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Aim: Following surgical correction in the neonatal period, patients born with oesophageal atresia have significant co-morbidity, particularly in childhood. This study evaluates health-related quality of life and its determinants such as concomitant anomalies and the presence of respiratory and/or gastro-intestinal symptoms 6-18 years after repair of oesophageal atresia., Methods: Parents of 24 patients with oesophageal atresia completed the child health questionnaire for parents and 37 patients completed the child form. Gastro-intestinal symptoms were assessed by a validated standardized reflux questionnaire. Results were compared with a healthy reference population., Results: Parents as well as patients themselves scored significantly lower on the domain general health perception. According to parents, general health perception was negatively affected by age at follow-up and concomitant anomalies. Patients reported that reflux symptoms reduced general health perception., Conclusion: In this first study describing health-related quality of life in children and adolescents born with oesophageal atresia, we demonstrated that general health remains impaired because of a high incidence of concomitant anomalies and gastrointestinal symptoms in patients with oesophageal atresia when compared with the healthy reference population.

Background: Vincristine (VCR) and actinomycin D (ACD) form the backbone of chemotherapeutic regimens of Wilms tumor treatment. Veno-occlusive disease (VOD) is a potentially life-threatening complication of ACD., Objectives: To investigate the incidence of VOD after preoperative chemotherapy and assess the effect of dose and frequency of administrating ACD on the occurrence of VOD., Methods: A single-center retrospective study of patients where liver biopsies were performed after 4 or 8 weeks of preoperative chemotherapy. Patients had localized or metastatic Wilms tumor and were treated according to SIOP 9, 93-1, or 2001 protocol. A correlation was analyzed between histologically confirmed VOD, laboratory parameters, and mode and frequency of ACD administration. Long-term hepatic toxicity was assessed 5 years after the end of therapy., Results: Ninety-one patients were included in this analysis. Forty-one patients (45.1%) had histological evidence of VOD. The incidence of histologically proven VOD was significantly correlated with single administration of 45 microg/kg ACD (SIOP 2001 protocol) as compared to repeated dosing of l5 microg/kg (P = 0.003). Fifty-two percent of all patients had mild-to-severe abnormal liver enzymes 5 years after accomplishing therapy., Conclusion: Despite short-course preoperative chemotherapy regimen, patients are at risk of developing histological VOD. This risk is higher when ACD is administered in a 1-day 45 microg/kg regimen as compared to 3 days l5 microg/kg.

Objective: To assess psychological and social functioning and health related quality of life and its early determinants in children born with congenital diaphragmatic hernia (CDH)., Design: Cross-sectional follow-up study., Setting: Outpatient clinic of a tertiary care hospital., Participants: 33 CDH survivors aged 6-16 years., Main Exposure: Patients who developed CDH associated respiratory distress within 24 h after birth., Main Outcome Measure: Psychological and social functioning assessed with the Wechsler Intelligence Scale for Children (WISC-R), Bourdon-Vos test, Beery Developmental Test of Visual Motor Integration, Child Behavior Checklist (CBCL) and Teacher Report Form (TRF), and health related quality of life assessed with the Child Health Questionnaire (CHQ) and Health Utilities Index (HUI)., Results: Normal mean (SD) total IQ (100.0 (13.2)) and normal visual-motor integration, but significantly lower results for sustained attention (Bourdon-Vos test, 38.8 (11.2) points) were found. Learning difficulties were reported by 30% of parents. Eight children had scores in the clinical range on the CBCL and/or TRF, indicating clinically significant behavioural problems. Except for the CHQ scale General Health, health status was not different from the reference population. No significant correlations between test results and severity of CDH were found, except for an association of general health and physical functioning with length of hospital stay., Conclusion: CDH patients are at risk for subtle cognitive and behavioural problems, probably not related to CDH severity. Perception of general health is reduced compared to the reference population, indicating that CDH survivors and their parents believe their health is poor and likely to get worse.

