Suction rectal biopsy (SRB) may not include sufficient submucosa for histological diagnosis of Hirschsprung’s disease (HD) and a repeat biopsy is required. Substitution of the conventional syringe for sustained suction from a machine or wall suction unit could provide a more consistently adequate tissue biopsy. This study was conducted to compare the adequacy of SRB specimens using a conventional method of syringe suction (SSRB) with those taken by wall or machine suction (WSRB). Hospital records of patients who had a SRB between 2002 and 2004 were studied retrospectively. The biopsy was considered inadequate if there was insufficient submucosa and a repeat specimen was requested. SSRB were taken using the conventional SSRB technique. WSRB were taken with the same Noblett forceps but with suction from wall suction or from a portable suction machine. Ninety-five infants had 103 SRB, 24 had WSRB and all (100%) were adequate for histopathological diagnosis or exclusion of HD. Seventy nine conventional SSRB were undertaken in 71 infants of which 64 (81.02%) were adequate. The difference in adequacy between WSRB and SSRB was statistically significant ( P = 0.02). There were no instances of haemorrhage or bowel perforation in either subgroup. WSRB with the Noblett biopsy forceps is more likely to produce an adequate specimen and to reduce the incidence of repeat biopsies.

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.

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.

Bladder pseudosarcoma or pseudosarcomatous myofibroblastic proliferation (PMP) is a rare but nevertheless very important differential diagnosis of any bladder mass. In childhood most such masses are usually malignant soft-tissue sarcomas, and distinction from these is therapeutically crucial. To date no aetiological factor for PMP has been identified in children, and treatment is limited to surgical intervention with no role for chemotherapy or radiotherapy. We report clinical, anatomical, and histological findings in a 3-year-old girl with a pseudosarcoma of the urinary bladder associated with a residual urachus, suggesting this as the originating tissue.

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.

EDITOR—The Children's Liver Disease Foundation recently published its report into the treatment of infants with biliary atresia by Kasai portoenterostomy.1This was covered in the BBC's news programme Northwest Tonight and in Trust Me, I'm a Doctor on 26 February. The report advocates that Kasai portoenterostomy should be performed only in centres that can produce outcome figures equivalent to those produced by group C in the table. Furthermore, the …

Adenocarcinoma of the appendix is unsual at any age but occurs mostly in an elderly population. The authors report a unique case presenting in a 10-year-old child and emphasize the importance of subjecting all resected specimens to histological examination.

In a 41-year period, 18 children with a diagnosis of an adrenal cortical tumor were identified (14 carcinoma: 4 adenoma). The majority of patients had clinical signs of endocrine dysfunction at presentation, with virilization (11 patients) and a cushingoid appearance (8 patients) the commonest findings. Abnormal biochemical activity was identified in 16 tumors (94%). The primary treatment in 17 patients was surgical. In addition, 12 children, all with carcinomas, had radiotherapy. Of those children with a carcinoma, 12 are dead, with a median survival of 52 months (range 1-317 months). The three second primary tumors all developed at sites within the field of previous radiotherapy; and proved fatal at 127, 176, and 317 months (median 207 months). This series confirms the poor prognosis in adrenocortical carcinoma in childhood, but a complete resection is compatible with cure of the primary disease. The frequency of second, fatal, primary tumors is of particular concern and long-term follow-up is mandatory in survivors, especially if radiotherapy was part of the treatment protocol.

Management of unilateral multicystic dysplastic kidneys (MCDK) presents physicians and surgeons with a significant dilemma. Recent studies have indicated that the incidence of short term complications of MCDK is low and many authors have recommended conservative non-operative treatment. Surgery has been proposed by some because of the potential complications of hypertension, infection, and malignant change. Three children with hypertension secondary to MCDK seen at this institution in the past four years, one of whom had been discharged from follow up as a result of 'disappearance' of the cystic kidney on ultrasound examination, are reported. We believe that the risks of hypertension secondary to MCDK have been understated, and that based on the conclusions of these studies, many children may be receiving suboptimal follow up. We currently favour elective nephrectomy as the treatment of choice for this lesion.

This report describes a 1-day-old term boy with sigmoid volvulus secondary to Hirschsprung's disease. The presentation and treatment are outlined.

This report describes three children, age range 7 weeks to 5 months, who presented with obstructive jaundice secondary to gallstones. Previous Escherichia coli septicaemia and frusemide therapy were predisposing risk factors in two of the patients. All three were successfully treated with cholecystectomy and exploration of the common bile duct.

This report describes an unusual variety of tracheo-oesophageal fistula which had the radiological appearance of an isolated oesophageal atresia.

The authors report a case of massive upper gastrointestinal bleeding in a child, originating from a Dieulafoy lesion. The intermittent nature of the hemorrhage may lead to considerable diagnostic difficulty.

