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Of 199 neonates undergoing primary or delayed primary repair of esophageal atresia, 34 (17%) developed anastomotic leakage, 7 of which (3.5%) were major anastomotic disruptions. Infants with major leaks developed signs within 5 days and all required early reoperation, necessitating abandonment of the esophagus in 6. The remaining 27 were minor leaks demonstrated by water-soluble contrast studies and were successfully treated nonoperatively. Gastroesophageal reflux was unassociated with this complication but the use of braided silk sutures was associated with a significantly increased risk of anastomotic leakage when compared with polyglycolic acid (relative risk, 3.2) or polypropylene (relative risk, 2.6) sutures. Following anastomotic leakage there was a significantly increased risk (relative risk, 2.04) of subsequent esophageal stricture formation.

In 10 years, 57 infants with Hirschsprung's disease underwent endorectal pull-through (ERPT). Postoperatively, 3 patients died. Of the 44 survivors with an intact endorectal anastomosis aged more than 3 years, 23 (52%) had an excellent result, and of the 28 children more than 5 years old, 23 (82%) had a satisfactory result. Of the 53 known survivors of all ages, 18% suffered from diarrhea with intermittent incontinence and 5 (9.4%) had undergone a Duhamel procedure within 4 years of ERPT.

In the decade 1981 to 1991, 16 infants developed recurrent necrotizing enterocolitis (NEC). They comprised 12 (6%) of 196 neonates referred for further management of NEC and four others referred with major congenital anomalies. Their median gestational age was 32 weeks (range, 27 to 40), median birth weight was 1,260 g (range, 790 to 3,230), and the sex distribution was equal. Recurrent NEC occurred after a median interval of 37 days (range, 11 to 163) from the onset of the initial episode. All but one of the 16 infants were either premature (n = 10) or mature with major congenital anomalies (n = 5). Nine patients had previously undergone surgery for NEC. Medical treatment was successful in 11 patients with recurrent NEC. Two infants died, both of liver failure related to parenteral nutrition; one of whom had suffered four separate episodes of NEC and was found to have a superior mesenteric artery occlusion. There was no consistent association between recurrent NEC and the type or timing of enteral feeds or the anatomical site or method of management of the original attack. The mortality of 12.5% was similar to that for primary NEC.

Rectal ectasia may be associated with anorectal anomalies. If not recognized at the time of surgical reconstruction it may lead to megarectosigmoid, resulting in severe constipation and overflow incontinence postoperatively. The authors treated four patients presenting with this condition. One patient born with a low anorectal anomaly and two with high anorectal anomalies experienced intractable constipation caused by megarectum despite otherwise adequate primary reconstructive procedures. A fourth patient had rectal stenosis in association with megarectosigmoid. The ectatic megarectum had to be resected in all the patients to achieve normal bowel actions. The authors feel that resection or tailoring of the ectatic segment should be an integral part of the primary reconstructive procedure.

Rectal atresia and stenosis are rare and peculiar anorectal malformations for which many and varied surgical procedures have been described, ranging from simple perforation of the atresia to extensive sacro-abdomino-perineal pull-through operations. The results of these operations have been generally unsatisfactory, chronic constipation being a common postoperative feature. In the authors' experience, the Duhamel pull-through is the operation of choice for this problem.

Seven full-term infants with aganglionosis extending into the small bowel presented with clinical, radiological, and operative features of meconium ileus. Misdiagnosis resulted in inappropriate treatment. The correct diagnosis was eventually established by rectal suction biopsy, mostly after either recurrent intestinal obstruction or stomal dysfunction, and after cystic fibrosis had been excluded. For two patients, the results of rectal suction biopsies were initially misleading. Two infants died. Extensive intestinal aganglionosis should be considered a rare possibility in all infants with meconium ileus. In such cases, histological examination of the appendix may avoid this potential pitfall.

The surgical problems encountered in treating three infants with short-bowel syndrome are reported. The two survivors demonstrate many of the problems associated with the very short-bowel syndrome and both are dependent on parenteral nutrition despite undergoing intestinal lengthening.