The anti-Hu syndrome is a well-known paraneoplastic syndrome and may be rarely seen in patients with neuroblastoma. However, it is relatively unknown that anti-Hu antibodies can cause gastro-intestinal signs and symptoms. We report on a child with neuroblastoma who presented with gastro-intestinal disturbances as a result of the anti-Hu syndrome and summaries two similar case reports reported in literature. Neuroblastoma patients with gastro-intestinal disturbances, ranging from constipation to a paralytic ileus, might suffer from the gastro-intestinal anti-Hu syndrome. The causative antibodies can be determined to diagnose or exclude this syndrome, and successful treatment is possible.

Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly with a mortality rate of approximately 40-50%, depending on case selection. It has been suggested that new therapeutic modalities such as nitric oxide (NO), high frequency oxygenation (HFO) and extracorporal membrane oxygenation (ECMO) might decrease mortality associated with pulmonary hypertension and the sequelae of artificial ventilation. When these new therapies indeed prove to be beneficial, a larger number of children with severe forms of CDH might survive, resulting in an increase of CDH-associated complications and/or consequences. In follow-up studies of infants born with CDH, many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. CDH has also been associated with persistent pulmonary vascular abnormalities, resulting in pulmonary hypertension in the neonatal period. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity, although the underlying mechanism has not been fully understood yet. In adult CDH survivors incidence of esophagitis is high and even Barrett's esophagus may ensue. Yet, in many CDH patients a clinical history compatible with GERD seems to be lacking, which may result in missing patients with pathologic reflux disease. Prolonged unrecognized GERD may eventually result in failure to thrive. This has been found in many young CDH patients, which may also be caused by insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. In patients treated with ECMO, the incidence of neurological deficits is even higher, probably reflecting more severe hypoxemia and the risk of ECMO associated complications. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but most of them concentrate on the first years after repair and only a few studies focus on the health-related quality of life in CDH patients. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are mandatory. Based on such studies a more focused approach for routine follow-up programs may be established.

Background: In African settings the treatment results of Burkitt's lymphoma (BL) seem to be less favourable compared with Western settings. The aim of this retrospective study was to analyse some factors that affect the treatment of BL., Patients and Methods: Over a 16 year period, data were extracted of 80 patients., Results: Complete remission 5%, very good partial response 35%, partial response 16%, no response 10%, data missing 34%. Of all patients, 56% did have a positive response to treatment. However, 51% of this subgroup of patients did not finish treatment. There was no difference in completion of treatment between patients living in Katete district finishing treatment vs. living outside Katete district (respectively 25% vs. 32%, P = 0.7148)., Conclusion: There is potential for higher cure rates for BL in tropical settings if full effort is put into compliance since a majority of patients, even while having a good prognosis, abandon treatment. Large distance to hospital makes no difference in completing the chemotherapy course.

An intrathoracic mass, which persisted during the remaining pregnancy, was first seen during routine ultrasound examination performed at 20 weeks gestation. After birth, the child was asymptomatic. Echocardiography showed the mass to be located intrapericardially. The mass was electively resected via sternotomy 3 weeks after the birth. Microscopic examination showed normal lung tissue surrounded by pleura corresponding to the diagnosis of extralobar pulmonary sequestration. To the authors' knowledge, this is the earliest described detection of such a lesion. Furthermore, this article reports the unique finding of a feeding vessel from the right pulmonary artery.

The aim of this study was to evaluate oesophageal function after correction of oesophageal atresia in adults, and to investigate the association between complaints, oesophageal function and quality of life (QoL). Twenty-five adults were included who participated in previous follow-up studies, during which complaints of dysphagia and gastro-oesophageal reflux (GOR), results of upper gastrointestinal endoscopy, oesophageal biopsies and QoL had been collected. Manometry was performed in 20 patients, 24 h pH-measurements were performed in 21 patients. pH-values (sample time 5 s) were calculated using criteria of Johnson and DeMeester. Associations were tested with ANOVA and chi (2)-tests. Ten patients (48%) reported complaints of dysphagia, seven (33%) of GOR. The amplitude of oesophageal contractions was low (<15 mmHg) in four patients (20%). pH-measurements showed pathological reflux in three patients (14%). Patients reporting dysphagia more often had disturbed motility (P = 0.011), and lower scores on the domains "general health perceptions" (SF-36) (P = 0.026), "standardised physical component" (SF-36) (P = 0.013), and "physical well-being" (GIQLI) (0.047). No other associations were found. This study shows a high percentage of oesophageal motility disturbances and a moderate percentage of GOR after correction of oesophageal atresia. Patients reporting dysphagia, whom more often had disturbed motility, seemed to be affected by these symptoms in their QoL.

