Nitric oxide synthase is absent in only a subset of cases of pyloric stenosis.

PURPOSE: The aim of this study was to study nitric oxide synthase (NOS) immunohistochemistry in the pyloric muscle and establish the role of nitric oxide in pyloric stenosis. METHODS: Pyloric muscle biopsy specimens were obtained from 20 patients with pyloric stenosis during pyloromyotomy. Ten control specimens without pyloric disease were obtained from autopsy performed less than 4 hours after death on age-matched babies who died of other causes. Tissues were fixed in 4% paraformaldehyde immediately. A monoclonal antibody against the neuronal form of NOS (bNOS) was used for immunohistochemistry. RESULTS: Immunohistochemistry showed activity of bNOS in the control specimens and some pyloric stenosis specimens. This shows that NOS is present in the pylorus in normal cases as well as in a few cases of pyloric stenosis. CONCLUSIONS: NOS deficiency leading to lack of locally available nitric oxide causes a failure of smooth muscle relaxation. This may account for the cause of pyloric stenosis in infants. However, this study shows that this is true probably only in a subset of cases. The etiology of pyloric stenosis may still be multifactorial. Further investigations are required regarding the etiology of pyloric stenosis. J Pediatr Surg 36:616-619.

The influence of delay in closure of the abdominal wall on outcome in gastroschisis.

To evaluate the effect of a delay in closure of the abdominal wall (AWC) on outcome in the management of gastroschisis, a retrospective analysis of 91 babies admitted over a 7-year period (1992-1998) to a single neonatal surgical unit with a diagnosis of gastroschisis was carried out. Antenatal diagnosis was made in 89 (98%) cases. Surgical intervention occurred in 90 babies at a median of 4 h (standard error 0.345, range 0.5-17) post-delivery. In 72 (80%) cases primary closure of the abdominal defect was achieved, with a silo fashioned in the remaining 18 (20%). One infant died prior to AWC. The median time to full oral feeding was 22 days (2.96, 5-160), and to discharge 28 days (4.03, 11-183). There was no correlation between time to AWC and any measured outcome parameter. There was no significant difference in mortality in those patients having closure before 6 h. Thus, no correlation between time to AWC and outcome was demonstrated. This would suggest that the time taken to optimally resuscitate a newborn infant prior to surgical closure does not have an adverse influence upon outcome and is to be recommended.

The contemporary outcome of gastroschisis.

BACKGROUND: The aim of this study was to evaluate the contemporary outcome in the management of gastroschisis. METHODS: A retrospective analysis was conducted of 91 babies admitted over a 7-year period to a single neonatal surgical unit with a diagnosis of gastroschisis. RESULTS: An antenatal diagnosis was made in 89 (98%) cases. Surgical intervention occurred in 90 babies, at a mean of 5 hours (range, 0.5 to 17) postdelivery. In 72 (80%) cases, primary closure of the abdominal defect was achieved, with a silo fashioned in the remaining 18 (20%). One child died before abdominal closure. The median time to full oral feeding was 30 days (range, 5 to 160 days), and to discharge was 42 days (range, 11 to 183 days). Those children who required a silo, took longer to feed (P =.008) and stayed longer in the hospital (P =.021). The 8 (8.8%) children with an intestinal atresia, required significantly more operative procedures (P =.0001) and took significantly longer to achieve full oral feeding (P =.04), but the presence of an atresia was not an independent risk factor for mortality. There were 7 deaths (7.7%), 3 within the first 7 days. Of the deaths, 5 (71%) were caused by overwhelming sepsis. CONCLUSIONS: The contemporary mortality rate from gastroschisis is less than 8%, and minimizing septic complications would contribute significantly to reducing this. Strategies designed to improve morbidity must focus on optimizing management of those factors associated with a prolonged recovery, namely intestinal atresia, prematurity, and the use of a silo.

Initial response to portoenterostomy determines long-term outcome in patients with biliary atresia.

