shown in Table 41-3. Most significant are the cases of conduction blocks or arrhythmias. Patients with a history of palpitations should have a 24-hour electrocardiogram as well as an echocardiogram to evaluate for mitral valve prolapse. Resolution of these supraventricular arrhythmias has been anecdotally reported after correction of a pectus excavatum deformity.
Deformity of the chest wall led many authors to attribute the symptomatic improvement in patients after surgery to initial impairment in pulmonary function. This was difficult to prove, however, with the wide range of pulmonary function that exists from individual to individual and its dependence on physical training and body habitus.
Table 41-3. Electrocardiographic Findings in a Group of 32 Patients with Pectus Excavatum | ||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||
Pulmonary Function Studies
As early as 1951, Brown and Cook performed respiratory studies on patients before and after surgical repair. They demonstrated that although vital capacity (VC) was normal, the maximum breathing capacity diminished (50% or more) in 9 of 11 cases and increased an average of 31% after surgical repair. Weg and associates (1967) evaluated 25 Air Force recruits with pectus excavatum and compared them with 50 unselected basic trainees. Although the lung compartments of both groups were equal, as were the VCs, the maximum voluntary ventilation was significantly lower in those with pectus excavatum than in the control population. Castile and co-workers (1982) evaluated seven patients with pectus excavatum, five of whom were symptomatic with exercise. The mean total lung capacity of the group was 79% of predicted. Flow volume configurations were normal, excluding airway obstruction as a cause of the symptoms. Workload tests demonstrated normal response to exercise in the dead space to tidal volume ratio and alveolar-arterial oxygen difference. The measured oxygen uptake, however, increasingly exceeded predicted values as workload approached maximum in the four symptomatic subjects with pectus excavatum. This pattern of oxygen consumption was different from that in normal subjects and in the three asymptomatic subjects with pectus excavatum, in whom a linear response was seen. The mean oxygen uptake in the symptomatic subjects at maximal effort exceeded the predicted values by 25.4%. The three asymptomatic subjects, on the other hand, demonstrated normal linear oxygen uptake during exercise. Increased oxygen uptake suggests increased work of breathing in these symptomatic individuals despite the normal or mildly reduced VCs. Increases in tidal volume with exercise were uniformly depressed in those with pectus excavatum.
Cahill and co-workers (1984) performed pre- and postoperative studies in 5 children and adolescents with pectus carinatum and in 14 with pectus excavatum. No abnormalities were demonstrated in the pectus carinatum group. The low normal VCs in excavatum patients were unchanged by operation, but a small improvement in the total lung capacity and a significant improvement in the maximal voluntary ventilation were seen. Exercise tolerance improved in those with pectus excavatum after operation, as determined both by total exercise time and maximal oxygen consumption. In addition, at any given workload, those with pectus excavatum demonstrated a lower heart rate, stable oxygen consumption, and higher minute ventilation after repair. Mead and associates (1985) studied rib cage mobility by assessing intraabdominal pressure. Normal abdominal pressure tracings in pectus excavatum suggested normal rib cage mobility.
Blickman and colleagues (1985) assessed pulmonary function in 17 children with pectus excavatum by xenon perfusion and ventilation scintigraphy before and after surgery. Ventilation studies were abnormal in 12 children before surgery and improved in 7 after repair. Perfusion scans
P.656
were abnormal in 10 children before surgery and improved after operation in 6 children. The ventilation-perfusion ratios were abnormal in 10 of the 17 children preoperatively and normalized after repair in 6 children.
Derveaux and colleagues (1989) evaluated 88 patients with pectus excavatum and carinatum by pulmonary function tests before and 1 to 20 years after repair (mean 8 years). The surgical technique used a fairly extensive chest wall dissection. Preoperative studies were within the normal range (>80% of predicted) except in subjects with both scoliosis and pectus excavatum. The postoperative values for forced expiratory volume in 1 second and VC were decreased in all groups when expressed as a percentage of predicted, although the absolute values at follow-up may have been greater than at preoperative evaluation. Radiologic evaluation of these individuals confirmed improved chest wall configuration, so the relative deterioration in pulmonary function was not the result of recurrence of the pectus deformity. An inverse relationship was found between preoperative and postoperative function. Those with less than 75% of predicted function had improved function after surgery, but results were worse after repair if the preoperative values were greater than 75% of predicted. Almost identical results were found in a study by Morshuis and associates (1994a), who evaluated 152 subjects before and a mean of 8 years after surgery for pectus excavatum. These physiologic results were in contrast to the subjective improvement in symptoms from the subjects and the improved chest wall configuration. The decline in pulmonary function in the postoperative studies was attributed to the operation because the preoperative defect appeared to be stable regardless of the age at initial repair. Both studies were marred by the obvious lack of an age- and severity-matched control group without surgery.
Derveaux and colleagues (1988) evaluated transpulmonary and transdiaphragmatic pressures at total lung capacity in 17 individuals with pectus excavatum. Preoperative and long-term follow-up evaluations were performed a mean of 12 years apart. Reduced transpulmonary and transdiaphragmatic pressures suggested that the increased restrictive defect was produced by extrapulmonary rather than pulmonary factors, or that surgery produced increased rigidity of the chest wall.
Wynn and colleagues (1990) assessed 12 children with pectus excavatum by pulmonary function tests and exercise testing. Eight children had repair and were evaluated pre- and postoperatively. Four children had two sets of evaluations, but no operation. A decline in total lung capacity was identified in the repaired children compared with stable values in the control group. Cardiac output and stroke volume increased appropriately with exercise before and after operation in both groups, and the operation was believed to have produced no physiologically significant effect on the response to exercise.
Kaguraoka and associates (1992) evaluated pulmonary function in 138 individuals preceding and after repair of pectus excavatum. A decrease in VC occurred during the first 2 months after surgery, with recovery to preoperative levels by 1 year after operation. At 42 months, the values were maintained at baseline, despite a significant improvement in the chest wall configuration. Tanaka and co-workers (1993) found similar results in individuals who had the more extensive sternal turnover technique; in fact, they demonstrated a more significant and long-term decrease in VC. Morshuis and co-workers (1994b) evaluated 35 subjects who had had pectus excavatum repaired as teenagers or young adults; ages were 17.9 5.6 years. Preoperative evaluations were performed and repeated 1 year after surgery. Preoperative total lung capacity (86.0 14.4% of predicted) and VC (79.7 16.2%) were significantly decreased from predicted valves and decreased further after surgery (-9.2 9.2% and -6.6 10.7%, respectively). The efficiency of breathing at maximal exercise improved significantly after surgery. Ventilatory limitation of exercise occurred in 43% of the subjects before repair, and there was a tendency toward improvement after surgery. However, the group with no ventilatory limitation initially demonstrated a limitation after surgery with a significant increase in oxygen consumption.
Quigley and colleagues (1996) evaluated 36 adolescents with pectus excavatum and 10 age-matched healthy controls at baseline and then an average of 8 months after surgery in 15 subjects and 9 months in controls. Adolescents with pectus excavatum had a decrease in VC compared with controls, although the mean values remained in the normal range. The mean total lung capacity was normal. There was no difference in workload between subjects with pectus excavatum and the controls, with both groups achieving a similar duration and level of exercise. No significant change in follow-up pulmonary function tests was seen in either group. The duration of exercise as well as the level of work increased significantly in those who had surgery but not in the controls. The absence of adverse effects on pulmonary function after surgery was attributed to a less extensive surgical procedure than was used in the studies reported by Derveaux (1988) and Morshuis (1994a, 1994b) and their colleagues.
In composite, these studies of pulmonary function over the past four decades have failed to document consistent improvement in pulmonary function resulting from surgical repair. In fact, studies have demonstrated deterioration in pulmonary function at long-term evaluation that was attributable to increased chest wall rigidity after surgical repair. Despite this finding, workload studies have shown improvement in exercise tolerance after repair.
Cardiovascular Studies
Posterior displacement of the sternum can produce a deformity of the heart, particularly anterior indentation of the right ventricle. Early pathologic studies demonstrated this finding (Fig. 41-3). Garusi and D'Ettorre (1964) showed by
P.657
angiography displacement of the heart to the left, often with a sternal imprint on the anterior wall of the right ventricle. Howard (1959) demonstrated by angiography its resolution by surgical repair. Elevated right heart pressures have been reported by some researchers, as have pressure curves similar to those seen in constrictive pericarditis. In 1962, Beveg rd studied 16 individuals with pectus excavatum by right heart catheterization and exercise testing. The physical work capacity in pectus excavatum at a given heart rate was significantly lower in the sitting than the supine position. Those with 20% or greater decline in physical work capacity from the supine to the sitting position had shorter sternovertebral distances than did those with less decrease in their physical work capacity. The measured stroke volume at rest decreased from supine to sitting positions a mean of 40.3%, similar to normal subjects. In the supine position, stroke volume increased with exercise to 13.2%. In the sitting position, the increase in stroke volume from rest to exercise was 18.5% for the pectus excavatum group, significantly lower (p < 0.001) than the 51% increase seen in normal subjects. Thus, in the pectus excavatum group, an increased cardiac output could be achieved primarily by increased heart rate because limited enhancement of the stroke volume could occur. Intracardiac pressures measured at rest and with exercise were normal in all subjects despite this apparent limitation of ventricular volume. Gattiker and B hlmann (1967) confirmed this limitation of the stroke volume in a study of 19 subjects. In the upright position at a heart rate of 170 beats per minute, the physical work capacity was lower than in the supine position (mean 18% decrease) because of the decrease in stroke volume. Beiser and associates (1972) performed cardiac catheterization in six adolescents and young adults with moderate degrees of pectus excavatum. Normal pressure and cardiac index were obtained at rest in the supine position. The cardiac index during moderate exercise was normal, but the response to upright exercise was below that predicted in two patients and at the lower limit of normal in three patients. The cardiac index was 6.8 0.8 L/min/m2 compared with 8.9 0.3 L/min/m2 in a group of 16 normal controls (p < 0.01). The difference in cardiac performance again appeared to be produced primarily by a smaller stroke volume in the group with pectus excavatum in an upright position. Stroke volume was 31% lower and cardiac output 28% lower during upright as compared with supine exercise. Postoperative studies were performed in three individuals: two of them achieved a higher level of exercise tolerance after surgery. The cardiac index increased an average of 38%. Because heart rate at maximal exercise was not higher after repair, an enhanced stroke-volume response was responsible for this increase.
 |
Fig. 41-3. Anatomic drawing from an autopsy of a male with pectus excavatum reported in 1912 demonstrates compression of the heart, particularly the right ventricle by the sternum. From Bien G: Zur Anatomie und tiolgie der Trichterbrust. Beitr Pathol Anat Allg Pathol 52:567, 1912. With permission. |
Peterson and associates (1985) performed radionuclide angiography and exercise studies in 13 children with pectus excavatum. Ten of 13 were able to reach the target heart rate before surgical repair, 4 without symptoms. After operation, all but 1 child reached the target heart rate during the exercise protocol, and 9 of 13 reached the target without becoming symptomatic. The left and right ventricular end-diastolic volumes were consistently increased after repair at rest, and the mean stroke volume was increased 19% after repair. These findings substantiated the ventricular volume changes previously demonstrated by cardiac catheterization, although an increase in the cardiac index was not demonstrated. Recent echocardiographic studies by Kowalewski and associates (1999) of 42 patients before and 6 months after surgery revealed statistically significant changes in the right ventricular volume indices after surgery. However, no correlation was seen between the pectus index and the changes in the right ventricular volume indices.