--Undescended testis (UDT) is one of the most common urogenital abnormalities in boys. --UDT is defined as a testis which cannot be brought into a stable scrotal position. --At present, congenital and acquired forms of UDT are recognised. Congenital UDT is defined as a UDT which has never descended from birth. Acquired UDT is defined as a UDT which has been fully descended in the past. --Congenital UDT should be treated surgically between 6 to 12 months of age. --The treatment of acquired UDT is still disputed. As yet, awaiting spontaneous descent at early puberty seems to be the most rational treatment. --In the Netherlands, the high number of late orchidopexies is due to surgery for acquired UDT. To reduce this high number, the guidelines of the first development conference on 'non-scrotal testis' dating back to 1986 should be revised on several points.

Objectives: To analyze the long-term results of Boerema anterior gastropexy using the Visick grading system in a university teaching hospital., Methods: Retrospective review of 247 children with severe or complicated gastroesophageal reflux disease (GERD). Patients who underwent primary gastropexy between 1990 and 2001 were divided in 3 groups: (A) neurologic impaired patients (n = 83); (B) esophageal atresia patients (n = 36); and (C) patients without underlying disease (n = 128). The median length of follow-up after gastropexy was 7 years and 8 months (range, 1.5-13 years). Follow-up was carried out using a standardized questionnaire and was obtained by telephone. Postoperative recurrence of symptoms during follow-up was considered as failure of the operation., Results: Postoperative complications occurred in 20 patients (24%) in group A, in 13 patients (36%) in group B and in 23 patients (18%) in group C. Thirty-three patients died during the follow-up period, 5 of whom during the postoperative period. Six children could not be traced; thus, 208 were available for long-term follow-up. Postoperative failures occurred in 12 patients (23%) in group A, in 11 patients (31%) in group B and in 17 patients in group C (14%). Reoperation for recurrence of symptoms due to GERD was performed in 6 patients in group A, in 7 patients in group B and in 2 patients in group C. The final outcome at the time of interview was successful in 81% of group A, in 88% of group B and in 91% of group C., Conclusions: In the long term, Boerema anterior gastropexy is an effective operation for complicated GERD in children without underlying disease as well as in neurologically impaired children and patients with esophageal atresia.

The first description of oesophageal atresia dates back to 1670. Oesophageal atresia used to be regarded as a lethal disease, but since the successes of Leven and Ladd in 1939 and Haight in 1941 surgical correction is possible. The Dutch history ofoesophageal atresia begins in 1946. After the successes in America became known, several Dutch surgeons started to treat patients with oesophageal atresia, notably J.ten Kate, L.D.Eerland, M. Schoorl and P.J.Kooreman. In those days, paediatric surgery did not yet exist as a separate specialty. Today, paediatric surgery is concentrated in 6 paediatric surgical centres in the Netherlands. Thanks to the pioneers mentioned and the concentration of knowledge and expertise in the centres, the mortality ofoesophageal atresia patients in the Netherlands has decreased to approximately 9%, despite the fact that currently the gestational age and birth weight of patients is lower and the number of patients with comorbidity is higher.

Aim: To report 24 years of pre-treatment biopsy and surgical experience in primary liver tumours in children., Methods: Between 1979 and 2003, 53 children presented with a primary liver tumour of whom 48 who underwent surgical resection were evaluated (two died, two were unresectable, and one was transplanted). Biopsy data, per- and post-operative complications, mortality, and survival were retrospectively reviewed., Results: Benign tumours were diagnosed in eight patients. Surgical resection for a malignant tumour was performed in 40 patients (26 hepatoblastomas (HB), eight hepatocellular carcinomas (HCC) (four had fibrolamellar HCC), three rhabdomyosarcomas (RMS), one neuroblastoma, one non-hodgkin lymphoma (NHL), and one teratoma). Primary resection was performed in one HB, and four HCCs. The cumulative survival without evidence of disease was 73% for HB (median 7 years) and 88% for HCC (median 3.5 years)., Conclusion: The treatment results are comparable with those of larger international series except for HCC. The existing diagnostic pitfalls in differentiating between the various liver malignancies justify the use of a diagnostic biopsy.

