Conservatively aggressive management with bilateral Wilms' tumors.

Radical Primary Excision, up to and including bilateral nephrectomy with renal allotransplantation, has been advocated for bilateral Wilms' tumors. Since 1953 we have treated nine children with bilateral Wilms' tumors. Only two of them died of their malignancies; both had primary radical excision and received less than the currently recommended dosaes of radiotherapy and chemotherapy.More recently, we have excised the more involved kidney and treated the less involved side with radiotherapy and multiple courses of chemotherapy. We have termed this conservatively aggressive management; it has resulted in no evidence of residual malignancy in any of the six children so treated. Three of these six patients were left with residual benign rhabdomyomas. These results suggest that this less radical approach to bilateral Wilms' tumors is an effective method of management.     

Liver transplantation in children with biliary atresia and vascular anomalies

Eight of 29 infants and children undergoing orthotopic liver transplantation for extrahepatic biliary atresia had associated major vascular anomalies. A distinctive and highly unusual vascular malformation consisting of absent inferior vena cava, anomalous origin of the hepatic artery, and preduodenal portal vein was encountered in three of these children. Although at times technically difficult, single anomalies of hepatic vasculature were satisfactorily handled. In contrast, transplantation attempts were lethal in all three infants having the complex vascular malformation. The suggestion is made that this specific subgroup of patients with biliary atresia be identified in advance and that, at the moment, children with this composite anomaly are highly questionable candidates for liver transplantation.     

Portal decompression by interposition mesocaval shunt in patients with biliary atresia

Surgical correction of portal hypertension in infants and children has often been frustrated by limitations imposed by the diminutive vessel size. During the past 18 mo, five patients from 1 to 7 yr of age and weighing between 9 and 19 kg, have required portal decompression for bleeding from esophageal varices. In two patients, a previous dissection in the area of the porta hepatis imposed a further technical obstacle. Both of these patients had biliary atresia and one had undergone a successful portoenterostomy (Kasai).¹ Success of the interposition mesocaval graft in adult patients led us to adopt this procedure in our five patients. Autogenous jugular vein was utilized for construction of the shunt in the smaller patients. Radiographic confirmation of shunt patency has been obtained 1 yr postoperatively.     

Cannulation through the left atrial appendage facilitates Mustard's operation for transposition of the great arteries

In a patient with transposition of the great vessels and juxtaposition of the atrial appendages, improved exposure for placement of Mustard's interatrial baffle was gained by inserting the superior vena caval catheter into the juxtaposed right atrial appendage. In 3 subsequent patients with normal atrial appendages the left atrial appendage was used for introduction of this catheter, which was then guided across the atrial septal defect into the superior vena cava. Advantages of this method over conventional cannulation are discussed.     

Surgical treatment of congenital esophageal atresia

Severe gastroesophageal reflux was found in 9 out of 18 infants with congenital esophageal atresia studied radiographically shortly after primary reconstruction. Pulmonary complications were recorded in 18 out of 32 similar patients in long-term follow-up. Strictures at the level of the anastomosis were detectable in 18 out of 32 patients; eleven strictures were severe enough to require dilation or surgical revision. These findings suggest that early evaluation for gastroesophageal reflux may be useful in management of infants with esophageal atresia. The precautions taken preoperatively to prevent complications of gastroesophageal reflux should be continued in the postoperative interval unless a competent lower esophageal sphincter is demonstrated.     

Peptic ulcer in children with gastric tube interposition.

An infrequent, but potentially serious, complication of gastric tube interposition is ulceration within the conduit. It is important, therefore, to recognize ulcer formation in its early stages by serial radiographs throughout the childhood years. While redundancy and partial obstruction with impaired drainage of the tube appear to be etiologic factors, distension of the transposed antrum may lead to hyperacidity and may play a role in ulcerogenesis. A course of dietary and antacid therapy may heal the ulcer, but surgical revision of the tube may prove necessary.     

Life-threatening anoxic spells caused by tracheal compression after repair of esophageal atresia: Correction by surgery

Three infants who developed anoxic spells 2, 5, and 20 mo following repair of esophageal atresia developed apneic spells during or within a few minutes of feeding. These episodes began with stridor and cyanosis; when severe, they progressed to apnea and loss of consciousness. Mouth-to-mouth resuscitation was often necessary. Investigations failed to detect esophageal obstruction and/or a recurrent tracheoesophageal fistula. No neurologic or cardiac abnormalities were found. The cause was compression of a 1- to 3-cm segment of trachea anteriorly by a vascular structure and posteriorly by a dilated esophagus that emptied slowly because of poor motility. Endoscopy confirmed the x-ray findings. The aortic arch and innominate artery were suspended to the sternum anteriorly, which relieved the apneic spells in all patients.     