A premature 740-g infant who had esophageal atresia and tracheoesophageal fistula was treated with a primary anastomosis. The postoperative recovery was excellent, but the need for close cooperation with neonatal intensivists is essential for survival.

A 7-week-old boy born at 32 weeks' gestation developed massive upper gastrointestinal bleeding from a duodenal ulcer. The base of the ulcer had eroded into the hepatic artery. Haemorrhage was only controlled following operation.

Introduction: Traditionally Hirschsprung's disease has been treated by a three-stage procedure. This approach has been associated with stoma problems, three episodes of hospitalization, and a theoretical disadvantage, because of the delayed passage of feces via the rectum, in controlling defecation. The aim of this study was to assess the results of one-stage neonatal pull-through for the treatment of Hirschsprung's disease. Methods: This was a 10-year retrospective review of all patients treated with a neonatal pull through for Hirschsprung's disease. information was obtained from the patients' records. All descriptive data are expressed as mean +/- standard deviation. Results: Fifty-one patients were treated in the 10-year period studied. The mean gestational age was 39.6 +/- 1.7 weeks, weight was 3.3 +/- 0.54 kg, with 76% being boys. The surgery was performed at 10.3 +/- 5.8 days of age. A Swenson procedure was carried out in 38 patients and a Soave in 13. The extent of the disease Was rectosigmoid in 39, and the remaining were sigmoid, descending colon, and total colonic. The mean length of the surgery was 181 +/- 40 minutes. Blood transfusions were required in 13 patients with a mean volume of 55 mL given. The complications observed were: an anastomotic leak in two cases, requiring laparotomy and colostomy (both in patients with Down's syndrome), and wound infection in three cases. There were two delayed deaths both associated with other congenital anomalies. In total, three patients required further surgery; two had colostomies, one requiring a redd pull-through and one had an ileostomy for enterocolitis. The total length of hospital stay was 20.4 +/- 11.2 days. Mean follow-up was 3.73 +/- 2.9 years. In 21 patients the follow-up was greater than 4 years End in this group none were totally incontinent. Four patients complained of soiling at least once a week; and 17 had normal bowel control. Conclusion: Hirschsprung's disease can be successfully treated in the neonatal period with a one-stage pull-through. The short- and long-term results are as good as those with the three-stage procedure, with the child usually benefitting by not having a stoma and a shorter hospital stay. Copyright (C) 1997 by W.B. Saunders Company.

The short-term outcome of surgery for congenital duodenal stenosis or atresia has been evaluated by studying the case-notes of 37 patients treated between 1988 and 1993. Eighteen underwent 'tapered' duodeno-duodenostomy; biliary complications occurred in 3 and 1 developed an adhesive intestinal obstruction. Transient post-operative vomiting occurred in 1 other patient. Nineteen patients underwent non-tapered duodeno-duodenostomy. One die 19 days post-operatively; 1 developed an intra-peritoneal abscess and another an adhesive intestinal obstruction. Transient post-operative vomiting occurred in 3 other patients. Full intra-gastric feeding was achieved between a mean of 13 and 14 days in both groups. There was no advantage to either technique with regard to the time of establishment of full intra-gastric feeding. Although there were surgical complications in both groups, it appears that tapered duodenoplasty carries a considerable risk of incidental biliary injury.

Preservation of the umbilical cord attachment (UCA) in gastroschisis (GS) is still not routine practice. In a prospective series of 36 children with GS, it was always possible to preserve the UCA, even in those undergoing a temporary silo and delayed closure. Reconstruction by 'umbilical cord capping' left no additional scar and achieved a normal abdominal wall. Mild cellulitis in 3 infants resolved on antibiotics, and an initial umbilical weakness in 7 did not require additional surgery. We conclude that preservation of the UCA should be an integral part of surgical technique for all infants with GS. Reconstruction by 'umbilical cord capping' alone achieves an unscarred abdominal wall with an umbilicus of normal shape and position.

Objective To assess the efficacy of the Whitaker diathermy hook in the treatment of posterior urethral valves (PUV). Patients and methods Seventeen patients with PUV underwent primary treatment under general anaesthesia using a diathermy hook. The results were assessed by the clinical course of the patient, serial measurements of serum creatinine level and repeat cystography 6 weeks and 6 months after treatment. Results There were no complications in 12 patients and they required no further treatment of the valves. Three patients required a repeat procedure which resolved the urethral obstruction. Two patients continued to show incomplete disruption of the valves and required endoscopic treatment. Two patients developed ureteric obstruction after disruption of the valves, which was treated by supravesical diversion and later reversed with no further treatment of the valves. Conclusions The Whitaker diathermy hook is a safe and effective instrument which has not gained its rightful place in the treatment of patients with PUV.