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Background: Whether psychomotor retarded persons should be treated by percutaneous endoscopic gastrostomy (PEG) or by surgical gastrostomy combined with an antireflux procedure is controversial. Therefore, the authors investigated the feasibility of a PEG and enteral feeding in these patients., Methods: Patients referred from specialized institutions for a PEG placement were assessed extensively by a multidisciplinary team. When considered eligible, age and general condition determined the choice of treatment under general anesthesia (group 1) or conscious sedation (group 2). Patients were followed up after 1 and 7 days, 4 and 12 weeks, and thereafter every 6 to 12 weeks. Data were collected prospectively over a period of 5 years until gastrostomy removal, death, or arrival at the censory date, 6 months after PEG placement. The endpoints were 1) to evaluate the procedure and its complications; 2) to discover barriers that impeded adequate nutrition; and 3) to explore the appropriateness of the choice of PEG or percutaneous endoscopic gastrojejunostomy (PEJ)., Results: The procedure was successful in 95% of patients, in every patient in group 1 (35/35; median age, 4.1 years) and in 20 of 23 patients (87%) in group 2 (median age, 22.0 years). There were no procedure-related deaths and no 30-day mortality. Major complications changed from procedure-related problems in the short term (5.4%) to tube-related problems in the long-term (24.1%). Nausea and vomiting interfered with adequate feeding mainly in young children, but dietary adjustments alleviated the symptoms and ensured an adequate intake. The choice of a PEG was incorrect in four patients: surgery was needed twice and two PEGs had to be converted into a PEJ. All seven primarily indicated PEJs seemed justified and of temporary need in five., Conclusions: In severely disabled patients, a PEG and adequate enteral nutrition is feasible in the setting of a multidisciplinary approach and protocol-wise follow-up. Yet, anatomic deformities and restricted pulmonary function rendered the procedure more difficult. In cases with obvious aspiration or gastroesophageal reflux, a PEJ combined with acid suppression and prokinetic drugs may be tried before surgery.

Experience of the International Society of Paediatric Oncology (SIOP) Trials and Studies indicates that the preoperative chemotherapy in Wilms' tumour improves stage distribution, decreases complication rate and reduces postoperative treatment. However, some situations may lead to prompt primary surgery. The aim of the study is to assess reasons leading to primary emergency nephrectomy. Records of 720 patients with non-metastatic unilateral nephroblastoma who were registered in the SIOP Trial and Study 9 were reviewed. Twenty-four (3%) cases of primary emergency nephrectomy were identified. Reasons leading to emergency nephrectomy were massive bleedings from ruptured tumours in 13 patients, suspicion of an "acute abdomen" in 7, bowel occlusion in 2 and other in 2. Postoperative treatment included radiotherapy in 71% of cases and anthracyclines in 92%. Complications were frequent and happened in 25% of patients, the outcome however, was favourable and 22 of 24 patients are alive (from 9 to 79 months). The 7 patients with a suspicion of an "acute abdomen" probably constitute the group which could have been markedly reduced if adequately diagnosed and observed prior to surgery.

The aim of the study was to assess rates and types of nephrectomy-related complications in children nephrectomized for nephroblastoma after preoperative chemotherapy. Records of 598 Wilms' tumour patients registered in the International Society of Paediatric Oncology Trial & Study No. 9 (SIOP-9), and pretreated correctly according to the protocol with vincristine + actinomycin D +/- epirubicine or adriamycin prior to nephrectomy, were retrospectively reviewed. Forty-nine patients (8%), who suffered from 54 complications, were identified. Most frequent events were small-bowel occlusions (3.7%) and tumour ruptures (2.8%). Other complications were registered in 2.0% of cases. The low rate of nephrectomy complications and no deaths related to registered ones, are another argument for preoperative chemotherapy in Wilms' tumour patients.