A boy aged 6 months and a girl aged 9 months were admitted due to vomiting, among others, and a boy aged 11 months due to pneumonia. It turned out that they had a congenital diaphragmatic hernia. Primary operative repair was performed successfully in all patients, followed by recovery. The older boy experienced a relapse nearly 1 year later, which was treated by surgical correction. Most congenital diaphragmatic hernias present directly after birth, with cyanosis and respiratory distress. However, 10-20% of the cases are discovered after this period. In these children diagnosis can be difficult because of the diverse symptoms such as vomiting, feeding difficulties, tachypnoea or recurrent respiratory tract infections. Physical signs include the absence of breath sounds or the presence of bowel sounds in the chest. Chest X-ray, contrast upper gastrointestinal series or ultrasound imaging confirms the diagnosis. Delay in treatment can lead to complications such as necrosis of the bowel. In young children with acute or chronic respiratory infections or gastrointestinal complaints, a congenital diaphragmatic defect should be considered.

In 3 patients with serious constipation or faecal incontinence the appendix was sewn open-ended into the abdominal wall (Malone-stoma) allowing antegrade bowel washouts. The patients were a 4-year-old boy with Hirschsprung's disease, a 15-year-old boy with chronic functional constipation and encopresis, and a 5.5-year-old boy with incontinence associated with a meningomyelocele. Following the procedure they were free from bowel problems. Faecal incontinence has a major detrimental effect on the psychosocial development of children. Severe constipation and faecal incontinence which do not optimally respond to conventional therapy can be treated by this procedure. Treatment is effective in around three-quarters of these children. Complications occur in an equal number of children, stenosis being the most frequently seen.

A 16-year-old girl presented with signs of proximal intestinal obstruction. In the neonatal period, surgical correction of an apple peel atresia had been performed, and she also had a situs inversus abdominalis. Revision of the anastomosis had been done when she was 3 years old. Contrast studies apparently again showed a stricture of the anastomosis, which was treated by stricturoplasty. Because of persistent obstruction, reexploration was done and revealed a duodenal membrane. Anastomotic strictures are very rare several years after the primary operation, so other causes of obstruction should be sought.

Three children presented with bilious vomiting due to malrotation at the age of 3, 5 and 12 years, respectively. They were treated surgically and recovered fully. In the period 1989-2002, 12 patients were operated for malrotation after the first year of life in the Emma Children's Hospital AMC, Amsterdam, the Netherlands. The mean age at operation was 5 years (range: 1-15). The most important symptoms were (bilious) vomiting and abdominal pain. Four patients had a history of bilious vomiting in the neonatal period. Nine patients had been previously admitted to a hospital with abdominal complaints. The most sensitive imaging technique was an upper gastrointestinal contrast study. Malrotation with intermittent volvulus can cause recurrent abdominal complaints and vomiting in children. Bilious vomiting is pathologic at any age and should lead to further investigations, preferably an upper gastrointestinal contrast study to exclude malrotation or other obstructions.