PURPOSE: The aim of this study was to assess the efficacy of portoenterostomy in biliary atresia and determine factors that predict outcomes. METHODS: The outcomes after portoenterostomy for biliary atresia from 1976 to 1996 were graded into 3 defined groups: G (good, jaundice free); I (intermediate, progressive liver disease with liver transplant if needed after 3 years of age); P (poor, liver transplant or death at less than 3 years of age). RESULTS: Twenty-seven of 49 children (55%) who underwent portoenterostomy responded. Of these, 13 (26%) were jaundice free (G). Of 14 children (28%) in I, 6 underwent liver transplant after 3 years of age. Of 22 children (44.8%) in P, 14 (28%) died before 3 years and 8 underwent liver transplant before 3 years. Thus, 42% of the children in this series are surviving with native liver. Age at operation in P was significantly different compared with G. Preoperative values of bilirubin and alanine transaminase were comparable in the 3 groups. Postoperative values of bilirubin and alanine transaminase were lower in G and I compared with P Complications were significantly lower in group G. CONCLUSIONS: Portoenterostomy alone in biliary atresia is beneficial in 40% of cases. Factors associated with the outcome include age at operation, postoperative cholangitis, and change in values of bilirubin and alanine transaminase. A classification based on decline of bilirubin and enzyme levels 3 months after portoenterostomy is proposed to predict the long-term outcome of an individual case of biliary atresia. It is particularly relevant in the intermediate group in which timing of referral may preclude or compromise liver transplant.

Advanced colonic carcinoma in children.

We report two cases of colorectal carcinoma in children with a delay in diagnosis due to the low index of suspicion of this pathology as the cause of presenting symptoms. We conclude that any child presenting with unexplained rectal bleeding and abdominal pain should be investigated with endoscopy and barium enema.

Hirschsprung's disease and mimicking conditions.

The pathophysiology and aetiology of Hirschsprung's disease are still uncertain. The presentation varies with age. Various methods are used to make the diagnosis. Differing views of management are held both for emergency and definitive surgery, e.g. emergency colostomy or not, a covering colostomy or not for the definitive surgery, the type of operation used. The problems of diagnosis and treating diseases which mimic Hirschsprung's disease are highlighted.

Colon interposition for wide gap oesophageal atresia.

A retrospective study covering December 1978 to December 1989 at Booth Hall Children's Hospital, Manchester, revealed that 12 colon interposition were performed in 11 children to reconstruct their oesophagus. All were for wide-gap oesophageal atresia with or without fistula. One patient underwent colon interposition twice due to ischaemia of the initial transplant. Waterston's transthoracic procedure was used mostly with a modification by Freeman performed in one patient. Main early postoperative complications included proximal anastomosis leak (45.5%) and respiratory infection (36.4%). Late complications were proximal anastomotic stricture (22.2%); gastrointestinal problems (66.7%); and recurrent respiratory infection (11.1%). Although colon redundancy radiologically was noted in two patients, they were well with no dysphagia. Mortality was 18.2%, the two patients dying suddenly two weeks after colon transplant from pulmonary complications. At follow-up (mean: 6.2 years) growth rate had improved slightly, and 11.1% weighed above the 10th percentile. However, 77.8% could swallow satisfactorily within one year of surgery.

Women in surgery in Scotland. A Working Party of the Royal College of Surgeons of Edinburgh.

Despite the fact that 50% of medical undergraduates are female, women comprise fewer than 1% of consultant general surgeons. The possible reasons for this were addressed by a working party of the Royal College of Surgeons of Edinburgh, which now reports the results of a survey of 35 women surgeons of SHO 3 grade and above working in Scotland. Findings were compared with those of 12 anaesthetists and 10 house officers. Surgeons had had an accurate perception of the work patterns their job would entail but no preparation for the lifestyle implications. The experience of gender discrimination was similar in all three groups but perceived discrimination was much more prevalent among surgeons/ophthalmologists. In contrast to the other groups surveyed, the surgical cohort was not deterred by training length, but rather discouraged by the lack of responsibility offered. Other factors which seem to contribute to the underrepresentation of women among surgeons may be the lack (1) of time for child rearing and (2) of same-sex role models.