Additional studies are needed to further define the relationship between pectus excavatum and cardiopulmonary function. Recent dynamic or exercise studies have been most promising in this area. Methods to more effectively evaluate preoperative cardiopulmonary function are needed to identify which children may achieve symptomatic and physiologic improvement from surgical repair.
Echocardiographic Studies
Bon Tempo (1975), Salomon (1975), and Schutte (1981) and their associates reported mitral valve prolapse in patients with narrow anterior-posterior chest diameters, anterior chest wall deformities, and scoliosis. Echocardiographic prospective studies of adults with pectus excavatum demonstrated mitral valve prolapse in 6 of 33 (18%) subjects studied by Udoshi and associates (1979) and in 11 of 17 subjects (65%) of Saint-Mezard and colleagues (1986). Anterior compression of the heart by the depressed sternum may deform the mitral annulus or the ventricular chamber and produce mitral valve prolapse in these patients. Preoperative
P.658
evaluation by echocardiogram of children with pectus excavatum by the author and associates (1987) identified 23 children with mitral valve prolapse. Postoperative studies did not demonstrate mitral valve prolapse in 10 (43%) of these children, suggesting resolution after correction of the chest wall deformity.
Surgical Repair
The first surgical corrections of pectus excavatum were reported by Meyer in 1911 and Sauerbruch in 1920. In 1939, Ochsner and DeBakey summarized their early experiences with various techniques. In 1949, Ravitch reported a technique that included excision of all deformed costal cartilages with the perichondrium, division of the xiphoid from the sternum, division of the intercostal bundles from the sternum, and a transverse sternal osteotomy securing the sternum anteriorly in an overcorrected position. He used Kirschner wire fixation in the first two patients and silk suture fixation in later patients.
Baronofsky (1957) and Welch (1958) reported a technique for the correction of pectus excavatum that emphasized total preservation of the perichondrial sheaths as well as the attachment of the upper sheaths and intercostal bundles to the sternum. Anterior fixation of the sternum was achieved with silk sutures. The technique I use today remains unchanged from these methods, except for the use of retrosternal strut fixation in all children. Haller and associates (1970) later developed a technique called tripod fixation, in which subperichondrial resection of the abnormal cartilages is performed followed by a posterior sternal osteotomy. The most cephalad normal cartilages are then divided obliquely in a posterolateral direction. When the sternum is elevated, the sternal ends of the cartilage rest on the costal ends, providing further anterior support of the sternum.
Several researchers have promoted supporting the sternum by metallic struts after mobilization of the costal cartilages. Rehbein and Wernicke (1957) developed struts that could be placed into the marrow cavity of the ribs at the costochondral junction. An arch was then formed by the struts anterior to the sternum, and the sternum was secured to this arch. Paltia and associates (1958) placed a transverse strut through the caudal end of the sternum, firmly fixing its location. The two ends of the strut are supported by the ribs laterally. Adkins and Blades (1961) and Jensen and associates (1970) used retrosternal elevation by a metallic strut. Willital (1981) used a similar retrosternal strut after creating multiple chondrotomies in the costal cartilages to provide flexibility. Recent innovations in these methods include bioabsorbable struts, Marlex mesh, or a Dacron vascular graft as a strut, but there is no evidence that these methods are preferable to traditional methods. No randomized studies have compared the recurrence or complication rates between suture or strut fixation techniques. Oelsnitz (1981) and Hecker and co-workers (1981), using suture fixation, reported satisfactory repairs in their large series in 90% to 95% of patients.
The sternal turnover was first proposed by Judet and Judet (1954) and Jung (1956) in the French literature. In this method, the sternum is mobilized and the costal cartilages are divided, allowing the sternum to be rotated 180 degrees. Wada and colleagues (1970) have reported a large series from Japan using this technique, which is essentially a free graft of sternum. It is a radical approach and has been associated with major complications if infection occurs. Modifications of this technique by Taguchi and associates (1975) have involved either preservation of the internal mammary vessels by wide dissection or reimplantation of the internal mammary artery. These methods were developed because of the reported incidence of osteonecrosis and fistula formation, which occurred in up to 46% of patients over 15 years of age.
A method described by Allen and Douglas (1979) is that of implantation of Silastic molds into the subcutaneous space to fill the deformity. Although this approach may improve the external contour of the chest, extrusion of the molds has occurred, and this method does nothing to increase the volume of the thoracic cavity or relieve compression on the heart.
A method of elevation of the sternum with a retrosternal bar without resection or division of the costal cartilages was reported by Nuss and associates (1998). He repaired 42 patients under 15 years of age (median age 5 years) by placing a convex steel bar under the sternum and anterior to the heart through small bilateral thoracic incisions. As initially described, a long clamp was passed blindly behind the sternum and out the contralateral opening (Figs. 41-4A,B). A tape was then drawn across and used to pull the bar through the chest. The bar is initially placed with the concave side anteriorly and then it is rotated once in position (Figs. 41-4,E,F). The bar is left in position for 2 years before removal, when presumed permanent remodeling of the cartilages has occurred. Although Nuss, in his initial report, warned that the upper limits of age for this procedure require further evaluation, the technique has been widely used in older patients and long-term results from this population have not yet been reported. In 2002, the results by Nuss and his associates using this technique in 303 patients was reported (Croitoru). This included an older group of children than in the initial report (range 21 months to 29 years; median age 12.4 years). Two bars were required in 12.5% of the patients. Routine use of thoracoscopy to avoid cardiac injury was instituted in 1998. Lateral stabilizers were placed in 69.4% of the cases and were routinely used after 1998 and were wired to the bar in 65.4% of cases (Fig.41-4D). Epidural analgesic was used for 2 to 4 days, and the median length of stay was 5 days, with a range of 3 to 10 days. The frequency of early complications was low. It included pneumothorax requiring aspiration 1.0%, pericarditis 2.3% (with only 0.3% requiring drainage), pneumonia
P.659
0.7%, hemothorax 0.3%, transient extremity paralysis 0.3%, superficial wound infection 2.3%, and bar infection requiring eventual removal of the bar 0.7%.
 |
Fig. 41-4. A long Lorenz tunneler is passed across the chest behind the sternum and anterior to the heart. (Walter Lorenz Surgical, Inc. Jacksonville, FL, U.S.A.). B. Umbilical tape is then drawn back across the chest with the device and to it is secured the convex steel Lorenz bar, which is guided into the substernal tunnel using the umbilical tape to keep it on track. C. The Lorenz bar is shown with its convex aspect directed posteriorly. It is then rotated 180 degrees with a special device, the Lorenz flipper, compressing the sternum anteriorly. D. A stainless-steel cross-piece is then secured to one end of the bar (or both ends in some patients needing extra support) with heavy #3 wire. Once it is wired together, the whole apparatus is then sutured to the soft tissues of the chest with multiple absorbable sutures to achieve secure fixation to the chest wall to prevent rotation of the bar and loss of correction of the deformity or side-to-side movement of the bar. The entrance of the bar under the rib is at the inner aspect of the pectus ridge. E. Schematic depiction of the Lorenz bar after placement and then (F) after rotation 180 degrees, producing anterior displacement of the sternum and costal cartilages. Reproduced with permission from Donald Nuss, M.D. Children's Hospital of The King's Daughters, Norfolk, VA. |
Late complications in this series included bar displacement requiring repositioning in 8.6% which included a high proportion (over 50%) of patients in whom a stabilizer was not used, or in patients where the stabilizer was not wired to the bar. When both modifications were used, displacement occurred in only 5% of the patients. An unexpected occurrence of allergies to the metal bar was encountered in 1% of the patients presenting with rashes in the area of the bars, requiring revision to bars composed of other alloys. Late hemothorax occurred in two patients, one secondary to trauma, in which the source was not defined. The occurrence of a mild overcorrection in the deformity was seen in 3.6% and a pectus carinatum deformity developed in 1.3%, all of whom had either Marfan's syndrome or Ehlers-Danlos syndrome. The outcome of children in this large series was good, with excellent appearance in 84.5%, good in 14.8%, and failed in only one patient, but the bars had been removed at the time of the report in only 23.4% of the patients.
Hebra and associates (2000) reported the results of a survey of members of the American Pediatric Surgery Association who had used the minimally invasive (Nuss) technique. Thirty institutions contributed their cases, which totalled 251, although it should be noted that 42% were performed by one surgeon. The complications reported were similar to those of Nuss and his associates, but the frequency was higher, presumably because the procedures were performed by more individuals less familiar with the operation. Displacement of the bar occurred in 9.2% of cases and pneumothorax requiring tube thoracostomy in 4.8%. Less frequently encountered complications included thoracic outlet syndrome, pericarditis, blood loss requiring a transfusion, cardiac injury, persistent cardiac arrhythmias, and erosion of the sternum by the bar. Many of the surgeons had adopted the use of thoracoscopy to improve the safety of passing the clamp anterior to the heart. Other surgeons elevate the sternum with a bone hook during passage of the clamp to open the retrosternal space anterior to the heart.
Engum and co-workers (2000) reported their series of 21 patients with a mean age of 8.2 years. Their patients had an average hospital stay of 4.9 days, which was comparable with that for the open repair. Complications encountered in their series were similar to the experience of others and included rotation of the bar, production of a marked pectus carinatum deformity, progressive chest wall asymmetry, and chronic persistent pain requiring removal of the bar in one case.
P.660
Molik and colleagues (2001) later enlarged this single institution review and in a retrospective review compared 68 patients who underwent a standard surgical repair with 35 patients who underwent a Nuss repair. The Nuss procedure required less time (3.3 hours) compared with the open technique (4.7 hours), but had a higher complication rate (43%) than the open method (20%). Four patients with the standard operation (6%) and eight with the Nuss technique (29%) required reoperation. Length of stay was comparable between the open (4.8 days) and Nuss (4.0 days) techniques. The Nuss patients had a higher frequency of epidural analgesics postoperatively and an increased duration of patient-controlled analgesia after surgery.
Fonkalsrud and associates (2002) reported a similar retrospective comparison of the two techniques each utilized at a single institution. Sixty-eight patients had the minimally invasive procedure and 139 had the open technique during a 5-year interval. There was a higher incidence of reoperations and hospitalizations in the Nuss group, but it was noted that 90% of the complications of this technique occurred in the first 25 cases, again clearly demonstrating the role of experience in determining the frequency of surgical complications. It was difficult to differentiate whether the differences noted in the use of epidural catheters and intravenous narcotics was attributable to distinct patient requirements or institutional bias. There was a shorter mean hospitalization noted for the open procedure (2.9 days) compared with the minimal access procedure (6.5 days), and a similar difference between mean time before return to work or school (12 vs. 18 days). These investigators concluded that long-term follow-up also will be required to assure both health professionals and the public that this is the procedure of choice for patients with pectus excavatum.