Congenital esophageal stenosis caused by tracheobronchial remnants occurred in eight children, six of whom had associated esophageal atresia and/or tracheoesophageal fistula. Symptoms usually began in early infancy but delayed diagnosis was a common feature. The mean lag period between presentation and definitive operation was 4.6 years (range, 1 month to 16 years). Errors in diagnosis were common. Six were initially diagnosed as having inflammatory strictures secondary to reflux esophagitis. Seven children were subjected to repeated esophagoscopy and bouginage of the “stricture” (mean no. = 3.4), with invariable failure to ameliorate dysphagia. Antireflux procedures were performed in three patients. In all children, symptoms were dramatically relieved following resection of the stenotic segment or esophageal replacement. Although a rare entity, congenital esophageal stenosis due to tracheobronchial remnants should be considered a possibility in patients with esophageal stricture, presumed to be inflammatory in nature, which fails to respond to standard therapy.

CHARGE association was diagnosed in 61 infants, 20 of whom died, mainly during the first 2 years of life. Esophageal atresia and/or tracheoesophageal fistula were present in 10 neonates. Axial skeletal anomalies occurred in 7 of the 10, but none had preaxial limb defects typical of the VATER association. All had major cardiac anomalies, predominantly tetralogy of Fallot. The majority of patients had primary repair of the esophagus. The postoperative course was stormy in all patients, with a high incidence of complications and 70% died. The recognition of features of the CHARGE association is important because it has major prognostic and therapeutic implications.

Four neonates with double duodenal atresia/stenosis are described. Preoperative plain radiographs in two patients demonstrated atypical appearances suggestive of complex pathology. Cystic dilatation of the second part of the duodenum was observed at laparotomy in two patients and in one of these the "cyst" was palpable preoperatively, causing diagnostic confusion. All four patients underwent successful surgery but one infant with Down's syndrome subsequently died of congenital heart disease.

During a 15-year period, 12 infants with apple-peel bowel were treated; the first 4 by initial enterostomy with delayed anastomosis and the remaining 8 by primary anastomosis. All four infants in the enterostomy group required parenteral nutrition, compared with only six of the eight who had a primary anastomosis. Seven of the eight infants treated by primary anastomosis were in hospital for less than 50 days, (however, the remaining child was an in-patient for over 2 years, but did suffer from short-bowel syndrome with only 17 cm of small intestine). In comparison, all four of the enterostomy group were hospitalised for between 70 and 175 days. All twelve patients survived.

Twenty-seven patients with sacrococcygeal teratoma were followed for a mean period of 5 years (range, 2 to 12 years). Eleven (41%) had some form of functional impairment. Fecal and/or urinary incontinence was present in nine children and two had weakness of the lower limbs secondary to sciatic nerve palsy. Tumors with large intrapelvic extensions requiring an abdominoperineal approach for resection were associated with a higher incidence of functional sequelae (67%). It is recommended that patients with sacrococcygeal teratoma (particularly those with a presacral extension) be closely monitored for functional deficits so that early treatment can be instituted.

Anastomotic strictures developed in 74 (37.2%) of 199 patients undergoing primary or delayed primary repair of esophageal atresia with or without tracheoesophageal fistula. Significant predisposing factors included the use of braided silk sutures (relative risk 1.72 and 1.49, compared with polyglycolic acid and polypropylene sutures), the presence of gastroesophageal reflux and leakage of the anastomosis (relative risk 2.29 and 2.04, respectively). Tracheomalacia, personnel factors, and recurrent fistula did not affect the rate of stricture formation. Seventy-one patients responded to dilatation alone, whereas three required stricture resection or esophageal substitution. All three patients requiring surgical intervention and 14 requiring five or more dilatations developed symptoms within the first 6 months after esophageal anastomosis. Antireflux surgery was carried out in 19 (25.7%) of the 74 patients.

An analysis of 18 infants with combined esophageal and duodenal atresia is presented. A total of 50 other associated anomalies were identified in the 18 infants. These predominantly involved the genitourinary (11), cardiac (9), anorectal (8), and gastrointestinal (8) systems. There were 6 early survivors (33% survival rate), 1 of whom died later from an unrelated cause. It is suggested that in an otherwise healthy infant the primary approach should be towards the esophageal atresia and tracheoesophageal fistula. A wide gastrostomy is mandatory. Repair of the duodenal obstruction may safely be postponed for a few days. In the absence of an associated tracheoesophageal fistula, a primary duodenoduodenostomy and gastrostomy with a transanastomotic feeding tube is the approach of choice.

