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Hirschsprung's disease is the congenital absence of autonomic ganglia (group of nerve cells) in the smooth muscle wall of the colon, resulting in poor or absent peristalsis (intestinal movement). This results in the accumulation of feces and dilation of the bowel. Usually, the first sign of this disease in the newborn is delayed passage of meconium (first stool of the newborn), but Hirschsprung's disease may cause perforation of the bowel. Three cases of newborns with bowel perforation due to Hirschsprung's disease are presented. Age at the time of referral was 27 hours, 30 hours, and 1 week old; in all cases, failure to pass meconium was the reason for referral. These three cases, plus a review of the literature, revealed that 90 percent of perforations occur in the proximal colon, terminal ileum (small intestine), or appendix. Perforation of the bowel may be related to increased pressure within the intestine. Between 5 and 8 percent of neonatal ileocolic (small bowel to colon) perforations are due to Hirschsprung's disease; the majority are due to necrotizing enterocolitis (severe inflammation of the intestines and colon). If there is no intestinal gas present, or evidence of appendiceal or cecal perforation, Hirschsprung's disease should be considered. If the cause of perforation is Hirschsprung's disease, and correct diagnosis is not made, it may be fatal, or at least result in a stoma (diversion of the bowel to the abdominal wall) without proper innervation. (Consumer Summary produced by Reliance Medical Information, Inc.)

The number of in-hospital deaths in children aged 0 to 14 years from acute appendicitis in England and Wales has fallen from an annual average of 36.2 in 1963 through 1967 to 1.8 in 1993 through 1997, and the case-fatality rate from 1.06 to 0.16 per 1,000 discharges, a reduction of 85%. During these 4 decades, the child population has fallen by 8.5%, and the number of children discharged from the hospital with a diagnosis of acute appendicitis by 63%. These data highlight a trend noted in Scotland in the first 50 years of the 20th century. Approximately three quarters of the fall in the number of deaths from acute appendicitis since 1963 is accounted for by a lower incidence of appendicitis in a slightly reduced child population. A quarter of the fall is caused by a marked decline in the hospital case-fatality rate during the last 30 years, likely to be related to improvements in clinical care. Delay in diagnosis is the main contributory factor in the residual small number of deaths each year. Despite the availability of numerous diagnostic aids, clinical acumen remains a fundamental component of expert management of acute appendicitis in children.

Despite advances in prenatal diagnosis and improvements in postnatal medical and surgical management of infants with congenital diaphragmatic hernia, the overall mortality in this condition exceeds 50%. The option of antenatal surgery has evolved in the last two decades with the first successful in-utero repair reported in 1990. Subsequent experience has been disappointing even though major technical obstacles have been overcome. Remaining unsolved issues include the problems of premature labour, case selection and the safe reduction of herniated liver. Newer, less invasive, techniques such as fetal tracheal occlusion are being developed, holding out the opportunity for a major clinical advance in the antenatal treatment of congenital diaphragmatic hernia.

Aim To evaluate long term detailed pancreatic endocrine and exocrine function in children with persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) after 85-95% pancreatectomy. Methods Six children with PHHI between 0.9 and 12.7 years after pancreatic resection underwent clinical and investigative follow up at 1.0 to 14.9 years of age. One child with PHHI who had not had pancreatectomy was also assessed. Standard endocrine assessment, pancreatic magnetic resonance imaging (MRI), and detailed direct and indirect tests of exocrine pancreatic function were performed. Results Pancreozymin-secretin stimulation test results were normal in only one child, borderline in two, and deficient in four, one of whom requires daily pancreatic enzyme supplements. Pancreolauryl tests performed in three children were borderline in two and abnormal in the other. Only one child had low faecal chymotrypsin values. One child developed insulin dependent diabetes at 9 years and two children at 1.0 and 13.3 years require diazoxide to maintain normoglycaemia. MRI showed no major regrowth of the pancreatic remnant after resection (n = 5). Conclusions Clinical evidence of endocrine or exocrine dysfunction has developed in only two patients to date, but detailed pancreatic function testing suggests subclinical deficiency in all but one of our patients with PHHI. Although 95% pancreatectomy results in postoperative control of blood glucose, subclinical pancreatic insufficiency is present on long term follow up and development of diabetes mellitus and exocrine failure remain ongoing risks.