The 'Inguinal hernia' guideline was written over a period of two years by nine surgeons (including one epidemiologist) from all regions of the Netherlands with demonstrable clinical and scientific expertise in the area of inguinal surgery after a training course on 'The development of evidence-based guidelines'. A draft of the guideline was on the website of the Association of Surgeons of the Netherlands for a period of three months, during which time the members of the society could comment on its contents interactively. The guideline comprises chapters on risk factors and prevention, diagnostics, indications for treatment, treatment, day surgery, antibiotics, thrombosis prophylaxis, training, anaesthesia, postoperative pain control, complications, costs, aftercare, and specific aspects of inguinal hernia in children. For the treatment of adult patients a mesh technique is recommended. The Lichtenstein technique is recommended as the first choice for uncomplicated primary inguinal hernia. Laparo-endoscopic techniques can be used by trained teams for specific indications. Other techniques have not been compared with the current methods of treatment sufficiently. It is recommended that the operations be carried out in daycare and that the use of local anaesthesia should be considered more often. The diagnosis of inguinal hernia in a child is based on the physical examination. It is recommended that the surgeon should not rely solely on the history but confirm the presence of a hernia personally. The treatment of a paediatric inguinal hernia is always operative. Generally, the younger the child, the more urgent the operation because of the increased risk of incarceration in infants, particularly premature babies. There is no indication for routine exploration of the contralateral groin. If an incarcerated hernia cannot be reduced, emergency operation is necessary and referral to a paediatric surgical centre must be considered. The implementation and effectiveness of the guideline will be measured by taking an inventory of all inguinal hernia operations performed in the Netherlands before and after its publication.

Purpose: The aim of this study was to compare presentation, complications, diagnosis, and treatment of choledochal cysts in pediatric and adult patients., Methods: Forty-two patients were analyzed after subdivision into 3 groups: group A, less than 2 years (n = 10); group B, 2 to 16 years (n = 11); group C, greater than 16 years (n = 21)., Results: The cysts were classified as extrahepatic (n = 33), intrahepatic (n = 5), and combined (n = 4). Seventy-six percent of patients presented with abdominal pain, (20 of 21 group C), and 57% with jaundice, (10 of 10 group A). Cholangiocarcinoma occurred in 6 patients, 4 of whom had previously undergone internal drainage procedures. Excision of the extrahepatic cyst was performed in 27 of 37 patients. Five patients, of whom, 4 had cholangiocarcinoma, were beyond curative treatment at the time of diagnosis. Six patients had died at the closure of this study, 5 of them had carcinoma., Conclusions: Presenting symptoms are age dependent with jaundice prevailing in children and abdominal pain in adults. In view of the high risk of cholangiocarcinoma, early resection and not internal drainage is the appropriate treatment of extrahepatic cysts. Patients who had undergone internal drainage in the past still should undergo resection of the cyst., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

In a 5-year-old boy idiopathic scrotal edema was diagnosed, a rare self-limiting disease.

Objective: To quantify the potential impact of simple measures to reduce the risk of iatrogenic HIV infection through blood transfusion in a Zambian district hospital., Methods: Three studies were conducted at St. Francis' Hospital, Katete, Zambia: (1) From 1991 to 1995 HIV seroprevalence among all listed blood donors and the impact of proper subgroup selection were studied retrospectively; (2) the sensitivity of locally used rapid antibody assays (HIV-spot/Wellcozyme HIV 1 & 2) for the detection of HIV in donor blood and the influence of the expiration date of the tests on this sensitivity were determined prospectively from June 1993 until March 1994 by screening all consecutive surgical patients and blood donors; (3) the number of unnecessary blood transfusions was determined retrospectively from January 1995 through January 1996 and prospectively from February 1996 through March 1996, and possibilities to reduce the total number of blood transfusions were considered., Results: (1) Excluding prisoners, who have an HIV seroprevalence of 19-25%, from the donor population significantly reduces the overall HIV seroprevalence from 13-16% to 8-9% (P < 0. 01). (2) Under local circumstances the sensitivity of the used rapid antibody assays was 6.8-17.9% lower than claimed by the manufacturer. Usage of non-expired tests increased the sensitivity significantly from 88.2% to 91.7% (P < 0.05). (3) None of the 294 studied blood transfusions can be classified as inappropriate according to international standards., Conclusions: Simple measures such as proper subgroup selection among blood donors and correct use of non-expired tests may decrease the risk of iatrogenic HIV transmission. Stricter indications for blood transfusions will not substantially reduce the number of transfusions.

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.