The occurrence of overcorrection of the deformity or production of a true carinate deformity was first reported by Croitoru and associates (2002) and was associated with underlying connective tissue disorders (Marfan's syndrome and Ehlers-Danlos syndromes). It was reported, however, by Hebra (2002) to occur in a healthy 13-year-old boy 1 year after Nuss repair. What factors predispose some patients to this complication is not understood.
A prospective multi-institutional study of patients undergoing repair of pectus excavatum is in progress. It is hoped that this study will better define the role as well as the risks and benefits of the open and minimally invasive surgical procedures in the repair of pectus excavatum.
Children should be followed long-term after repair by any technique until they reach full stature. Only by so doing can each surgeon assess the ultimate results of his or her surgical technique. Regrettably, recurrence can occur until full stature is achieved.
Surgical Technique
The open surgical technique for correction of pectus excavatum is depicted in Fig. 41-5. In girls, particular attention is focused on placing the incision within the projected inframammary crease, thus preventing the complications of breast deformity and development described by Hougaard and Arendrup (1983). Skin flaps are mobilized by electrocautery to the angle of Louis superiorly and to the xiphoid inferiorly. Pectoral muscle flaps are elevated off the sternum and costal cartilages, thus preserving the entire pectoralis major and portions of the pectoralis minor and serratus anterior muscles in the flap (Fig. 41-5A). Ellis and associates (1997) have described elevating the skin and muscle together in a single flap, which is a reasonable but not widely adopted alternative method.
Perioperative antibiotics are used, giving one dose of cefazolin immediately before surgery and three postoperative doses. The Hemovac drain (Snyder Laboratories, Inc., New Philadelphia, OH, U.S.A.) is removed when the drainage is less than 15 mL for an 8-hour period. All patients are warned to avoid aspirin containing compounds for 2 weeks before surgery.
I currently use the retrosternal bar (Baxter Healthcare Co., Deerfield, IL, U.S.A.) for internal fixation to secure the sternum firmly in an anterior position and to avoid the need to skeletonize the sternum to achieve adequate mobility for suture fixation. Although correction of pectus excavatum is technically most easily performed in a young child, I have become increasingly concerned about long-term recurrence in these children as well as impairment in the growth of the chest wall. I currently delay surgery until the children are well into their pubertal growth. At this age, the chest has less remaining growth and opportunity for recurrence of the pectus excavatum (Fig. 41-6). In contrast, Humphreys and Jaretzki (1980) and Backer and associates (1961) have found no correlation between the age at repair and frequency of recurrence.
The closed technique developed by Donald Nuss is depicted in Fig. 41-4. Various modifications have been developed to minimize the risk for cardiac injury during passage of the clamp behind the sternum. Some surgeons place a small incision along the lower margin of the sternum through which they place a bone hook, which is used to elevate the sternum and increase the retrosternal space. Others create a small additional opening into the chest through which a small thoracoscope is placed to observe passage of the clamp behind the sternum and anterior to the pericardium.
Complications
Complications of pectus excavatum repair that the author and Welch (1988b) reported in 704 patients (Table 41-4) are few and relatively unimportant, except for major recurrence in 17 patients. In 2% of patients, a limited pneumothorax required aspiration or was simply observed. Tube thoracostomy has not been required in the past two decades and was used in only four patients in the entire series. Wound infection is rare with the use of perioperative antibiotic coverage.
The most distressing complication after surgical correction of pectus excavatum is major recurrence of the deformity.
P.661
It is difficult to predict which patients will have a major recurrence, but it appears to occur with increased frequency in children with poor muscular development and an asthenic or marfanoid habitus. All children with Marfan's syndrome should be repaired with strut fixation because of the high risk for recurrence reported without struts. Scherer and associates (1988) reported a low recurrence rate (one of eight cases) using a retrosternal strut.
Fig. 41-5. Open surgical technique for repair of pectus excavatum. A. A transverse incision is placed below and well within the nipple lines and, in females, at the site of the future inframammary crease. The pectoralis major muscle is elevated from the sternum along with portions of the pectoralis minor and serratus anterior bundles. B. The correct plane of dissection of the pectoral muscle flap is defined by passing an empty knife handle directly anterior to a costal cartilage after the medial aspect of the muscle is elevated with electrocautery. The knife handle is then replaced with a right-angle retractor, which is pulled anteriorly. The process is repeated anterior to an adjoining costal cartilage. Anterior distraction of the muscles during the dissection facilitates identification of the avascular areolar plane and avoids entry into the intercostal muscle bundles. Muscle elevation is extended bilaterally to the costochondral junctions of the third to fifth ribs and a comparable distance for ribs 6 and 7. C. Subperichondrial resection of the costal cartilage is achieved by incising the perichondrium anteriorly. It is then dissected away from the costal cartilages in the bloodless plane between the perichondrium and costal cartilage. Cutting back the perichondrium 90 degrees in each direction at its junction with the sternum (inset) facilitates visualization of the back wall of the costal cartilage. D. The cartilages are divided at their junction with the sternum using a knife with a Welch perichondrial elevator held posteriorly to elevate the cartilage and protect the mediastinum (inset). The divided cartilage can then be held with an Allis clamp and elevated. The costochondral junction is preserved with a segment of costal cartilage on the osseous ribs by incising the cartilage with a scalpel. Costal cartilages 3 through 7 are generally resected, but occasionally the second costal cartilages must be removed if posterior displacement or funneling of the sternum extends to this level, as may be seen in older patient (see Fig.41-1). Segments of the sixth and seventh costal cartilages are resected to the point where they flatten to join the costal arch. Familiarity with the cross-sectional shape of the medial ends of the costal cartilages facilitates their removal. The second and third cartilages are broad and flat, the fourth and fifth are circular, and the sixth and seventh are narrow and deep. E. The sternal osteotomy is created above the level of the last deformed cartilage and the posterior angulation of the sternum, generally the third cartilage but occasionally the second. Two transverse sternal osteotomies are created through the anterior cortex with a Hall air drill (Zimmer USA, Inc., Warsaw, IN, U.S.A.) 3 to 5 mm apart. F. The base of the sternum and the rectus muscle flap are elevated with two towel clips, and the posterior plate of the sternum is fractured. The xiphoid can be divided from the sternum with electrocautery, allowing entry into the retrosternal space. This step is not necessary with the use of a retrosternal strut unless the xiphoid is protruding anteriorly when the sternum is in its corrected position. Preservation of the attachment of the perichondrial sheaths and xiphoid avoids an unsightly depression that can occur below the sternum. G. When a strut is not used, the osteotomy is closed with several heavy silk sutures as the sternum is elevated by the assistant. H. Correction of the abnormal position of the sternum is achieved by creation of a wedge-shaped osteotomy, which is then closed, bringing the sternum anteriorly into an overcorrected position. I. This figure demonstrates the use of both retrosternal struts and Rehbein struts. Rehbein struts are inserted into the marrow cavity (insert) of the third or fourth rib, and the struts are then joined medially to create an arch anterior to the sternum. The sternum is sewn to the arch to secure it in its new anterior position. The retrosternal strut is placed behind the sternum and is secured to the rib ends laterally to prevent migration. J. Anterior depiction of the retrosternal strut. The perichondrial sheath to either the third or fourth rib is divided from its junction with the sternum, and the retrosternal space is bluntly dissected to allow passage of the strut behind the sternum. It is secured with two pericostal sutures laterally to prevent migration. The wound is then flooded with warm saline and cefazolin solution to remove clots and inspect for a pleural entry. A single-limb medial Hemovac drain (Snyder Laboratories, Inc., New Philadelphia, OH, U.S.A.) is brought through the inferior skin flap to the left of the sternum and placed in a parasternal position to the level of the highest resected costal cartilage. K. The pectoral muscle flaps are secured to the midline of the sternum, advancing the flaps inferiorly to obtain coverage of the entire sternum. The rectus muscle is then joined to the pectoral muscle flaps, closing the mediastinum. A H and K from Shamberger RC, Welch KJ: Surgical repair of pectus excavatum J. Pediatr Surg 23:615, 1988b. With permission. D and F adapted from original figures. |
Although recurrences appear symmetric, they are in fact frequently right-sided, with a deep right parasternal gutter and sternal obliquity. The third, fourth, and fifth rib ends migrate medially, with apparent foreshortening of the costal cartilages. Correction of recurrent pectus excavatum is generally a formidable task. Sanger and associates (1968) reported their experience in secondary correction. They resected the regenerated fibrocartilage plate, repeated the
P.662
P.663
P.664
osteotomy, and closed the pectoral muscles behind the sternum. Ten patients had an early good result. In the Boston Children's Hospital experience, 12 children and adolescents underwent secondary repair. Resection of the segments of the third to fifth costal cartilages was necessary to correct the deformity. After clearing the tip of the sternum, resection of the left fibrocartilage plate to the level of the third or second perichondrial sheath allowed the sternum to be brought forward and rotated to an acceptable position. Ten of 12 repeat operations were accomplished without pleural entry. Follow-up of patients with secondary correction ranged from 10 to 17 years. Eight have acceptable thoracic contour; two have a broad shallow depression; and two have frank recurrence. I recommend use of strut fixation on all patients with secondary repair because cartilage regeneration will be slower and less adequate than that after primary operation.
 |
Fig. 41-6. A. Preoperative photographs of a 14-year-old boy with pectus excavatum. B. Postoperative photograph 7 months after repair using a retrosternal strut. |
Table 41-4. Complications of Pectus Excavatum Repair: 70 Cases in 704 Patients | ||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||||
In 1990, Martinez and associates first described a deficiency in thoracic growth in children after repair of pectus excavatum that was most noticeable in children who underwent repair early during the preschool years. In 1996, Haller and co-workers reported three boys who had presented in their teens with apparent limited growth of the ribs after resection of the costal cartilages at an early age, producing a bandlike narrowing of the middle chest (acquired Jeune's disease) (Fig. 41-7). In some cases, the first and second ribs in which the costal cartilages have not been resected have apparent relative overgrowth, producing anterior protrusion of the upper sternum (Fig. 41-8). Haller and coinvestigators (1996) attributed this occurrence to injury during surgical repair of the costochondral junctions, which are the longitudinal growth centers for the ribs, and to decreased growth of the sternum resulting from injury to its growth centers or vascular supply.