Purpose: After nephrectomy for unilateral Wilms' tumor (WT), two potential hazards threaten the remaining kidney. The development of a metachronous WT (2% to 3%), and decrease in renal function after chemotherapy. Our study aims included: (1) to clarify how many WT patients could benefit from partial nephrectomy (PN) without jeopardizing the high cure rate, (2) to establish the function of the kidney remnant, and (3) to test the radiological criteria for preoperative selection of PN candidates., Methods: A retrospective analysis of 90 consecutive cases of histologically verified WT (1982 to 1992) was carried out. Data were studied independently by the surgeon, the radiologist, and the pathologist. The preoperative selection criteria for PN were functioning kidney, tumor confined to upper or lower pole leaving two thirds of the kidney tumor free, and no hilar or vascular structures involved. After PN, renal function was estimated by a calculated creatinine clearance (CC) combined with the assessment of the relative function of each kidney by renal scintigraphy., Results: There were 46 boys and 44 girls with median age at diagnosis of 3 years 6 months (range, 3 months to 16 years 7 months). Stage I disease occurred in 40 patients, stage II in 23, stage III in 16, and stage IV occurred in 11 patients. Nearly all patients received preoperative chemotherapy. Radiological analysis of 85 available data sets (5 missing) suggested that PN was possible in 13 patients, and data were inconclusive in two. All 13 patients selected by the radiologist appeared to be in agreement with the pathological criteria for resectability. Of these cases, complete resection by PN was performed in five, could have been possible in retrospect in two, and appeared impossible in six. In two more patients, PN was performed; in both cases the radiologist had predicted PN to be impossible. Median follow-up after PN was 61 months, range, 45 to 167 months. Two patients died of distant metastasis, and no local recurrence occurred. Five are in remission with a median CC level of 110 mL/min, range, 85 to 124. Relative functions of the kidney remnants were 20% to 50%. Contralateral nephrectomy for progressive obstruction was performed in one, but CC level remained normal (85 mL/min). Comparing the preoperative radiological prediction with the combined surgical and pathological findings in a 2 x 2 frequency table, the sensitivity of the radiological findings could be calculated to be 80%, the specificity 97%, and the accuracy 87%., Conclusions: (1) Partial nephrectomy is safe in approximately 10% of Wilms' tumor patients. (2) The function of the kidney remnant remains well, and even permits contralateral nephrectomy. (3) Preoperative imaging shows an accuracy of 87% to predict the possibility for partial nephrectomy.

Treatment of children with severe impairment of bladder function requires a large-volume, low-pressure reservoir combined with a continent, easily catheterizable valve. The Mitrofanoff principle (MP) appears to meet these requirements. Between 1986 and 1993, the MP was applied in 15 children (4 girls) aged 4 to 14 years. The primary diagnosis was bladder exstrophy in 8 (2 girls), neuropathic bladder in 3 (2 girls), urethral valves in 2, and rhabdomyosarcoma (RMS) in 2. In 10 patients bladder augmentation with an intestinal patch was performed in addition to a Mitrofanoff procedure; in 5 a neobladder and continent appendicostomy were made. One boy with RMS died of distant metastases with a well-functioning appendicostomy and adequate renal function. At 2 to 9 years follow-up of the other 14 patients, 12 have a good result defined as: (1) adequate reservoir capacity; (2) continence; (3) normal renal function; and (4) no hydronephrosis. In 1 exstrophy patient with pre-existing impairment of renal function, further deterioration necessitated frequent catheterization and additional medical treatment. In 1 boy with fulgurated urethral valves, spontaneous micturition became subsequently possible, allowing closure of his appendicovesicostomy. Complications occurred in 10 patients, necessitating reintervention in 7. The MP in combination with the creation of an adequate reservoir gives good results in children with severe impairment of bladder function. Careful attention should be given to patient education regarding emptying of the reservoir. Long-term follow-up of renal function is mandatory.

The favourable results from the treatment of Wilms' tumour are an example of the success of multimodal therapy in paediatric oncology. The epidemiology, methods of diagnosis, benefits of pre-operative chemotherapy, basic principles of surgery and post-operative treatment modalities are presented. The approach to the management of Wilms' tumour considered in this paper is mainly that of the International Society of Paediatric Oncology.

Three children treated for appendicitis developed anuria and acute renal insufficiency several days after appendicectomy. Associated hydronephrosis or hydroureters were present in two. At cystoscopy, marked swelling of the trigonum and ureteric orifices was seen. One patient developed unilateral acute tubular necrosis, a complication not reported before. Recognition of this rare complication of acute appendicitis, which need not be accompanied by hydronephrosis, should lead to prompt decompression by the introduction of ureteric stents.

