Kenneth J. Welch

Surgical repair of pectus excavatum

From 1958 to March 1987 we corrected 704 patients with pectus excavatum. The condition occurred more frequently in boys (544 patients) than girls (160 patients). In the majority of patients (86%), the defect was evident at birth or within the first year of life. Musculoskeletal abnormalities were identified in 133 patients (scoliosis, 107; kyphosis, 4; myopathy, 3; Polands syndrome, 3; Marfans syndrome, 2; Pierre Robin syndrome, 2; prune belly syndrome, 2; neurofibromatosis, 3; cerebral palsy, 4; tuberous sclerosis, 1; and congenital diaphragmatic hernia, 2). Sixteen patients had associated congenital heart disease. A family history of chest wall deformity was present in 37% of the cases and a history of scoliosis in 11%. Surgical correction was performed using a uniform technique for bilateral subperichondrial resection of the deformed costal cartilages and sternal osteotomy resecting a wedge of the anterior cortex and fracturing the posterior cortex. Anterior displacement was maintained with silk sutures closing the osteotomy defect. In 28 early cases, the sternum was secured by intramedullary fixation with a Steinman pin. All repairs were completed with a low complication rate (4.4%; pneumothorax, 11; wound infection, 5; wound hematoma, 3; wound dehiscence, 5; pneumonia, 3; seroma, 1; hemoptysis, 1; hemopericardium, 1). Six complications were associated with Steinman pin fixation (hemoptysis, seroma, hemopericardium, pneumothorax, 3). Major recurrence occurred in 17 patients (2.7%) and led to revision in 12. Satisfactory long-term results were achieved in the remaining 687 patients, with follow-up ranging from 2 weeks to 27 years. Mean follow-up was 4.3 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical treatment of thoracic deformity in Poland's syndrome

In 1841, Poland described congenital deficiency of the pectoralis major and minor muscles associated with syndactyly. This syndrome is a spectrum, often involving chest wall and breast deformity as well. Identification of the various musculoskeletal components involved permits optimal thoracic reconstruction in the small proportion of patients who will require it. From 1955 to 1988, 75 patients (40 males and 35 females) with Polands syndrome were treated or evaluated. Patients with isolated deficiencies of the pectoral muscles, breast, or hand deformity were excluded. The complex was right-sided in 44 patients, left-sided in 30, and bilateral in one. The pectoralis minor and the costal portion of the pectoralis major muscle were absent in all patients. Hand anomalies were present in 50 patients. Athelia and/or amastia were noted in 37 patients. In ten patients, the rib cage deformity required reconstruction, and in three cases, rib or cartilage grafts were needed for complete repair. Often unappreciated in these cases is the significant rotation of the sternum toward the involved side and contralateral carinate deformity. Correction is achieved by bilateral subperichondrial costal cartilage resection and sternal osteotomy (seven of ten patients), thus allowing anterior displacement and orthorotation of the sternum. Chest wall reconstruction must be tailored to the requirements of each patient. No intraoperative or postoperative complications occurred in these ten patients. In males without rib cage deformity, generally no treatment is required to replace the absent pectoral muscles, although in two cases rotation of the latissimus dorsi muscle was performed. In all females, reconstruction of the ipsilateral breast is required at full development.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical correction of pectus carinatum

Pectus carinatum is an infrequent but eminently correctable chest wall deformity. It is encountered much less frequently than pectus excavatum. In 12 years, from 1973 to 1985, 152 pectus carinatum (16.7%) and 758 pectus excavatum deformities (83.3%) were corrected. It occurs more frequently in boys (119 patients) than girls (33 patients). The majority, 89 cases, were symmetric, while 49 were asymmetric, and 14 were mixed deformities (ipsilateral carinatum, contralateral excavatum). In almost half the patients the deformity was not identified until after the 11th birthday. A family history of chest wall deformities was present in 26%, and of scoliosis in 12%. Associated musculoskeletal abnormalities were identified in 34 patients (scoliosis 23, Polands syndrome 4, neurofibromatosis 2, Morquios disease 2, vertebral anomalies 1, hyperlordosis 1, and kyphosis 1). Surgical correction required bilateral resection of the third through seventh costal cartilages in 143 patients, and unilateral resection in nine patients with an isolated abnormality. A single osteotomy was used in 88 patients and a double osteotomy in 53 patients. In 11 cases no osteotomy was required. Mixed deformity with posterior angulation of the sternum was managed by osteotomy and anterior displacement. The remaining cases had sternal osteotomy and fracture of the posterior cortex to correct anterior angulation. The operation was completed with a low complication rate 3.9% (pneumothorax 4, wound infection 1, atelectasis 1, and local tissue necrosis 1). Three patients required revision with additional unilateral lower cartilage resection for persistent malformation of the costal arch. All patients ultimately had a satisfactory result.