Wound infection in pediatric surgery: a study in 1,094 neonates.

In an analysis of 1,433 wounds created in 1,094 neonates admitted to a regional neonatal surgical unit during the period April 1975 to December 1987, the mean incidence of infection was 16.6%. During this time there was an increase in the incidence of infection from 12.5% in the first 6 years to 18.8% in the last 7 years (P < .01). Contaminated wounds had an infection rate of 20.7%, whereas the rate in clean wounds was 11.1% (P < .001). Gestational age and birth weight had no influence on the incidence of wound infection. Increasing wound length (P < .001), increasing duration of operation (P < .001), and contamination at operation (P < .001) were all associated with a higher incidence of infection. Staphylococcal species were the most frequently isolated organisms from all categories of infected wounds (clean, potential, and actual contamination).

Hypogastrinemia and esophageal atresia.

To investigate extraesophageal anomalies in infants with esophageal atresia, preoperative plasma gastrin was measured in 12 infants. The median plasma gastrin was 32 ng/L (interquartile range, 24 to 44). There was significant correlation with birth weight (rs = .73, P less than .05) and gestational age (rs = .74, P less than .05). Within this group 9 infants of greater than 36 weeks' gestation were matched to a group of 20 control infants without esophageal atresia. Infants with esophageal atresia had a significantly lower median plasma gastrin (38 ng/L v 55 ng/L, P less than .05). This may indicate preexisting vagal abnormalities in esophageal atresia unrelated to surgical intervention.

Achalasia of the cardia in childhood and the syndrome of achalasia alacrima and ACTH insensitivity.

Achalasia of the cardia is an uncommon condition with an incidence of 0.1 cases per year per 100,000 population under 14 years. A review of experience at Booth Hall Children's Hospital over the past 10 years has revealed six cases of achalasia in children. This includes two cases of the rare syndrome of achalasia, alacrima and ACTH insensitivity (also known as Triple A syndrome) and its variants. There are 23 previously reported cases of Triple A syndrome and a further eight cases of its variants reported in the world literature. One-third of these cases (10 out of 31) have associated neurological abnormalities. The clinical and radiological features of achalasia are reviewed. The importance of barium studies in making the diagnosis is emphasized. However, early cases may show only spasm or incoordination, and manometry is required to confirm the diagnosis in these equivocal cases. In this series only three of the six barium studies were diagnostic and the remainder were diagnosed by manometry; all cases were subsequently confirmed by histology. Delay in diagnosis may result in severe lung damage due to repeated aspiration; this occurred in one of the six cases and was the cause of the child's death.

Mesenteric fibromatosis presenting with ascites in childhood.

A 7-year-old boy presented with acute ascites due to mesenteric fibromatosis. The pathophysiological mechanisms for formation of ascitic fluid and some aspects of the aetiology of intra-abdominal fibromatosis and its association with Gardener's syndrome are discussed.

Colonic atresia: current results of treatment.

Eleven cases of colonic atresia presenting in a 13-year period are described. The incidence of this condition is estimated at 1 per 66,000 live births in the north-west of England. A higher incidence of involvement of the transverse colon has been noted in comparison with previously reported cases. Survival was unaffected by the type of surgical procedure, with both primary anastomosis after resection or staged reconstruction after initial colostomy giving excellent results. No deaths occurred in the ten patients subjected to surgical treatment.

Use of laparoscopy in children with impalpable testes.

Our experience in Manchester using laparoscopy in children with impalpable testes is detailed. Eighty-seven testes were investigated in 77 patients. Of these, 36% had no testes, although in 28% evidence of a vas was found. In those testes which were found, surgery was performed, either orchidopexy or, if positioned high, microsurgery. Orchidectomy was rarely carried out (5 testes only).