Administration of a tumour-seeking compound labeled with a low-energy isotope and intraoperative screening with the gamma probe (radioguided surgery, RGS) could be useful in reoperations for advanced neuroblastoma when the normal anatomy is altered. A pilot study was performed to test the feasibility of this technique. Five patients underwent six relaparotomies for recurrent stage III or IV neuroblastoma. All had been treated with intensive chemotherapy and/ or metaiodobenzylguanidine (MIBG)-I131 with or without hyperbaric oxygen. Reoperation was performed to achieve near-total (greater than 95%) excision. In all instances, active tumour was seen on the preoperative MIBG scan. Before the operation, a tracer dose of MIBG-I123 was given. At laparotomy, a search was made with the gamma probe for areas of increased activity. The gamma probe correctly identified active neuroblastoma tissue that was seen on the preoperative MIBG scan. There appeared to be a relationship between intensity of radioactivity and degree of maturation on histologic examination. This pilot study shows that RGS with MIBG and intraoperative use of the gamma probe is able to identify recurrent neuroblastoma. Whether this method is able to detect occult tumour and whether RGS will result in better outcome are the subjects of ongoing research.

Objective: To investigate the frequency of lumbosacral anomalies, the association with urogenital abnormalities, and the correlation with defaecation pattern by magnetic resonance imaging (MRI)., Methods: A prospective analysis was performed of routine MRI in patients with anorectal malformations. Between 1990 and 1994, MRI was performed in 43 such patients: 31 boys and 12 girls. Twenty four had a high anorectal malformation, 16 had a low anorectal malformation, and three had Currarino's triad. MRI was performed before reconstruction in 26, and postoperatively in 17. Urogenital anomalies were found in 21., Results: Abnormalities of the spinal cord and spine were found with MRI in 20 patients (46.5%); caudal regression syndrome in 10, tethered cord in two, a combination of both in three, and other spinal anomalies in five. These anomalies were found in 30% of the patients with low anorectal malformations, and in 50% with high anorectal malformations. In patients with urogenital malformations, MRI more often showed spinal anomalies (13/21, 62%) than in patients without (7/22, 32%). In high anorectal malformations, defaecation was more often a problem in patients with spinal anomalies (12/15, 80%) than in patients without (2/8, 25%)., Conclusions: Spinal anomalies in the lumbosacral region were found with MRI in 46.5% of patients with anorectal malformations. Since presence of these anomalies seems to be related to clinical outcome, MRI should be performed routinely in all such patients.

Two cases of caecal duplication are presented, one in a neonate and one in an infant. The diagnosis was made at laparotomy, which had been undertaken for the presumptive diagnosis of intestinal atresia and torsion of an ovarian cyst respectively. Also the literature on alimentary tract duplications is reviewed, referring to the incidence, presenting symptoms, and location of the duplication, in particular that of the caecum.

The object of this study was to establish the relation of symptomatic diverticula to the age and gender of the patients and to the presence of ectopic tissue. A total of 136 patients with surgically treated diverticula were collected from the medical charts of five Amsterdam hospitals; 51 had undergone resection because of diverticulum-related symptoms and 85 during laparotomy for other causes. Obstruction was the predominant symptom (39%) in the 51 symptomatic patients. Hemorrhage, perforation, diverticulitis, and intussusception were the other symptoms (12-14% each). Obstruction occurred mainly in patients under age 10 years and perforation in patients 10 to 30 years old. All symptoms, hemorrhage excepted, occurred two to four times more in men. Hemorrhage and perforation were associated with the presence of ectopic gastric tissue. We concluded that symptoms caused by Meckel's diverticula are mainly due to the presence of bands or ectopic gastric tissue. The symptoms manifest at an early age (77% in those under age 30) and predominantly in males. Diverticula found incidentally in patients younger than 30 years should be resected. In the older patients, resection is indicated if ectopic gastric tissue is suspected. Diverticular bands can simply be cut.