Martinez and associates (1990) demonstrated experimentally in 6-week-old rabbits that resection of the costal cartilages produced a marked impairment in chest growth, particularly the anterior-posterior diameter, during a 5.5-month period of observation. Less severe impairment occurred if only the medial three fourths of the costal cartilage was resected, preserving the growth centers at the costochondral junction. This impairment was attributed to fibrosis and scarring within the perichondrial sheaths. Perichondrial sheaths, bone, or other prosthetic tissues that cannot grow also should not be joined posterior to the sternum because they will form a bandlike stricture across the chest. This complication of delayed thoracic growth was described primarily in children who underwent repair in early childhood and can be avoided by delaying surgery until the children are older. Preservation of the costochondral junction leaving a segment of the cartilage on the osseous portion of
P.665
the rib may partially minimize growth impairment. Weber and Kurkchubasche (1998) described a method of improving the severe pulmonary impairment encountered in one patient with the acquired Jeune's syndrome. In this patient, a sternotomy was performed and wedged open permanently using rib struts, the pleura was opened bilaterally, and bilateral resection of six ribs was performed. Pulmonary function was improved after the procedure in this patient.
 |
Fig. 41-7. Sequence of photographs demonstrating deterioration in the quality of a repair that can occur with time. This boy had an initial excellent result from a Welch repair with suture fixation of the sternum at age 4 years 3 months. The follow-up photographs at 7 years 6 months (A), 9 years 3 months (B), and 12 years 9 months (C) demonstrate progressive depression of the sternum and costal cartilages and relative overgrowth of the upper chest. |
 |
Fig. 41-8. A 10-year-old girl 7 years after repair of pectus excavatum. She demonstrates relative overgrowth of the upper costal cartilages, which were not resected during her repair, and a bandlike narrowing of her chest below that level. |
Patients are followed after surgery to full growth: age 16 for girls and 19 for boys. Use of clinical and Moir photography, as reported by Shochat and associates (1981), for initial evaluation and follow-up studies leads to improved clinical assessment of results and obviates the need for multiple radiographic examinations.
PECTUS CARINATUM
Pectus carinatum, or anterior protrusion of the sternum, occurs much less frequently than pectus excavatum: the former accounts for 16.7% of all chest wall deformities in the Boston Children's Hospital experience. The anterior protrusion occurs in a spectrum of configurations often divided into four categories (Table 41-5). The most frequent form,
P.666
termed chondrogladiolar by Brodkin (1949), consists of anterior protrusion of the body of the sternum with symmetric protrusion of the lower costal cartilages. Howard (1958) described it as appearing as if a giant hand had pinched the chest from the front, forcing the sternum and medial portion of the costal cartilages forward and the lateral costal cartilages and ribs inward (Fig. 41-9). Asymmetric deformities with anterior displacement of the costal cartilages on one side and normal cartilages on the contralateral side are less common (Fig. 41-10). Mixed lesions have a carinate deformity on one side and a depression or excavatum deformity on the contralateral side, often with sternal rotation. Some researchers classify these as a variant of the excavatum deformities. The most infrequent deformity is the upper chondromanubrial or pouter pigeon deformity. It consists of protrusion of the manubrium and second and third costal cartilages with relative depression of the body of the sternum (Fig. 41-11).
Table 41-5. Frequency of Pectus Carinatum Deformities | ||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||
 |
Fig. 41-9. A. Symmetric chondrogladiolar pectus carinatum in a 19-year-old man. B. Postoperative photograph shows correction of the protruding sternum and costal cartilages. |
 |
Fig. 41-10. A 12-year-old boy with marked asymmetric pectus carinatum demonstrates protrusion of the costal cartilages limited to the right side of the chest. |
Etiology
The etiology of pectus carinatum is no better understood than that of pectus excavatum. It appears as an overgrowth of the costal cartilages with forward buckling and anterior displacement of the sternum. Again, there is a clear-cut increased family incidence, which suggests a genetic basis. In a review by the author and Welch (1987) of 152 patients, 26% had a family history of chest wall deformity. A family history of scoliosis was detected in 12% of the patients. It was much more frequent in boys (n = 19, or 78%) than in girls (n = 33, or 22%). Scoliosis and other deformities of the spine are the most common associated musculoskeletal anomalies (Table 41-6).
Pectus carinatum usually appears in childhood, and in almost half of the patients the deformity was not identified until after the eleventh birthday (Fig. 41-12). The deformity may appear in mild form at birth, but often progresses during early childhood, particularly in the period of rapid growth at puberty. The chondromanubrial deformity, in contrast with the chondrogladiolar form, is often noted at birth
P.667
and is associated with a truncated, comma-shaped sternum with absent sternal segmentation or premature obliteration of the sternal sutures (Fig. 41-13). Currarino and Silverman (1958) described its association with an increased risk of congenital heart disease. Lees and Caldicott (1975) reviewed 1,915 radiographs and identified 135 children with sternal fusion anomalies. Eighteen percent of these children had documented congenital heart disease.
 |
Fig. 41-11. A. A 15-year-old boy with the chondromanubrial deformity. Note the posterior depression of the lower sternum, accentuated by the anterior bowing of the second and third costal cartilages. B. After repair, the sternal contour is improved and costal cartilages are reformed in a more appropriate fashion. From Shamberger RC, Welch KJ: Surgical correction of the chondromanubrial deformity. J Pediatr Surg 23: 319, 1988. With permission. |
Surgical Repair
Correction of carinate deformities has had a colorful history, beginning with the first repair by Ravitch (1952) of a chondromanubrial deformity. He resected multiple costal cartilages and performed a double sternal osteotomy. In 1953, Lester reported two methods of repair for chondrogladiolar deformity. The first approach, resection of the anterior portion of the sternum, was abandoned because of excessive blood loss and unsatisfactory results. The second method, subperiosteal resection of the entire sternum, was a no less radical technique. Chin (1957), and later Brodkin (1958), advanced the transected xiphoid and attached the rectus muscles to a higher site on the sternum, the xiphosternopexy. This produced posterior displacement of the sternum in younger patients with a flexible chest wall. Howard (1958) combined this method with subperichondrial costal cartilage resection and a sternal osteotomy. Ravitch (1960) reported repair of the chondrogladiolar deformity by resection of costal cartilage in a one- or two-stage procedure, with placement of reefing sutures to shorten and posteriorly displace the perichondrium. A sternal osteotomy was used in one of three cases. Robicsek and associates (1963) described repair by subperichondrial resection of costal cartilages, transverse sternal osteotomy, and resection
P.668
of the protruding lower portion of the sternum. The xiphoid and rectus muscles were reattached to the new lower margin of the sternum, pulling it posteriorly. In 1973, Welch and Vos reported an approach to these deformities that I continue to use today. Recent attempts at treating children with pectus carinatum by orthotic bracing have been reported by Haje and Bowen (1992) and Mielke and Winter (1993). Success has been achieved in younger children, but compliance is limited in older patients because of the pain or discomfort associated with the bracing.
Table 41-6. Musculoskeletal Abnormalities Identified in 30 of 152 Cases of Pectus Carinatum | ||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||
 |
Fig. 41-12. Age at appearance of pectus carinatum deformity in 141 infants and children. Note the appearance of protrusion in almost half of the children at puberty. From Shamberger RC, Welch KJ: Surgical correction of pectus carinatum. J Pediatr Surg 22:48, 1987. With permission. |
 |
Fig. 41-13. Lateral chest radiograph of a boy with chondromanubrial pectus carinatum. The short, comma-shaped sternum lacking segmentation is apparent. |
Surgical Technique
The placement of the skin incision, mobilization of the pectoral muscle flaps, and subperichondrial resection of the costal cartilage are identical to the method described for pectus excavatum. Management of the sternum is shown in Fig. 41-14 for the various deformities. In the chondromanubrial deformity, the costal cartilages must be resected
P.669
from the second cartilage inferiorly, as described by the author and Welch (1988). A single-limb medium Hemovac drain (Snyder Laboratories, Inc., New Philadelphia, OH, U.S.A.) is brought through the inferior skin flap, as for excavatum patients, with the suction ports in a parasternal position to the level of the highest resected costal cartilage. The pectoralis muscle flaps and skin flaps are closed. Perioperative antibiotics are used as in pectus excavatum.
 |
Fig. 41-14. A. A single or double osteotomy after resection of the costal cartilages allows posterior displacement of the sternum to an orthotopic position. B. The mixed pectus deformity is corrected by full and symmetric resection of the third to seventh costal cartilages, followed by transverse offset (0 10 degree wedge-shaped sternal osteotomy). Closure of this defect achieves both anterior displacement and rotation of the sternum. From Shamberger RC, Welch KJ: Surgical correction of pectus carinatum. J Pediatr Surg 22:48, 1987. With permission. C. The chondromanubrial type of deformity is depicted with a broad, wedge-shaped sternal osteotomy placed through the anterior cortex of the obliterated sternomanubrial junction. Closure of the osteotomy after fracture of the posterior cortex achieves a posterior displacement of the superior portion of the sternum, which is secured only by its attachment to the first rib. The lower portion of the sternum is overcorrected 20 to 35 degrees and is secured in position by strut or suture fixation. From Shamberger RC, Welch KJ: Surgical correction of chondromanubrial deformity. J Pediatr Surg 23:319, 1988. With permission. |
Table 41-7. Complications of Pectus Carinatum Repair: 7 Cases in 152 Patients | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||
Operative Results
Results are overwhelmingly successful in these patients. In a review of 152 cases by Welch and myself (1987), postoperative recovery was generally uneventful. Blood transfusions are rarely required, and none have been given in the past 10 years. There is a 3.9% complication rate (Table 41-7). Only three patients have required revision, each having additional lower costal cartilages resected for persistent unilateral malformation of the costal arch.
POLAND'S SYNDROME
In 1841, Poland described congenital absence of the pectoralis major and minor muscles associated with syndactyly. Despite a prior report of this entity by Froriep (1839), the label Poland's syndrome has been used since 1962, when Clarkson first applied it to a group of similar patients. Subsequent reports have described other components of the syndrome, including absence of ribs, chest wall depression, and abnormalities of the breasts. Each component of the syndrome may occur with variable severity. The extent of thoracic involvement may range from hypoplasia of the sternal head of the pectoralis major and minor muscles with normal underlying ribs to complete absence of the anterior portions of the second to fifth ribs and costal cartilages (Fig. 41-15). Breast involvement is frequent, ranging from mild hypoplasia to complete absence of the breast (amastia) and nipple (athelia) (Fig. 41-15C). Minimal subcutaneous fat and an absence of axillary hair are additional components. Hand deformities may include hypoplasia (brachydactyly) and fused fingers (syndactyly), primarily involving the central three digits. The most severe expression of the anomaly, mitten or claw deformity (ectromelia) is rare, as noted by Clarkson (1962) and Walker and associates (1969). Poland's syndrome may also occur in combination with Sprengel's deformity, in which there is decreased size, elevation, and winging of the scapula.
