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A 6-year-old girl presented with intractable constipation. On investigation, she was found to have an association of anorectal stenosis, anterior sacral defect, and anterior meningocoele (Currarino's triad). The meningocoele was excised and a Duhamel pull-through procedure performed after resection of a massively distended rectosigmoid. Post-operatively, she started having spontaneous bowel action. Early diagnosis and management is recommended to avoid the high mortality and morbidity associated with this condition.

A neonate underwent resection of an extensive sacrococcygeal teratoma after receiving a bolus of 40,000 Kallikrein Inhibitory Units of aprotinin at induction of anaesthesia. The measured blood loss was 30 ml, less than 10% of blood volume, and was replaced with an intravenous colloid solution, avoiding blood transfusion. The blood loss was low compared to previous resections of similar sacrococcygeal teratomas (mean 118 ml, range 60–250 ml) and suggests that aprotinin may be beneficial in blood conservation during extensive dissections in infants.

A retrospective audit was performed of renal ultrasonography in 73 of 79 consecutive patients treated for oesophageal atresia during an 81/2-year period at one neonatal surgical unit. Anorectal atresia was present in 13 (16.5%) infants, of whom 9 (69%) had abnormal ultrasound scans and 2 (15%) required operative intervention. Of the 60 infants without associated anorectal anomalies, 11 (18%) had abnormal ultrasound scans but only 2 (3%) received active treatment. In the absence of associated anorectal atresia, less than 5% of patients with oesophageal atresia will benefit from routine renal ultrasonography.

During the 5 years 1985–1989, 24 (32%) of 76 patients treated for Hirschsprung's disease (HD) developed enterocolitis, this being present at the time of diagnosis in 10 (13%) infants, 7 of whom were neonates. HD presented as necrotizing enterocolitis in 5 neonates, 4 of whom were premature. The enterocolitis developed postoperatively in 14 (18%) patients, in 7 after an enterostomy and in 7 after a pull-through procedure. Recurrent episodes of enterocolitis occurred in 4 of the patients who developed postoperative enterocolitis. The risk of enterocolitis was increased in girls, in patients with associated Down's syndrome, those with a family history of HD, and those managed by an endorectal pull-through procedure. The Duhamel procedure was associated with a low (5%) incidence of postoperative enterocolitis. Long-segment aganglionosis was not associated with an increased risk of enterocolitis and preoperative enterocolitis conferred no increased risk of postoperative enterocolitis.

Of 149 infants with esophageal atresia treated during a 13-year period, 5 (3%) developed postoperative necrotizing enterocolitis (NEC). Four of the 5 were premature with birth weights below 1.8 kg (Waterston group C), and all were from a group of 17 awaiting delayed primary esophageal anastomosis. In each case, NEC occurred suddenly and death ensued within 5 days. NEC should be included in the list of complications resulting from, or associated with, delayed primary repair of esophageal atresia.

In surgical neonates receiving total parenteral nutrition a prospective study was undertaken to assess the performance of fine-bore central venous Silastic catheters inserted percutaneously via a peripheral vein. During a 1-year period a total of 33 catheters was inserted into 28 neonates. The mean duration of catheter survival was 14.9∓8.0 (range 4–34) days. No complications occurred with 22/33 (66%) catheters which were removed when redundant. Although there were no long-term sequelae, 11 (33%) catheters was removed because of complications, the commonest being sepsis, which occurred in 5 (15%) patients. No septic complications were detected in the 13 patients having a catheter for 10 days or less. Catheters positioned in the great veins peripheral to the superior vena cava and right atrium proved to be safe and reliable in the short term.

An audit of 143 consecutive neonatal surgical operations performed in one neonatal surgical unit is reported. Wound infections developed in 13 patients (9.1%) and bacteraemia in 9 (6.3%). Three groups at increased risk were identified: small-for-dates infants subjected to major surgery, infants subjected to invasive monitoring, and infants over 1 week of age with Staphylococcus aureus as a skin commensal. Infections were significantly less frequent in potentially contaminated wounds if antibiotic prophylaxis had been used (7.6% compared to 26.7%: P

Children with total colonic aganglionosis pass through three critical phases, the first extending from birth to the establishment of the correct diagnosis with construction of a ganglionic stoma. The second phase lasts from the raising of the stoma to its closure, including the definitive surgical procedure. The third phase begins with closure of the stoma and ends in a gradual decrease in the frequency of defaecation together with the acquisition of continence. The three phases have been analysed in two groups of patients; group I presented between 1978 and 1982 (n = 23) and group II between 1983 and 1987 (n = 17). In the first phase, 9 children in each group underwent inappropriate surgery. The mortality in the second phase was 26% in group I and 6% in group II and in the third phase 6% and 0% respectively. Lack of awareness of the condition and a reluctance to transfer neonates with intestinal obstruction to specialised centres led to delay in diagnosis and inappropriate treatment in both groups. In the second phase, improvements in results were due to increased use of parenteral nutrition, careful monitoring and replacement of stomal electrolyte losses, and improved management of enterocolitis. During the third phase, nocturnal incontinence remained a problem.

We report two boys with oesophageal atresia and the CHARGE association, both of whom eventually died from the effects of their cardiac anomalies. It is important to distinguish the CHARGE association from the VATER association because the former collection of anomalies conveys a much gloomier prognosis than the latter.

Between 1978 and 1986, 55 neonates were treated for small-bowel atresia, stenosis, or segmental dilatation with an overall survival rate of 89%. Before 1981, 21 patients were treated with a mortality rate of 14%, whereas the mortality rate for the 34 treated after 1981 was 9%. Whenever possible, an end-to-end single layer anastomosis was performed with a mortality rate of 6%. In very sick patients with complicated lesions, defunctioning stomas were the safest form of treatment, but patients so treated had a mortality rate of 12.5%. The main causes of death were other severe congenital anomalies.

Over a 6.5-year period, 25 infants and children with symptomatic tracheomalacia underwent aortopexy. Seventeen of the patients had immediate and dramatic relief of symptoms and 5 others were greatly improved. The operation failed in 1 patient who required two subsequent procedures. Early recourse to aortopexy is recommended when symptoms of tracheomalacia become evident.