Aims To undertake a prospective, single blind, randomised trial comparing the efficacy and tolerance of two outpatient colonoscopy bowel preparation regimens. Methods Patients aged between 18 months and 16 years being admitted for day case colonoscopy were allocated randomly to receive either Picolax (an oral, sugar free powder containing sodium picosulphate 10 mg/sachet with magnesium citrate) and clear fluids or bisacodyl tablets with an unrestricted diet and a phosphate enema just before colonoscopy. Patient compliance, bowel frequency, and associated symptoms were recorded, and the adequacy of the bowel preparation was assessed in a blinded manner. Results63 of 66 patients completed the trial. Mean age, mean weight, extent of colonoscopy, and distribution of underlying pathology were similar in both groups. Bowel preparation was good or excellent in all of the patients in the Picolax group (n = 32) compared with 22 patients in the bisacodyl/phosphate enema group (n = 31). The latter group experienced more abdominal discomfort during bowel preparation but three of the Picolax group vomited and the lack of solid food distressed some children. ConclusionsAll bowel preparation methods have limitations and unpleasant side effects but the use of Picolax and clear fluids proved superior to bisacodyl tablets and a phosphate enema in children undergoing day case colonoscopy.

Background Femoral hernias are rare in children, accounting for fewer than 1 per cent of all paediatric groin hernias. Misdiagnosis is common and a source of complications. There is no consensus on the age and sex distribution or the optimum method of repair.
Methods A personal experience of four children with femoral hernia is reported together with an institutional review of a further ten hernias encountered during the past 11 years.
Results Peak incidence was between 5 and 10 years of age. Misdiagnosis was common, partly because of the variability in presenting symptoms and signs. In this series, boys were more commonly affected but a literature review indicated a similar sex incidence.
Conclusion A femoral hernia should be positively excluded if the operative findings at inguinal exploration are inconsistent with the preoperative signs and in any child with a suspected recurrent inguinal hernia. Excision of the sac and repair of the femoral canal is curative.

Background Perianal sepsis is a relatively common surgical problem in children and yet the contribution of rare aetiological factors, the frequency of fistula formation complicating perianal abscess, the significance of bacteriological findings and the optimum treatment of fistula in ano are poorly understood.
Methods A consecutive series of 58 children (51 boys, seven girls) with a perianal abscess or fistula attending a tertiary referral centre over a 4-year period was reviewed.
Results Thirty-eight children (34 boys, four girls), ranging in age from 1 month to 12 years, presented with an abscess. After treatment, three developed a further abscess but only four developed a fistula. Fistula formation only occurred in those with enteric bacterial infection. Of 24 children with a perianal fistula five required further surgery for recurrence, four of whom were initially treated by fistulotomy. In most patients, the fistula was simple, low and direct, and treatment by fistulectomy resulted in significantly fewer recurrences. Apart from Crohn's disease, identifiable aetiological factors were rare.
Conclusion Perianal sepsis is most common in otherwise healthy boys under 2 years of age. Abscess is best treated by incision and drainage, and fistulectomy is successful for the small proportion who develop a fistula.

AimsTo assess the long term morbidity and quality of life in survivors of gastroschisis.DesignAll babies born with gastroschisis between 1972 and 1984 and who survived more than one year were identified. Those who could be traced were questioned about their general health, growth, abdominal symptoms, cosmetic concerns, education, employment, and fertility.ResultsOf the 35 patients, two have died, seven could not be traced, and three declined to be interviewed. Twenty three subjects (70% of survivors) with a median age of 16 years (range 12-23 years) responded. Twenty two (96%) were in good health and overall growth was within normal limits. Eight subjects (35%) have had further surgery related to gastroschisis, including two for adhesive small bowel obstruction and three for scar complications. In 13 (57%), absence of an umbilicus caused distress during childhood.ConclusionMost gastroschisis survivors can eventually expect normal growth and good health. Adhesive bowel obstruction is an uncommon, but potentially late, complication. The umbilicus should be conserved during gastroschisis repair.