Poland's syndrome is present from birth and has an estimated incidence of 1 in 30,000 to 1 in 32,000, as reported by Freire-Maia and colleagues (1973) and McGillivray and Lowry (1977). Abnormalities in the breast can be defined at birth by absence of the underlying breast bud and the hypoplastic nipple, which is often superiorly displaced. The etiology of Poland's syndrome is unknown. Bouvet and associates (1978) have proposed hypoplasia of the ipsilateral subclavian artery as the origin of this malformation, but, as noted by David (1979), decreased blood flow to the extremity may be the consequence rather than the cause of decreased muscle mass of the hypoplastic limb. Although some forms of syndactyly have been described as autosomal-dominant traits, a similar pattern has not been demonstrated in patients with Poland's syndrome, which is generally sporadic. Multiple cases within a family are rare, as described by Sujansky and co-workers (1977), David (1982), and Cobben and associates (1989). Poland's syndrome is associated with a second rare syndrome, M bius' syndrome: bilateral or unilateral facial palsy and abducens oculi palsy. Nineteen such cases have been identified by Fontaine and Ovlaque (1984), but a unifying etiology is lacking. Boaz and colleagues (1971) have reported an unusual association between Poland's syndrome and childhood leukemia.
The Boston Children's Hospital experience with Poland's syndrome from 1970 to 1987, reported by myself and associates (1989), included 41 children and adolescents, of whom 21 were males. The lesion was right sided in 23 patients, left sided in 17 patients, and bilateral in 1 patient. Hand anomalies were noted in 23 (56%) patients and breast anomalies in 25 (61%) patients. In 10 children, the underlying thoracic abnormality required reconstruction, and in 3 children, rib or cartilage grafts were needed for complete repair.
Surgical Repair
Assessment of the extent of involvement of the various musculoskeletal components is critical for optimal thoracic reconstruction. If the extent of the deformity is limited to the sternal component of the pectoralis major and minor muscles, there is little functional deficit and repair is not necessary, except to facilitate breast augmentation in women (Fig. 41-16). If the underlying costal cartilages are depressed or absent, repair must be considered to minimize the concavity, to eliminate the paradoxic motion of the chest wall if ribs are absent, and in girls to provide an optimal base for breast reconstruction. Ravitch (1966) reported correction of posteriorly displaced costal cartilages by unilateral resection of the cartilages; a wedge osteotomy of the sternum, allowing rotation of the sternum; and fixation with Rehbein struts and Steinmann pins. I have achieved suitable repair in most cases with bilateral costal cartilage resection and an oblique osteotomy, which corrects the sternal rotation, as in the patients with mixed pectus carinatum and excavatum deformity (Fig. 41-17). The sternum is then displaced anteriorly
P.670
P.671
P.672
and supported with a retrosternal strut, which allows correction of the posteriorly displaced costal cartilages. An unappreciated carinate deformity is often present on the contralateral side, which accentuates the ipsilateral concavity (Fig. 41-16B).
 |
Fig. 41-15. A. Muscular 15-year-old boy with Poland's syndrome with loss of the left axillary fold owing to absence of the pectoralis major muscle. He has an orthotopic sternum and normal cartilages. He compensates adequately for loss of the pectoralis major and minor muscles. Surgery is not indicated in males with these findings. B. Eight-year-old boy with Poland's syndrome and more extensive thoracic involvement. The pectoralis major and minor muscles and the serratus to the level of the fifth rib are absent. There is sternal obliquity, and the third to fifth ribs are aplastic, ending at the level of the nipple. The corresponding cartilages are absent. The endothoracic fascia lies beneath a thin layer of subcutaneous tissue. Note the hypoplastic nipple and ectromelia of the ipsilateral hand, the most severe malformation of the hand associated with Poland's syndrome. C. Fourteen-year-old girl with Poland's syndrome. Note the high position of the right nipple, amastia, sternal rotation, and depressed right chest. The anterior second to fourth ribs and cartilages were missing. Breast augmentation will be required after the ipsilateral breast achieves full growth. |
 |
Fig. 41-16. The spectrum of thoracic abnormalities seen in Poland's syndrome. A. Most frequently, an entirely normal thorax is present, and only pectoral muscles are absent. B. Depression of the involved side of the chest wall, with rotation and often depression of the sternum. A carinate protrusion of the contralateral side is frequently present. C. Hypoplasia of ribs on the involved side but without significant depression may be seen. It usually does not require surgical correction. D. Aplasia of one or more ribs is usually associated with depression of adjacent ribs on the involved side and rotation of the sternum. From Shamberger RC, Welch KJ, Upton J III: Surgical treatment of thoracic deformity in Poland's syndrome. J Pediatr Surg 24:760, 1989. With permission. |
 |
Fig. 41-17. A. A transverse incision is placed below the nipple lines and, in females, in the inframammary crease. B. Schematic depiction of the deformity, with rotation of the sternum, depression of the cartilages of the involved side, and carinate protrusion of the contralateral side. C. In patients with aplasia of the ribs, the endothoracic fascia is encountered directly below the attenuated subcutaneous tissue and pectoral fascia. The pectoral muscle flap is elevated on the contralateral side, and the pectoral fascia, if present, on the involved side. Subperichondrial resection of the costal cartilages is then executed, as shown by the bold dashed lines. Rarely, this must be carried to the level of the second costal cartilages. D. A transverse, offset, wedge-shaped sternal osteotomy is created below the second costal cartilage. Closure of this defect with heavy silk sutures or elevation with a retrosternal strut corrects both the posterior displacement and the rotation of the sternum. E. In patients with rib aplasia, split rib grafts are harvested from the contralateral fifth or sixth rib and then secured medially with wire sutures into previously created sternal notches and with wire to the native ribs laterally. Ribs are split as shown along their short axis to maintain maximum mechanical strength. From Shamberger RC, Welch KJ, Upton J III: Surgical treatment of thoracic deformity in Poland's syndrome. J Pediatr Surg 24:760, 1989. With permission. |
Absence of the medial portion of the ribs can be managed with split rib grafts taken from the contralateral side. These must be secured to the sternum medially and to the dagger point hypoplastic rib ends laterally. The grafts can be covered with a prosthetic mesh, if needed, for further support. In these cases, it must be remembered that there is little tissue present between the endothoracic fascia and the fascial remnants of the pectoral muscles. Coverage of the area can also be augmented with transfer of a latissimus dorsi muscle flap. This is particularly helpful in girls who will require breast augmentation, as described by Ohmori and Takada (1980) and Haller and associates (1984). Flap rotation is seldom, if ever, required in boys and has the disadvantage of adding a second posterior thoracic scar and decreasing the strength of the latissimus dorsi muscle.
STERNAL DEFECTS
Sternal defects are rare compared with pectus excavatum and carinatum, yet they have received a great deal of attention in the medical literature because of their dramatic presentation and potentially fatal outcome. Entities involving failure of ventral fusion of the sternum can be divided into four groups: (a) cleft sternum, (b) thoracic ectopia cordis, (c) thoracoabdominal ectopia cordis, and (d) cervical ectopia cordis. The heart is in a normal position in the chest in cleft sternum but is displaced in the other three entities. In thoracic ectopia cordis, the heart protrudes anteriorly and is free of any covering tissues. In cervical ectopia cordis, the protrusion is even more pronounced and the heart is often fused with the head. In thoracoabdominal ectopia cordis, the heart is covered but often displaced into the abdomen through a defect in the diaphragm.
Cleft Sternum
An infant with cleft sternum has a complete or partial separation of the sternum but a normally positioned intrathoracic heart. This deformity results from nonfusion of the sternal bars, which should occur about the eighth week of gestation. In all such cases, despite the sternal separation, normal skin coverage is present, with an intact pericardium and a normal diaphragm. Omphaloceles do not occur in these children. The condition causes few functional difficulties. A dramatic increase in the deformity occurs with crying or Valsalva's maneuver. The sternal defects described in 109 cases were summarized by the author and Welch in 1990 (Table 41-8). The cleft almost invariably involves the upper sternum, whereas patients with thoracic or thoracoabdominal ectopia cordis have clefts primarily of the lower sternum.
The second distinction between cleft sternum and the other sternal defects is that children with cleft sternum rarely have
P.673
intrinsic congenital heart disease. An unexplained association does exist, however, between cleft sternum and cervicofacial hemangiomas, which were reported in 14 cases since the first description of this association by Fischer in 1879.
Table 41-8. Sternal Defects Reported in 109 Cases of Cleft Sternum | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||
Surgical Repair
Maier and Bortone accomplished the first primary repair of cleft sternum in 1949 in a 6-week-old infant. The flexibility of the newborn chest allows approximation of the sternum without cardiac compression (Fig. 41-18). A summary of the reported repairs for cleft sternum in 69 cases is shown in Table 41-9.
 |
Fig. 41-18. A. Repair of bifid sternum is best performed through a longitudinal incision extending the length of the defect. B. Directly beneath the subcutaneous tissues the sternal bars are encountered, with pectoral muscles present lateral to the bars. C. The endothoracic fascia is mobilized off the sternal bars posteriorly with blunt dissection to allow safe placement of the sutures. Approximation of the sternal bars may be facilitated by excising a wedge of cartilage inferiorly. D. Closure of the defect is achieved with 2 0 Tevdek or PDS (Ethicon, Inc., Sommerville, NJ, U.S.A.) sutures. From Shamberger RC, Welch KJ: Sternal defects. Pediatr Surg Int 5:156, 1990. With permission. |
Sabiston reported reconstruction of cleft sternum using multiple oblique chondrotomies (1958). The chondrotomies increase the chest wall dimensions and flexibility. The technique is useful in older infants and children with a less flexible chest and a wide defect. Meissner (1964) described a variation of repair in which the cartilages are divided laterally and swung medially to cover the defect. Autologous grafts of costal cartilage, split ribs, and segments of the costal arch have been used since Burton (1947) first repaired
P.674
this defect with a portion of the costal arch. Repairs with prosthetic material are far less satisfactory because of the risks for infection and the inability of these tissues to grow with the child. Most investigators now recommend treatment of cleft sternum in the newborn period, when simple direct closure is possible without the use of prosthetic materials or grafts.
Table 41-9. Methods of Repair of Cleft Sternum in 69 Cases | ||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||
Ectopia Cordis
Although treatment of isolated cleft sternum is routinely successful, surgical repair of ectopia cordis has a high mortality rate, particularly thoracic ectopia cordis. The lethal factor in thoracic ectopia cordis and cervical ectopia cordis is the extrathoracic location of the heart, which makes tissue coverage difficult. In thoracoabdominal ectopia cordis [the Cantrell pentalogy (1958)], the major impediment to survival is the high incidence of intrinsic congenital heart disease.
Etiology
The etiology of thoracic ectopia cordis and thoracoabdominal ectopia cordis is much debated. Higginbottom (1979), Opitz (1985), Hersh (1985), and Kaplan (1985) and their colleagues consider these anomalies to be the result of disruption of the amnion and possibly disruption of the chorionic layer or yolk sac as well. This disruption occurs during the third or fourth week of gestation at a time when cardiac chamber formation is occurring rapidly. This timing may account for the high incidence of abnormal cardiac development. Von Praagh (1987) has the intriguing notion, based on embryology studies by Patten (1946) and Bremer (1939), that acute hyperflexion of the craniocervical segment of the embryo pins the heart down in the extrathoracic position with the submental cardiac apex. The abnormal fetal configuration produced by oligohydramnios may persist to delivery and oppose traction by the gubernaculum cordis, which normally pulls the cardiac apex into caudal alignment. Chromosome abnormalities have been reported by Say and Wilsey (1978), King (1980), and Stoll and associates (1987), who also commented on the supraumbilical raphe and gubernaculum cordis.