Sacrococcygeal germ cell tumors in childhood: An updated experience with 118 patients

The histopathology of 118 sacrococcygeal germ cell tumors (SGCT) was correlated with clinical presentation, therapeutic management, and prognosis. There were 97 teratomas (78 mature, 19 immature), 19 embryonal, and 2 anaplastic carcinomas. Mature and immature teratomas usually presented externally (AAP type I) permitting relatively early detection and surgical removal. The immature group was significantly larger (11.6 cm vs. 7.5 cm) with a greater incidence of subsequent embryonal carcinoma (16%). The embryonal and anaplastic carcinomas were diagnosed at a later age (average 21 mo) and had a substantial presacral or endopelvic component (AAP types II-IV); this group had the worst prognosis with no survivors. Our data suggest that combined analysis of clinical findings and histopathology may help to identify some of the children at risk for early recurrence.

Scoliosis in children with pectus excavatum and pectus carinatum

Between 1974 and 1985, 461 patients with pectus excavatum and 135 patients with pectus carinatum underwent operative repair of their anterior chest wall deformities. Twenty-one percent of patients with anterior chest wall deformity had mild scoliosis by clinical and radiographic examination. The average lateral spinal deformity was 15° (range 6–78°) for pectus excavatum patients and 16° (range 5–57°) for pectus carinatum patients. Eighteen percent of the pectus excavatum patients with scoliosis and 14% of the pectus carinatum patients with scoliosis required therapeutic intervention of bracing and/ or arthrodesis.

Surgical treatment of thymic cysts and neoplasms in children

Based on experience with 17 patients and review of the literature, complete excision is possible for all benign thymic cysts and neoplasms. Recurrence is rare (2%). Most malignant thymomas in children are epithelial (73%). As with adults, prognosis depends on accurate surgical staging and aggressive surgical intervention. Even the largest tumors can be completely excised removing portions of lung, pleura, diaphragm, and pericardium in continuity as required. Cardiopulmonary bypass may be necessary when the innominate veins or the superior vena cava is involved. Irradiation is required for stage II and stage III lesions. Stage IV patients require irradiation and chemotherapy.

Surgical correction of chondromanubrial deformity (Currarino silverman syndrome)

Chondromanubrial (arcuate) pectus carinatum is the rarest protrusion deformity of the chest. Its surgical correction was first described by Ravitch in 1952. We have recently encountered five patients with this deformity who have provided additional insight into the anatomy and optimal repair of this condition. It is notable for a short nonsegmented sternum with marked posterior angulation at the site of the normal chondromanubrial junction. It is optimally corrected by subperichondrial resection of the second to the seventh costal cartilages with a broad wedge-shaped osteotomy through the anterior cortex of the sternum at the point of maximal angulation. Anterior displacement of the sternum is achieved by closing the osteotomy with heavy silk sutures while the costal cartilages are regenerating.

Pulmonary agenesis (Aplasia), esophageal atresia, and Tracheoesophageal fistula: A different treatment strategy

This is a report of a patient with esophageal atresia, distal tracheoesophageal fistula, and right pulmonary agenesis who survived for 10 months. Death was due to progressive respiratory failure following a complicated clinical course. Several lessons were learned. First, the remaining lung is at high risk. Since the number of respiratory units in these infants is already decreased, the loss of more units has a more profound effect than in an otherwise healthy child. Second, while the establishment of esophageal continuity is important, all attempts to perform an esophagoesophagostomy should be abandoned if doing so further jeopardizes the solitary lung. Third, since all of these children have some degree of tracheomalacia, early tracheostomy should be considered. The tracheostomy will not only act as a tracheal stent but will also avoid progressive laryngeal pathology, which would result from long-term endotracheal intubation.

Non-Obstructive Megacystis and Refluxing Megaureter in Pre-Teen Enuretic Boys with Minimal Symptoms

We herein describe 4 older boys with non-obstructive, non-neurogenic refluxing megacystis-megaureter syndrome. These patients require immediate investigation and aggressive surgical intervention, the goal being total reconstruction of the bladder and upper tracts in continuity. This goal was achieved in 3 patients with followup in excess of 4 years. Failure to recognize the syndrome, as with our fourth patient, may result in improper classification and treatment. A plea is made for urological investigation of pernicious nocturnal enuresis in older boys and in all children with a history of urinary tract infection.