Long-term anorectal function was assessed in children operated on using Duhamel's technique for Hirschsprung's disease. The files of 75 patients (16 girls and 59 boys) operated on between 1977 and 1991 were reviewed. Questionnaires were analysed on 63 (12 girls and 51 boys) over 2 years of age. The median age at completing the questionnaire was 6.6 years. Forty-one of these 63 patients had aganglionosis of the rectosigmoid, 15 of a long colonic segment, and 7 of the total colon. Of 14 children age 4 or less, 6 had severe constipation, whereas 8 had regular spontaneous defaecation. Ten of the 49 over 4 years of age were continent without constipation, 22 had soiling and/or constipation, and 17 were incontinent, one of whom had a permanent colostomy. There was no correlation between age or sex and anorectal function. Anorectal function in children with long segment was not worse than in those with rectosigmoid aganglionosis, but only 1 of 7 with total colonic aganglionosis was continent. Because many adapt themselves to the handicap, symptoms are often underreported. A detailed questionnaire appears to be a reliable tool for elucidating the real situation. A systematic follow-up of patients with Hirschsprung's disease is proposed to anticipate the complications and to institute proper measures at an early stage.

33 previously untreated advanced stage neuroblastoma patients were treated with [131I]meta-iodobenzylguanidine (MIBG). The number of treatments varied between 2 and 7 per patient (mean 3). Toxicity was seldom severe. Only thrombocytopenia WHO-grade 4 was noticed. Response was documented before surgery for the primary tumour was performed. There was one complete response (CR), 18 partial responses (PR), 11 had stable disease (SD) and 3 had progressive disease (PD). After MIBG therapy and surgery, 12 of 33 patients achieved a CR. This approach is feasible, comparable to multidrug chemotherapy in efficacy and less toxic. Long term results are not known yet.

Background: A retrospective analysis was performed of the results of surgical excision of lung metastases in children to identify prognostic factors., Methods: From 1970 to 1992, 139 thoracotomies were performed in 91 patients between 1 and 19 years of age with metastases of osteogenic sarcoma (40 patients), nephroblastoma (24 patients), Ewing sarcoma (12 patients), and various other tumors (15 patients)., Results: There were no perioperative deaths and only one serious complication, chylothorax necessitating reoperation. Twenty-three patients (25%) are currently alive, two with residual disease. Twelve patients (50%) with nephroblastoma are alive; 7 patients (18%) with osteogenic sarcoma and 4 (27%) with other tumors are alive. Negative prognostic factors were incomplete excision, primary tumor not controlled, or metastases developing during treatment. The number of metastases, the disease-free interval, unilateral versus bilateral metastases, preoperative and postoperative adjuvant treatment, and the number of thoracotomies performed were not of significant influence on outcome., Conclusions: The most important prognostic factor is the type of primary tumor. Excision of lung metastases in children with Ewing or soft tissue sarcoma is not warranted. All other patients who are able to withstand a major operation should not be denied the chance because the surgical risks appear minimal and the outcome cannot be predicted beforehand.

Gastro-oesophageal reflux (GER) in neurologically impaired children often causes feeding problems and complications of oesophagitis and is frequently resistant to medical treatment. Fifty neurologically impaired children underwent anterior gastropexy as anti-reflux operation, combined with gastrostomy in 23, between 1976 and 1992. There was no operative mortality. There were 25 early complications in 14 patients and 9 late complications in 9 patients. Twelve patients needed 17 re-operations for delayed gastric emptying [4], intestinal obstruction [3], para-oesophageal hernia [3], oesophageal stenosis [4], and recurrent GER, revision of gastrostomy, subphrenic abscess (one each). Nine patients died during the follow up period. Death in two children was related to the operation (incarcerated para-oesophageal hernia and blow-out of the stomach). Out of 41 survivors, the operation was judged successful in 35. It is concluded that antireflux operations in neurologically impaired children carry a high risk of complications. Preoperative identification of risk factors is not possible. The improvements in the quality of life achieved in the majority of patients outweigh the risks.