Thoracic Ectopia Cordis
Thoracic ectopia cordis is one of the most dramatic occurrences in the delivery room (Fig. 41-19). The naked beating heart is external to the thorax. Clearly visible are the atrial appendages, coronary vasculature, and cephalic orientation of the cardiac apex. The gubernaculum cordis initially extends to the supraumbilical raphe. Thoracic ectopia cordis was first reported by Stensen (1671). Stensen's report was later translated by Willius (1948). Stensen identified the four components of the tetralogy of Fallot in this patient with thoracic ectopia cordis (such is the fate of eponyms). Cardiac anomalies are unusually frequent in thoracic ectopia cordis. Table 41-10 lists the cardiac anomalies reported up to 1990. Only 4 of 75 cases had no intrinsic cardiac anomalies.
Infants with thoracic ectopia cordis are severely deficient in the midline somatic tissues that normally cover the heart. Many attempts at primary closure fail because of the inability to mobilize adequate tissues for coverage. An abdominal defect is often present as well. Recent computed tomogram evaluation by Haynor and associates (1984) also shows reduced intrathoracic volume in these infants. Most allegedly successful repairs have been not of true thoracic ectopia cordis but, rather, thoracoabdominal ectopia cordis. Cutler and Wilens first attempted repair in 1925 by skin flap coverage, but failed because of cessation of cardiac function, presumably from compression of the heart. Only three reported survivors of more than 29 attempts have been recorded (Table 41-11).
 |
Fig. 41-19. An infant with thoracic ectopia cordis without a significant abdominal wall defect. The cardiac apex is cephalad. Any movement of the heart results in bradycardia and arrest. The patient had severe overriding of the aorta and complex tetralogy of Fallot. From Shamberger RC, Welch KJ: Sternal defects. Pediatr Surg Int 5:156, 1990. With permission. |
Table 41-10. Intrinsic Cardiac Lesions Reported: 75 Cases of Thoracic Ectopia Cordis | ||||||||||||||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||||||||||||||||||||||||||||
P.675
The first successful repair of ectopia cordis was achieved by Koop in 1975 and was reported by Saxena (1976). An infant with a normal heart had skin flap coverage at 5 hours of age, with inferior mobilization of the anterior attachments of the diaphragm. The sternal bars were 2 inches apart and could not be approximated primarily without cardiac compression and compromise. At 7 months of age, an acrylic resin of Dacron and Marlex mesh was inserted to close the sternal cleft, followed by primary skin closure. Necrosis of the skin flaps complicated the postoperative course with infection of the prosthetic material, which was later removed. This patient is alive at 20 years and is reported to be entirely well.
Table 41-11. Reported Survivors of Thoracic Ectopia Cordis and Their Repair | ||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||
Successful closure in two other infants is reported. Dobell and associates (1982) also achieved closure in two stages. Skin flap coverage was provided for the newborn. Rib strut grafts were placed over the sternal defect at 19 months of age and covered with pectoral muscle flaps. The pericardium was divided from its anterior attachments to the chest wall, allowing the heart to fall back partially into the thoracic cavity. Only Amato and colleagues (1988) achieved complete coverage of the heart in one stage. The unifying theme of successfully managed cases is mobilization of adequate soft tissue to cover the heart in its extrathoracic location and avoiding attempts to return the heart to an orthotopic location. Of note, in the successful cases, intrinsic cardiac lesions and associated abdominal defects were absent. These are the characteristics that most distinguish the successes from the failures, rather than any differences in surgical techniques. Coverage of the heart with autologous tissues, whether by flap rotation or bipedicle flaps, generally produces excessive compression on the heart, which limits cardiac output either by kinking outflow vessels or impeding cardiac filling. In most instances, attempts are abandoned in the operating room because of severe impairment of cardiac function. In patients who are repaired with autologous tissue grafts (bone or cartilage) or synthetic materials, infection and extrusion of the graft invariably occurs. Ultimate success with this lesion will be achieved only by accomplishing tissue coverage of the heart that avoids posterior displacement into an already limited thoracic space. This will require use of tissues from sites distant from the chest wall. Severe intracardiac defects associated with thoracic ectopia cordis in most cases also limit survival. The only recent advancement in management of this lesion has been early ultrasonographic diagnosis, as described by Kragt (1985) and Mercer (1983) and their colleagues, including definition of the intracardiac lesion and termination of the pregnancy, if acceptable to the parents.
Abdominal wall defects are also frequent in these patients, including an upper abdominal omphalocele or diastasis recti and, rarely, eventration of the abdominal viscera
P.676
(Fig. 41-20). Associated abdominal wall defects are summarized in Table 41-12. The presence of abdominal defects should not, however, lead to classification of these lesions as thoracoabdominal ectopia cordis, which should be reserved for those infants in whom the heart is covered at birth.
 |
Fig. 41-20. Infant with thoracic ectopia cordis and eventration of the abdominal viscera. |
Thoracoabdominal Ectopia Cordis (Cantrell's Pentalogy)
In thoracoabdominal ectopia cordis, the heart is covered by an omphalocelelike membrane or thin skin, which is often pigmented. The sternum is generally cleft inferiorly, and the heart lacks the severe anterior rotation present in thoracic ectopia cordis. An early report of this lesion by Wilson in 1798 clearly defined the associated somatic defects of the abdominal wall, diaphragm, and pericardium (Fig. 41-21) as well as the intrinsic cardiac anomalies. This entity was subsequently reviewed by Major (1953) and Cantrell and associates (1958). It is now frequently called Cantrell's pentalogy, although it was described long before Cantrell's relatively recent review. The five essential features of thoracoabdominal ectopia cordis are: (a) a cleft lower sternum; (b) a half moon-shaped anterior diaphragmatic defect resulting from lack of development of the septum transversum; (c) absence of the parietal pericardium at the diaphragmatic defect; (d) omphalocele (Table 41-13); (e) and, in most patients, an intrinsic cardiac anomaly (Table 41-14 and Fig. 41-22). A left ventricular diverticulum occurs with surprising frequency in this anomaly. In many cases, the diverticulum protrudes through the diaphragmatic and pericardial defects into the abdominal cavity.
Table 41-12. Abdominal Wall Defects Reported in 75 Cases of Thoracic Ectopia Cordis | ||||||||
|---|---|---|---|---|---|---|---|---|
| ||||||||
 |
Fig. 41-21. Earliest drawing of thoracoabdominal ectopia cordis. It clearly demonstrates the anterior semilunar diaphragmatic and pericardiac defects, allowing abdominal displacement of the heart. The cardiac defect was a single atrium and single ventricle (cor biloculare with truncus arteriosus). An omphalocele was found as well but is not shown. From Wilson J: A description of a very unusual formation of the human heart. Philos Trans R Soc Lond 11:346, 1798. With permission. |
Successful repair and long-term survival are more frequently achieved in thoracoabdominal ectopia cordis than in thoracic ectopia cordis. Arndt attempted the first repair in 1896, but return of the heart to the thoracic cavity resulted in death. Wieting performed the first successful surgical repair in 1912. He achieved primary closure of the diaphragm and abdominal wall fascia, but ignored the ventricular diverticulum. Initial surgical intervention must address the skin defects overlying the heart and abdominal cavity. Primary excision of the omphalocele with skin closure prevents
P.677
infection and mediastinitis, although several cases have been successfully managed by local application of topical astringents, thus allowing secondary epithelialization to occur. Several early cases, as in that of Cullerier (1806), document the long-term viability of individuals with thoracoabdominal ectopia cordis with intact skin coverage despite the abnormal location of the heart.
Table 41-13. Abdominal Wall Defects Reported in Patients with Thoracoabdominal Ectopia Cordis | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||
Table 41-14. Intrinsic Cardiac Lesions Reported in Patients with Thoracoabdominal Ectopia Cordis | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
 |
Fig. 41-22. Newborn male with thoracoabdominal ectopia cordis. Note flaring of the lower sternal area merging with large epigastric omphalocele. The septum transversum and the inferior portion of the pericardium were absent. Tetralogy of Fallot was present. |
Advances in cardiac surgery now allow correction of the intrinsic cardiac lesions, which were previously fatal. An aggressive approach to repair in infants with thoracoabdominal ectopia cordis is appropriate. Repair of the abdominal wall defect or diastasis has been achieved by primary closure or prosthetic mesh (Table 41-15). Primary closure of the thoracoabdominal defect may be difficult to achieve because of the wide separation of the rectus muscles and their superior attachment to the costal arches. Complete repair of the intracardiac defect is best performed before placement of prosthetic mesh overlying the heart. Repair of the abdomen and chest wall is important primarily for mechanical protection of the heart and abdominal viscera. Early diagnosis by prenatal ultrasonography has not altered the surgical approach or overall mortality of this lesion. Three cases in the Boston Children's Hospital series had severe pulmonary hypoplasia, which was lethal in two reported by the author and Welch (1990), a previously unreported association.
THORACIC DEFORMITIES IN DIFFUSE SKELETAL DISORDERS
Asphyxiating Thoracic Dystrophy (Jeune's Disease)
In 1954, Jeune and colleagues described a newborn with a narrow rigid chest and multiple cartilage anomalies. The patient died of respiratory insufficiency early in the perinatal period. Subsequent researchers have further characterized this
P.678
form of osteochondrodystrophy, which has variable degrees of skeletal involvement. It is inherited in an autosomal-recessive pattern and is not associated with chromosomal abnormalities. Its most prominent feature is a narrow, bell-shaped thorax and protuberant abdomen. The thorax is narrow in both the transverse and sagittal axis and has little respiratory motion due to the horizontal direction of the ribs (Fig. 41-23). The ribs are short and wide, and the splayed costochondral junctions barely reach the anterior axillary line. The costal cartilage is abundant and irregular, like a rachitic rosary. Microscopic examination of the costochondral junction demonstrates disordered and poorly progressing endochondral ossification, resulting in decreased rib length.
Table 41-15. Reported Methods of Repair of Thoracoabdominal Ectopia Cordis | ||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||
 |
Fig. 41-23. Jeune's disease (asphyxiating thoracic dystrophy). A. Anteroposterior radiograph shows short horizontal ribs and narrow chest. B. Lateral radiograph demonstrates that the short ribs end at the midaxillary line. Abnormal flaring at the costochondral junctions is also present. The patient died of progressive respiratory insufficiency at 1 month of age. There was no surgical intervention. Postmortem examination revealed alveolar hypoplasia. |
Skeletal abnormalities associated with this syndrome include short stubby extremities with relatively short and wide bones. The clavicles are in a fixed and elevated position, and the pelvis is small and hypoplastic, with square iliac bones.