A retrospective analysis of 21 patients with jejunal atresia and 24 with ileal atresia showed more differences than similarities between the two categories. The incidence of jejunal atresia appeared to be increasing during the period of observation (1978 to 1987). In the past 2 years, many children with jejunal atresia, who belonged to a set of heterozygotic twins, were admitted to this institution. The mean birth weight and gestational age in jejunal atresia were significantly lower than in ileal atresia. The majority of jejunal atresias were multiple, whereas most ileal atresias were single. Antenatal perforation occurred rather frequently (10 cases) in ileal atresia but only twice in jejunal atresia. The postoperative course was more often prolonged in jejunal than in ileal atresia. Mortality was higher in jejunal atresia: three deaths, all in apple peel atresia, versus one death in ileal atresia. An explanation for some of these observations can be found in a difference of compliance of the bowel wall between jejunum and ileum: the more compliant jejunal wall allows for massive dilatation with subsequent loss of peristaltic activity. It is postulated that because of the many differences, jejunal and ileal atresia may be considered as separate diseases.

In the past 2 1/2 years a sudden increase in the frequency of jejunal atresia in discordant, nonidentical twins was observed. Reported are the details of 11 cases and some reflections on possible pathogenetic mechanisms involved.

Two neonates with intestinal obstruction and two children (aged 1 and 4 years) with severe constipation since birth are reported in whom stenosis of the distal rectum was found. In association with the rectal anomaly, three of them had a presacral tumour (teratoma in two, hamartoma in one) and all had a deformed sacrum. An embryological hypothesis to explain this association has been postulated by Currarino, after whom this triad has been named. Two patients were related (father and daughter). The role of hereditary factors in the occurrence of the syndrome has been reported before. Operative treatment of the rectal stenosis was necessary in all patients. Preoperative diverting colostomy was performed in three cases, followed by a posterior sagittal approach to excise the rectal stenosis and the presacral mass. In one case, persistent cerebrospinal fluid leakage required re-exploration for closure of a tear in a congenitally abnormal dural sac. The fourth patient had undergone a low anterior resection in the past via the abdominal route and needed rectal dilatation afterwards for some time. The final result in all patients appears satisfactory, although follow-up is short. Most cases of this triad have been reported in children but a number of patients have been diagnosed only as adults. Recognition of this triad should imply a careful search for neural crest malformations. Operative treatment to correct all soft tissue anomalies leads to good results.

This report describes eight children with esophageal atresia who developed 'dyspnea spells' during feeding. These spells were caused by a combination of tracheomalacia and tracheal compression. One patient in addition had a vascular ring. Common characteristics in these patients were: low birth weight, other congenital abnormalities and a short esophagus. The diagnosis was confirmed endoscopically. Operative therapy consisted of hemithymectomy and aortopexy. In six patients the symptoms disappeared completely, the two other children improved. The operation appears an effective means of dealing with severe dyspnea spells in children after correction of esophageal atresia.

During 1971-1980, three consecutive clinical studies on nephroblastoma were conducted by the International Society of Paediatric Oncology (SIOP). Besides several questions on different postoperative treatment modalities, the emphasis of these trials was to determine the role of preoperative therapy. In SIOP-1 (1971-1974) we compared preoperative radiotherapy to immediate surgery. There is no difference in survival or recurrence-free survival between both groups. Tumour ruptures, however, did occur significantly less in the pretreated group, and the recurrence-free survival is clearly lower in patients with intraoperative tumour rupture. These results were confirmed by SIOP-2, a non-randomised study conducted between 1974 and 1976. The question tackled by SIOP-5 (1977-1980) was whether preoperative chemotherapy could be a substitute for preoperative radiotherapy. The two preoperative therapies produced equally satisfactory results in terms of recurrence-free survival and 4-year survival rates, and the reduction in the number of operative ruptures. In addition, it was shown that with preoperative chemotherapy about 45% of an unselected population of patients with Wilms' tumour can be treated and cured without any irradiation and its well-known sequelae. The actual need for radiotherapy in stage II tumours with negative lymph node involvement (about 30% of the patients after pretreatment) is under investigation in SIOP-6. Cytohistological grading is equally possible after chemotherapy, and also without pretreatment. If antitumour treatment is begun before histopathological diagnosis, then the risk of overtreating patients with benign conditions, or of administering an inappropriate treatment to patients with malignant tumours of other types, is about 6%.(ABSTRACT TRUNCATED AT 250 WORDS)