Between 1970 and 1990, 23 patients were treated for embryonal rhabdomyosarcoma of the bladder (8), prostate (7), uterus (4) or vagina (4). One girl underwent exenteration without chemotherapy. Of the patients 22 received chemotherapy as initial treatment. There was 1 treatment-related death. A total of 21 patients completed the first phase: 4 were treated with chemotherapy alone and 17 subsequently underwent an operation. Three boys underwent total cystoprostatectomy and local resection was performed in 14 patients. One boy was lost to followup. Mean followup was 11.2 +/- 6.3 years in 15 survivors. Complete remission was achieved in 19 patients, while 11 (53%) had relapse at an interval of 3 to 102 months. Of 13 patients with bladder and prostate tumors 8 had relapse and 7 survived. Of 8 patients with uterus and vagina tumors 3 had relapse and all survived. Six patients died of recurrent disease (overall mortality rate 32%). The interval from initial diagnosis until death was always less than 5 years. The mortality rate was greater in boys (5 of 12) than in girls (1 of 9). Preservation of pelvic organs in girls was 75%, while of the boys 44% retained the bladder. Since disease relapsed in patients in whom no tumor was found in the resection specimen after chemotherapy, we conclude that a conservative surgical approach combined with chemotherapy towards urogenital rhabdomyosarcoma is justified. Although late relapses do occur, they can usually be salvaged by a combination of chemotherapy, radiotherapy and surgery.

Since traction-associated hypertension seems to be a relatively unknown phenomenon, a survey was done of its incidence in children treated with skeletal traction for fractures and orthopaedic diseases. The correlation with hypercalcaemia, a possible aetiological factor, was also explored. Blood pressure was recorded three times a day with an automatic oscillometric unit during the stay in the hospital. Serum calcium, creatinine and total protein concentrations were measured once a week. Patients with pre-existing diseases or renal trauma were excluded. Arterial hypertension (systolic and/or diastolic) was found in 31/50 children (62%). In almost half of these the rise in systolic blood pressure was 10 mmHg or more above the 95th percentile. Hypertension occurred in most cases within the first 3 weeks of treatment; in 7 children it developed after 3 or more weeks of traction. All children became normotensive within 1 week after discontinuation of traction. Clinical symptoms were rare: two children complained of headache. In no instance had traction to be discontinued before the planned date because of hypertension. In the hypertensive group were more preschool children and more humeral fractures as compared to the normotensive group (n = 19). Hypercalcaemia occurred in 11 children and was equally distributed in hypertensive and in normotensive children. It is concluded that arterial hypertension is a frequent finding in children in traction, but its clinical relevance is uncertain. Hypercalcaemia is not a rare finding in immobilized children, but probably plays no causative role in traction-related hypertension.

There is evidence that gastrostomy can induce gastroesophageal reflux (GER). We used pH monitoring in piglets to evaluate GER after gastrostomy and to assess the effect of anterior gastropexy. Oesophageal pH studies were performed before and after gastrostomy with (8) and without (9) anterior gastropexy. The reflux score was the percentage of time that pH was below 4.0. Short episodes (mean reflux score 2.6%; range, 1.6% to 30%) of GER occurred in half of the animals before surgery. After gastrostomy, reflux episodes occurred more frequently (77% of animals) and were more prolonged (mean reflux score 35.2%; P less than .001). Anterior gastropexy with gastrostomy prevented GER in all 6 piglets with an intact gastropexy. In two animals with reflux after gastrostomy with gastropexy the gastropexy appeared dehiscent at autopsy. Microscopic ulcerative esophagitis was present in more than half the animals with a positive pH study. We conclude that (1) piglets demonstrate short episodes of spontaneous GER; (2) gastrostomy increases the duration and incidence of the GER episodes; (3) anterior gastropexy prevents gastrostomy-induced GER in piglets; and (4) gastrostomy in piglets is a suitable model for studying GER. We advocate protective anterior gastropexy when performing a feeding gastrostomy.

Hypospadias is a congenital malformation of the penis, in which the external meatus is ventrally situated. Proximal localisation and chordee result not only in cosmetic-psychologic but also in functional disturbances. Complete operative correction (a straight penis with the meatus on top) is performed around the second year. Various procedures are used depending on the localisation of the meatus. Glandular and coronal forms are managed in our series by the MAGPI operation. In the more proximal forms a preputial island flap operation, either as an onlay or as tube, is performed. The results of the MAGPI were good: only 3 of 27 children needed a second procedure. After island onlay operations a second operation was necessary in 5 of 8 children. Six of 7 patients with an island tube operation developed a fistula which was closed subsequently. The final result was good in all children, both from cosmetic and functional point of view.

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.