The syndrome has a variable extent of pulmonary impairment. Although the initial cases reported resulted in neonatal deaths, subsequent reports by Kozlowski and Masel (1976) and others have documented that infants can survive for longer intervals of time with this syndrome. The pathologic findings in autopsy cases reveal a range of abnormal pulmonary development. In most cases, the bronchial development is normal and there are fewer alveolar divisions, as described by Williams and associates (1984).
 |
Fig. 41-24. Chest radiograph of an infant with spondylothoracic dysplasia. Severe abnormality of the spine is apparent, with multiple alternating hemivertebrae producing a crablike configuration to the ribs. |
P.679
Spondylothoracic Dysplasia (Jarcho-Levin Syndrome)
Spondylothoracic dysplasia is an autosomal-recessive deformity with multiple vertebral and rib malformations, described by Jarcho and Levin in 1938. Infants and children with this syndrome have multiple alternating hemivertebrae in most, if not all, of the thoracic and lumbar spine. The vertebral ossification centers rarely cross the midline, although bone formation is normal. Multiple posterior fusions of the ribs and remarkable shortening of the thoracic spine result in a crablike appearance of the ribs on the chest radiograph (Fig. 41-24).
The thoracic deformity is secondary to the spine anomaly, which results in close posterior approximation of the origin of the ribs. Although most infants with the entity die before 15 months of age, as reviewed by Roberts and colleagues (1988), no surgical efforts have been proposed or attempted. One third of patients with this syndrome have associated malformations, including congenital heart disease and renal anomalies. Heilbronner and Renshaw (1984) have reported its occurrence primarily in Puerto Rican families (15 of 18 cases).
REFERENCES
Pectus Excavatum
Adkins PC, Blades B: A stainless steel strut for correction of pectus excavatum. Surg Gynecol Obstet 113:111, 1961.
Allen RG, Douglas M: Cosmetic improvement of thoracic wall defects using a rapid setting Silastic mold: a special technique. J Pediatr Surg 14:745, 1979.
Backer OG, Br nner S, Larsen V: The surgical treatment of funnel chest: initial and follow-up results. Acta Chir Scand 121:253, 1961.
Baronofsky ID: Technique for the correction of pectus excavatum. Surgery 42:884, 1957.
Beiser GD, et al: Impairment of cardiac function in patients with pectus excavatum, with improvement after operative correction. N Engl J Med 287:267, 1972.
Beveg rd S: Postural circulatory changes at rest and during exercise in patients with funnel chest, with special reference to factors affecting the stroke volume. Acta Med Scand 171:695, 1962.
Bien G: Zur Anatomie und tiolgie der Trichterbrust. Beitr Pathol Anat Allg Pathol 52:567, 1912.
Blickman JG, et al: Pectus excavatum in children: pulmonary scintigraphy before and after corrective surgery. Radiology 156:781, 1985.
Bon Tempo CP, et al: Radiographic appearance of the thorax in systolic click-late systolic murmur syndrome. Am J Cardiol 236:27, 1975.
Brown AL, Cook O: Cardio-respiratory studies in pre- and postoperative funnel chest (pectus excavatum). Dis Chest 20:378, 1951.
Cahill JL, Lees GM, Robertson HT: A summary of preoperative and postoperative cardiorespiratory performance in patients undergoing pectus excavatum and carinatum repair. J Pediatr Surg 19:430, 1984.
Castile RG, Staats BA, Westbrook PR: Symptomatic pectus deformities of the chest. Am Rev Respir Dis 126:564, 1982.
Croitoru DP, et al: Experience and modification update for the minimally invasive Nuss technique for pectus excavatum repair in 303 patients. J Pediatr Surg 37:437, 2002.
Derveaux L, et al: Mechanism of pulmonary function changes after surgical correction for funnel chest. Eur Respir J 1:823, 1988.
Derveaux L, et al: Preoperative and postoperative abnormalities in chest x-ray indices and in lung function in pectus deformities. Chest 95:850, 1989.
Ellis DG, Snyder CL, Mann CM: The re-do chest wall deformity correction. J Pediatr Surg 32:1267, 1997.
Engum S, et al: Is the grass greener? Early results of the Nuss procedure. J Pediatr Surg 35:246, 2000.
Fonkalsrud EW, et al: Comparison of minimally invasive and modified Ravitch pectus excavatum repair. J Pediatr Surg 37:413, 2002.
Garusi GF, D'Ettorre A: Angiocardiographic patterns in funnel-chest. Cardiologia 45:312, 1964.
Gattiker H, B hlmann A: Cardiopulmonary function and exercise tolerance in supine and sitting position in patients with pectus excavatum. Helv Med Acta 33:122, 1967.
Greig JD, Azmy AF: Thoracic cage deformity: a late complication following repair of an agenesis of diaphragm. J Pediatr Surg 25:1234, 1990.
Haller JA Jr, et al: Pectus excavatum. A 20 year surgical experience. J Thorac Cardiovasc Surg 60:375, 1970.
Haller JA Jr, Kramer SS, Lietman SA: Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report. J Pediatr Surg 22:904, 1987.
Haller JA Jr, et al: Chest wall constriction after too extensive and too early operations for pectus excavatum. Ann Thorac Surg 61:1618, 1996.
Hebra A, et al: Outcome analysis of minimally invasive repair of pectus excavatum: review of 251 cases. J Pediatr Surg 35:252, 2000.
Hebra A, et al: Pectus carinatum as a sequela of minimally invasive pectus excavatum repair. Pediatr Endosurg Innov Techniques 6:41, 2002.
Hecker WC, Procher G, Dietz HG: Results of operative correction of pigeon and funnel chest following a modified procedure of Ravitch and Haller. Z Kinderchir 34:220, 1981.
Hougaard K, Arendrup H: Deformities of the female breasts after surgery for funnel chest. Scand J Thorac Cardiovasc Surg 17:171, 1983.
Howard R: Funnel chest: its effect on cardiac function. Arch Dis Child 32:5, 1959.
H mmer HP, Willital GH: Morphologic findings of chest deformities in children corresponding to the Willital-H mmer classification. J Pediatr Surg 19:562, 1984.
Humphreys GH II, Jaretzki A III: Pectus excavatum. Late results with and without operation. J Thorac Cardiovasc Surg 80:686, 1980.
Jensen NK, et al: Pectus excavatum and carinatum: the how, when and why of surgical correction. J Pediatr Surg 5:4, 1970.
Judet J, Judet R: Thorax en entonnoir. Un proc d op ratoire. Rev Orthop 40:248, 1954.
Jung A: Le traitement du thorax en entonnoir par le retournement p dicul de la cuvette sterno-chondrale. Mem Acad Chir 82:242, 1956.
Kaguraoka H, et al: Degree of severity of pectus excavatum and pulmonary function in preoperative and postoperative periods. J Thorac Cardiovasc Surg 104:1483, 1992.
P.680
Kowalewski J, et al: Pectus excavatum: increase of right ventricular systolic, diastolic, and stroke volumes after surgical repair. J Thorac Cardiovasc Surg 118:87, 1999.
Lester CW: The etiology and pathogenesis of funnel chest, pigeon breast, and related deformities of the anterior chest wall. J Thorac Surg 34:1, 1957.
Martinez D, et al: The effect of costal cartilage resection on chest wall development. Pediatr Surg Int 5:170, 1990.
Mead J, et al: Rib cage mobility in pectus excavatum. Am Rev Respir Dis 132:1223, 1985.
Meyer L: Zur chirurgischen behandlung der angeborenen trichterbrust. Verh Berl Med Gesellschaft 42:364, 1911.
Molik KA, et al: Pectus excavatum repair: experience with standard and minimal invasive techniques. J Pediatr Surg 36:324, 2001.
Morshuis W, et al: Pulmonary function before surgery for pectus excavatum and at long-term follow-up. Chest 105:1646, 1994a.
Morshuis WJ, et al: Exercise cardiorespiratory function before and one year after operation for pectus excavatum. J Thorac Cardiovasc Surg 107:1403, 1994b.
Nuss D, et al: A 10-year review of a minimally invasive technique for the correction of pectus excavatum. J Pediatr Surg 33:545, 1998.
Ochsner A, DeBakey M: Chone-chondrosternon: report of a case and review of the literature. J Thorac Surg 8:469, 1939.
Oelsnitz G: Fehlbildungen des brustkorbes. Z Kinderchir 33:229, 1981.
Paltia V, et al: Operative technique in funnel chest: experience in 81 cases. Acta Chir Scand 116:90, 1958.
Peterson RJ, et al: Noninvasive assessment of exercise cardiac function before and after pectus excavatum repair. J Thorac Cardiovasc Surg 90:251, 1985.
Quigley PM, et al: Cardiorespiratory function before and after corrective surgery in pectus excavatum. J Pediatr 128:638, 1996.
Ravitch MM: The operative treatment of pectus excavatum. Ann Surg 129:429, 1949.
Ravitch MM: Congenital Deformities of the Chest Wall and Their Operative Correction. Philadelphia: WB Saunders, 1977.
Rehbein F, Wernicke HH: The operative treatment of the funnel chest. Arch Dis Child 32:5, 1957.
Saint-Mezard G, et al: Prolapsus valvulaire mitral et pectus excavatum: association fortuite ou groupement syndromique? [Mitral valve prolapse and pectus excavatum. Fortuitous association or syndrome?] Presse Med 15:439, 1986.
Salomon J, Shah PM, Heinle RA: Thoracic skeletal abnormalities in idiopathic mitral valve prolapse. Am J Cardiol 36:32, 1975.
Sanger PW, Robicsek F, Daugherty HK: The repair of recurrent pectus excavatum. J Thorac Cardiovasc Surg 56:141, 1968.
Sauerbruch F: Die Chirurgie der Brustorgane. Berlin: Springer, 1920, p. 437.
Schaub VF, Wegmann T: Elektrokardiographische ver nderungen bei trichterbrust. Cardiologia 24:39, 1954.
Scherer LR, et al: Surgical management of children and young adults with Marfan syndrome and pectus excavatum. J Pediatr Surg 23:1169, 1988.
Schutte JE, et al: Distinctive anthropometric characteristics of women with mitral valve prolapse. Am J Med 71:533, 1981.
Shamberger RC, Welch KJ: Cardiopulmonary function in pectus excavatum. Surg Gynecol Obstet 166:383, 1988a.
Shamberger RC, Welch KJ: Surgical repair of pectus excavatum. J Pediatr Surg 23:615, 1988b.
Shamberger RC, Welch KJ, Sanders S: Mitral valve prolapse associated with pectus excavatum. J Pediatr Surg 111:404, 1987.
Shamberger RC, et al: Anterior chest wall deformities and congenital heart disease. J Thorac Cardiovasc Surg 96:427, 1988.
Shochat SJ, et al: Moir phototopography in the evaluation of anterior chest wall deformities. J Pediatr Surg 16:353, 1981.
Taguchi K, et al: A new plastic operation for pectus excavatum: sternal turnover surgical procedure with preserved internal mammary vessels. Chest 67:606, 1975.
Tanaka F, et al: Postoperative lung function in patients with funnel chest. J Jpn Assoc Thorac Surg 41:2161, 1993.
Udoshi M, et al: Incidence of mitral valve prolapse in subjects with thoracic skeletal abnormalities a prospective study. Am Heart J 97:303, 1979.