Colon interposition for the replacement of the esophagus in children.

Thirty-two new cases of colon interposition for esophageal reconstruction are presented. Only one late death occurred in this series, which was unrelated to the colon interposition. Fifteen severe complication were observed in five patients with preexisting esophageal atresia and previously complicated courses following disastrous primary esophageal repairs. The most common complication was pneumonia which occurred 13 times. Five late strictures responded well to dilatation or revision. Long-term weight gain was compared between a group of colon transplant patients and a group of patients with repaired esophageal atresia. Although the esophageal atresia group followed the 25th percentile and the colon interposition group followed the 3rd percentile on the growth curve, no significant difference existed in weight gain over 12 yr.     

Suture fistula as a means of connecting upper and lower segments in esophageal atresia

A technique is described whereby a fistula is created between the upper and lower esophageal segments by means of a silk suture.     

Intraoperative Identification of the Conduction System in Repair of Endocardial Cushion Defect

Electrophysiological identification in-traoperatively of the His-Purkinje system eliminates the complication of complete heart block consequent upon repair of endocardial cushion defect (ECD). Ten patients, 6 with complete ECD and 4 with incomplete ECD, underwent repair of the defect. None of the patients developed heart block. Slight variations were noted in the location of the His bundle in 9 patients, and a major deviation was found in 1 patient with incomplete ECD. The data support the use of intraoperative recordings as a necessary accompaniment to the operative repair of ECD.     

Ongoing cirrhosis after successful porticoenterostomy in infants with biliary atresia.

Thirty-one patients were treated by portoenterostomy procedures for extrahepatic biliary atresia. In 20, there was unequivocal evidence of bile drainage. Eleven of these 20 were restudied from 3 to 23 mo postoperatively by liver biopsy. The clinical condition of most of these infants was satisfactory. Despite absence of clinical jaundice, normal rose bengal scan, and normal liver function studies, eight showed progressive degrees of hepatic fibrosis when compared to the results of the biopsy taken at the time of portoenterostomy. Two patients showed stable histology and one, after progressing on a second biopsy, had normal liver histology on a third study. The implications of progressive hepatic histopathology despite bile drainage are discussed.     

Circular esophagomyotomy of upper pouch in primary repair of long-segment esophageal atresia

A method of performing circular esophagomyotomy of the upper pouch is described as an effective technique for use in repair of esophageal atresia where the ends cannot be brought together.     

Neonatal small left colon syndrome: intramural not intraluminal obstruction.

We have described a characteristic syndrome of intestinal dysfunction in infants of diabetic mothers. This finding appears to result from a transient intramural dysfunction. Many respond to rectal irrigations alone. However, a significant number will require close observation and possible diversion for persistent partial intestinal obstruction. Failure to recognize persistent obstruction may result in intestinal perforation.     

Iatrogenic ileal atresia secondary to clamping of an occult omphalocele.

Clamping of an occult omphalocele has led to complete division of an entrapped loop of ileum in two instances. The proximal end has been sealed in the process, producing an iatrogenic ileal atresia. The clinical picture in both instances differed from that usually found with ileal atresia. Escape of a small amount of meconium from the transected cord was noted in one, and the stump of the cord in the other appeared red and engorged. Clamping the umbilical cord routinely at least 5 cm from the abdominal wall is recommended.     

Anomalous origin of the left coronary artery from the pulmonary artery. Definitive surgical treatment by saphenous vein interposition in a 17-month-old child.

A 17-month-old child underwent successful operative correction of anomalous origin of the left coronary artery from the pulmonary artery by saphenous vein interposition. The clinical, hemodynamic, and angiographic features of the anomaly are presented. Three months after the operation, cardiac catheterization and angiography revealed a patent graft with good antegrade flow and improvement in left ventricular function.     

Vertebral anomalies associated with esophageal atresia and tracheoesophageal fistula with reference to the initial operative mortality

In a series of 40 patients with esophageal atresia and tracheoesophageal fistula, the incidence of musculoskeletal anomalies was 45% (18 patients). An extra mesodermal segment was found in 15 patients (13 paired thoracic vertebrae and 13 paired ribs in nine patients; six lumbar vertebrae in 6 patients). The mortality in grade A infants with an extra mesodermal segment was significantly higher than in those infants with a normal spine (p > 0.01). It is suggested that segmentation or abnormal segmentation of the embryo may be important as a cause of esophageal atresia.