One hundred forty-eight infants with abnormalities of the esophagus treated over a 5-year period were reviewed: 87% comprised esophageal atresia with distal tracheoesophageal fistula. The survival rate for infants in risk group A was 100%, for risk group B, 86%, and for risk group C, 73%. There were six infants with associated anomalies incompatible with survival. Anastomotic leaks occurred in 21% of cases, strictures in 18% and recurrent tracheoesophageal fistula in 12%. The use of braided silk for the repair was associated with a high complication rate. There was a distinct advantage in delaying surgery pending improvement in aspiration pneumonia. Low birth weight was not considered a contraindication for primary repair. Emergency ligation of the fistula was considered a more appropriate procedure than gastrostomy for the infant with associated severe respiratory distress syndrome in whom mechanical ventilation was providing difficult. Congenital cardiac anomalies were the single most common cause of mortality and correction of these anomalies should be pursued aggressively. There were definite advantages of preoperative endoscopy, particularly in inidentifying proximal fistulae. Elective postoperative ventilatory support for infants with a very tense anastomosis was successful in preventing leaks in five infants. There were no advantages to routine gastrostomy. Severe gastroesophageal reflux and tracheomalacia demand aggressive management. Mortality was directly related to the severity of associated congenital anormalies.

This is a report of ten patients in whom the esophagus is replaced by the whole stomach brought through the chest. It was used for children with long gap or previously failed esophageal atresia repair. It documents complications (anastomotic leak and stricture) and morbidity, and highlights precautions that must be taken.

In 113 children suspected of having CSF shunt dysfunction, the shunts were examined with a Doppler ultrasound flowmeter on 203 occasions. Shunt patency or blockage was correctly diagnosed in 90% of cases. There were 5% false-positive and 5% false-negative tests. Certain pitfalls of the investigation and its future applications are discussed.

Intrathoracic foregut duplication cysts are rare congenital foregut malformations that usually produce respiratory symptoms in early infancy. Surgical resection of this anomaly is usually followed by permanent cure. Two cases of intrathoracic foregut duplication cyst associated with esophageal atresia are reported. The first patient had an associated tracheoesophageal fistula while the second had an isolated atresia. Both patients survived following resection of the cysts.

During a period of 4 1/2 years, 37 infants with congenital diaphragmatic hernia were treated. The overall survival rate was 68%. Survival depended more on cardiopulmonary function than the size of the diaphragmatic defect. There was little evidence that infants with agenesis of the diaphragm formed a special group with a poor prognosis, and four of the ten patients with unilateral agenesis survived. A Dacron prosthesis is recommended as a substitute for the missing diaphragm.

A newborn boy with a giant tonsillar teratoma or epignathus is described along with details of his management. This is a rare lesion and few survivors have been reported.

During the 7-year period from 1980 to 1986, 56 neonates with gastrointestinal (GI) perforation were treated in the Hospital for Sick Children, London. The overall mortality rate was 30%. The highest mortality rate (60%) was associated with gastric perforation, which in this series occurred only in premature neonates. Colorectal perforation carried the lowest risk of mortality (17%). The mortality rate from small bowel perforation was 35%. Prematurity and low birth weight were significant factors increasing the mortality rate from neonatal intestinal perforation. The predominant cause of perforation in the small and large intestine was necrotizing enterocolitis and the most common site of perforation was the terminal ileum. Mechanical ventilation was thought to be the cause of the perforation in four of the five neonates with gastric perforation. Primary anastomosis is a definite option in neonates who are stable intraoperatively. The mortality rate and in-patient stay for neonates who had primary anastomosis were less than for those who had stomas.

Two unusual intestinal duplications are described. In each case the mesenteric duplication had a separate muscular coat from the normal bowel permitting complete excision of the duplication without interference with the blood supply to the normal intestine.

Two neonates with esophageal atresia and agenesis of the right lung are described. Review of the literature on the subject revealed an additional 11 cases of esophageal atresia associated with severe pulmonary hypoplasia. All of the affected infants died. Half of the deaths were caused by congenital heart disease, and the others were due to prematurity combined with various other severe anomalies and respiratory complications.