Vanamo K, et al: Chest wall and spinal deformities in adults with congenital diaphragmatic defects. J Pediatr Surg 31:851, 1996.
Wada J, et al: Results of 271 funnel chest operations. Ann Thorac Surg 10:526, 1970.
Waters PM, et al: Scoliosis in children with pectus excavatum and pectus carinatum. J Pediatr Orthop 9:551, 1989.
Weber TR, Kurkchubasche AG: Operative management of asphyxiating thoracic dystrophy after pectus repair. J Pediatr Surg 33:262, 1998.
Weg JG, Krumholz RA, Harkleroad LE: Pulmonary dysfunction in pectus excavatum. Am Rev Respir Dis 96:936, 1967.
Welch KJ: Satisfactory surgical correction of pectus excavatum deformity in childhood: a limited opportunity. J Thorac Surg 36:697, 1958.
Welch KJ: Chest wall deformities. In Holder TM, Ashcraft KW (eds): Pediatric Surgery. Philadelphia: WB Saunders, 1980.
Welch KJ, Kearney GP: Abdominal musculature deficiency syndrome: prune belly. J Urol 111:693, 1974.
Willital GH: Operationsindikation-Operationstechnik bei Brustkorbdeformierungen. Z Kinderchir 33:244, 1981.
Wynn SR, et al: Exercise cardiorespiratory function in adolescents with pectus excavatum. Observations before and after operation. J Thorac Cardiovasc Surg 99:41, 1990.
Pectus Carinatum
Brodkin HA: Congenital chondrosternal prominence (Pigeon breast): a new interpretation. Pediatrics 3:286, 1949.
Brodkin HA: Pigeon breast congenital chondrosternal prominence. Arch Surg 77:261, 1958.
Chin EF: Surgery of funnel chest and congenital sternal prominence. Br J Surg 44:360, 1957.
Currarino G, Silverman FN: Premature obliteration of the sternal sutures and pigeon-breast deformity. Radiology 70:532, 1958.
Haje SA, Bowen JR. Preliminary results of orthotic treatment of pectus deformities in children and adolescents. J Pediatr Orthop 12:795, 1992.
Howard R: Pigeon chest (protrusion deformity of the sternum). Med J Aust 2:664, 1958.
Lees RF, Caldicott JH: Sternal anomalies and congenital heart disease. Am J Roentgenol Radium Ther Nucl Med 124:423, 1975.
Lester CW: Pigeon breast (pectus carinatum) and other protrusion deformities of the chest of developmental origin. Ann Surg 137:482, 1953.
Mielke CH, Winter RB. Pectus carinatum successfully treated with bracing: a case report. Int Orthop 17:350, 1993.
Ravitch MM: Unusual sternal deformity with cardiac symptoms operative correction. J Thorac Surg 23:138, 1952.
Ravitch, MM: The operative correction of pectus carinatum (pigeon breast). Ann Surg 151:705, 1960.
Robicsek F, et al: The surgical treatment of chondrosternal prominence (pectus carinatum). J Thorac Cardiovasc Surg 45:691, 1963.
Shamberger RC, Welch KJ: Surgical correction of pectus carinatum. J Pediatr Surg 22:48, 1987.
Shamberger RC, Welch KJ: Surgical correction of chondromanubrial deformity (Currarino Silverman syndrome). J Pediatr Surg 23:319, 1988.
Welch KJ, Vos A: Surgical correction of pectus carinatum (pigeon breast). J Pediatr Surg 8:659, 1973.
Poland's Syndrome
Boaz D, Mace JW, Gotlin RW: Poland's syndrome and leukaemia. Lancet 1:349, 1971.
Bouvet JP, et al: Vascular origin of Poland syndrome? A comparative rheographic study of the vascularisation of the arms in eight patients. Eur J Pediatr 128:17, 1978.
Clarkson P: Poland's syndactyly. Guy Hosp Rep 111:335, 1962.
Cobben JM, et al: Poland anomaly in mother and daughter. Am J Med Genet 33:519, 1989.
David TJ: Vascular origin of Poland syndrome? Eur J Pediatr 130:299, 1979.
David TJ: Familial Poland anomaly. J Med Genet 19:293, 1982.
Fontaine G, Ovlaque S: Le syndrome de Poland-M bius. Arch Fr Pediatr 41:351, 1984.
Freire-Maia N, et al: The Poland Syndrome clinical and genealogical data, dermatoglyphic analysis, and incidence. Hum Hered 23:97, 1973.
Froriep R: Beobachtung eines Falles Von Mangel der Brustdr se. Notizen Gebiete Naturund Heilkunde 10:9, 1839.
Haller JA Jr, et al: Early reconstruction of Poland's syndrome using autologous rib grafts combined with a latissimus muscle flap. J Pediatr Surg 19:423, 1984.
P.681
McGillivray BC, Lowry RB: Poland syndrome in British Columbia: incidence and reproductive experience of affected persons. Am J Med Genet 1:65, 1977.
Ohmori K, Takada H: Correction of Poland's pectoralis major muscle anomaly with latissimus dorsi musculocutaneous flaps. Plast Reconstr Surg 65:400, 1980.
Poland A: Deficiency of the pectoralis muscles. Guy Hosp Rep 6:191, 1841.
Ravitch MM: Atypical deformities of the chest wall absence and deformities of the ribs and costal cartilages. Surgery 59:438, 1966.
Shamberger RC, Welch KW, Upton J III: Surgical treatment of thoracic deformity in Poland's syndrome. J Pediatr Surg 24:760, 1989.
Sujansky E, Riccardi VM, Matthew AL: The familial occurrence of Poland syndrome. Birth Defects 13:117, 1977.
Walker JC Jr, Meijer R, Aranda D: Syndactylism with deformity of the pectoralis muscle. Poland's syndrome. J Pediatr Surg 4:569, 1969.
Sternal Clefts
Burton JF: Method of correction of ectopia cordis. Arch Surg 54:79, 1947.
Fischer H: Fissura sterni congenita mit partieller Bauchspalte. Dtsch Z Chir 12:367, 1879.
Maier HC, Bortone F: Complete failure of sternal fusion with herniation of pericardium. J Thorac Surg 18:851, 1949.
Meissner F: Fissura sterni congenita. Zentralbl Chir 89:1832, 1964.
Sabiston DC Jr: The surgical management of congenital bifid sternum with partial ectopia cordis. J Thorac Surg 35:118, 1958.
Shamberger RC, Welch KJ: Sternal defects. Pediatr Surg Int 5:156, 1990.
Ectopia Cordis
Amato JT, Cotroneo JV, Gladieri RJ: Repair of complete ectopia cordis [Film]. Presented at the American College of Surgeons Clinical Congress, Chicago, October 23 28, 1988.
Bremer L: Textbook of Embryology. Philadelphia: WB Saunders, 1939.
Cantrell JR, Haller JA, Ravitch MM: A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 107:602, 1958.
Cutler GD, Wilens G: Ectopia cordis: report of a case. Am J Dis Child 30:76, 1925.
Dobell ARC, Williams HB, Long R: Staged repair of ectopia cordis. J Pediatr Surg 17:353, 1982.
Haynor DR, et al: Imaging of fetal ectopia cordis: roles of sonography and computed tomography. J Ultrasound Med 3:25, 1984.
Hersh JH, et al: Sternal malformation/vascular dysplasia association. Am J Med Genet 21:177, 1985.
Higginbottom MC: The amniotic band disruption complex: timing of amniotic rupture and variable spectra of consequent defects. Pediatrics 95:544, 1979.
Kaplan LC, et al: Ectopia cordis and cleft sternum: evidence for mechanical teratogenesis following rupture of the chorion or yolk sac. Am J Med Genet 21:187, 1985.
King CR: Ectopia cordis and chromosome errors. Pediatrics 66:328, 1980.
Kragt H, et al: Prenatal ultrasonic diagnosis and management of ectopia cordis. Eur J Obstet Gynecol Reprod Biol 20:177, 1985.
Mercer LJ, Petres RE, Smeltzer JS: Ultrasonic diagnosis of ectopia cordis. Obstet Gynecol 61:523, 1983.
Opitz JM: Editorial comment following paper by Hersh et al and Kaplan et al on sternal cleft. Am J Med Genet 21:201, 1985.
Patten BM: Human Embryology. Toronto: Blakiston, 1946.
Saxena N: Ectopia cordis child surviving; prosthesis fails. Pediatr News 10:3, 1976.
Say B, Wilsey CE: Chromosome aberration in ectopia cordis (46,XX,17q+). Am Heart J 95:274, 1978.
Stensen N: An unusually early description of the so-called tetralogy of Fallot. In Bartholin T (ed): Acta Medica et Philosophica Hafnienca. Vol. 1. 1671 1672, p. 202. [Translated into English by Willius FA. Proc Staff Meet Mayo Clin 23:316, 1948.]
Stoll SC, Vivier M, Renaud R: A supraumbilical midline raphe with sternal cleft in a 47,XXX woman. Am J Med Genet 27:229, 1987.
Willius FA: An unusually early description of the so-called tetralogy of Fallot. Proc Staff Meet Mayo Clin 23:316, 1948.
VonPraagh: Personal communication, 1987.
Thoracoabdominal Ectopia Cordis(Cantrell's Pentalogy)
Arndt C: Nabelschnurbruch mit Herzhernie. Operation durch Laparotomie mit t dlichem Ausgang. Centralbl Gynakol 20:632, 1896.
Cantrell JR, Haller JA, Ravitch MM: A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 107:602, 1958.
Cullerier M: Observation sur un d placement remarquable du coeur; par M. Deschamps, m decin Laval. J Gen Med Chir Pharm 26:275, 1806.
Major JW: Thoracoabdominal ectopia cordis. J Thorac Surg 26:309, 1953.
Shamberger RC, Welch KJ: Sternal defects. Pediatr Surg Int 5:156, 1990.
Wieting: Eine operative behandelte Herzmissbildung. Dtsch Z Chir 114:293, 1912.
Wilson J: A description of a very unusual formation of the human heart. Philos Trans R Soc Lond Biol Sci 2:346, 1798.
Miscellaneous Conditions
Heilbronner DM, Renshaw TS: Spondylothoracic dysplasia. A case report. J Bone Joint Am Surg 66:302, 1984.
Jarcho S, Levin PM: Hereditary malformation of the vertebral bodies. Bull Johns Hopkins Hosp 62:216, 1938.
Jeune M, et al: Polychondrodystrophie avec blocage thoracique d'evolution fatale. Pediatrie 9:390, 1954.
Kozlowski K, Masel J: Asphyxiating thoracic dystrophy without respiratory disease: report of two cases of the latent form. Pediatr Radiol 5:30, 1976.
Roberts AP, et al: Spondylothoracic and spondylocostal dysostosis. Hereditary forms of spinal deformity. J Bone Joint Surg 70:123, 1988.
Williams AJ, Vawter G, Reid LM: Lung structure in asphyxiating thoracic dystrophy. Arch Pathol Lab Med 108:658, 1984.
EAN: 2147483647
Pages: 203