Parental decisional satisfaction after hypospadias repair in the United Kingdom.

BACKGROUND: In hypospadias, the aim of surgical treatment is to achieve both desirable functional and cosmetic outcomes; however, complications following surgery are common and 18% of boys require re-operation. In mild degrees of hypospadias, repair may be offered entirely to improve cosmesis, meaning parents should be fully informed of this and the potential for complications, during the consent process. Parents' decision-making may be aided by making them aware of how others in a similar position have felt about the decision that they made for their child. One method of measuring parental satisfaction is decisional regret (DR). OBJECTIVES: To assess parental satisfaction following hypospadias surgery in the United Kingdom by assessing DR and to determine the feasibility of obtaining meaningful data via a mobile phone survey. STUDY DESIGN: The National Outcomes Audit in Hypospadias database was commissioned by the British Association of Paediatric Surgeons to capture clinical information from hypospadias repairs. Following ethical approval (16/NW/0819), a text message was sent to mobile numbers in the database inviting participation in a questionnaire incorporating the validated DR scale (DRS). The primary outcome measure was mean DRS score, which was correlated with clinical information, a score of zero indicated no regret and 100 indicated maximum regret. RESULTS: There were 340 (37%) responses. The median age at the primary procedure was 16 (interquartile range 13-20) months. No DR (score = 0) was detected in 186 (55% [95%CI 49-60]) respondents; however, moderate-to-severe DR (score = 26-100) was seen in 21 (6.2% [95%CI 3.6-8.7]) respondents. On multivariate analysis, a distal meatus, a small glans and developing complications requiring repeat surgery were all associated with increased levels of regret (Table). There was no association between DR and cases performed per surgeon. DISCUSSION: Around half of respondents demonstrated no DR and postoperative complications requiring surgery were associated with the highest levels of DR, which is similar to a Canadian study. Lorenzo et al. however found that DR was associated with circumcision, which was undertaken in all boys; however, in this UK study, around a third of boys were circumcised and regret levels between those circumcised and those not circumcised were similar. The limitations of this work include the following: surgeons submitting their own data on complications and there is potential of selection bias between respondents and non-respondents as with any survey. CONCLUSIONS: Data from this study can be used to improve pre-operative counselling during the consent process. Smart mobile phone technology can be used successfully to distribute and collect parent-reported outcomes.

Complete androgen insensitivity syndrome associated with bilateral Sertoli cell adenomas and paratesticular leiomyomas: case report and review of the literature.

BACKGROUND: Complete androgen insensitivity syndrome (CAIS) is a rare and usually unexpected cause of primary amenorrhoea that results from receptor resistance to androgens, producing a female phenotype in genetically male patients. CASE: A 16-year-old girl was diagnosed with CAIS after investigations for primary amenorrhoea. Her left inguinal gonad and the right intra-abdominal gonad were resected and histopathology revealed the presence of dysgenetic multinodular testes with absence of germ cells, significant hyperplasia of Sertoli cells (Sertoli cell adenoma) and presence of paratesticular leiomyomas. CONCLUSION: Although the risk of gonadal tumour development is considered to be low, a variety of tumours have been described in association with CAIS, but this is the first report of development of bilateral paratesticular leiomyomas developing concurrently with Sertoli cell adenomas.

Surgical strategies in the management of ecthyma gangrenosum in paediatric oncology patients.

Clinical presentation and microbiology profiles of neutropenic paediatric oncology patients presenting with ecthyma gangrenosum (EG) were studied. Surgical strategies deployed for these critically ill children are reported. Between 1994 and 2005, all children with EG were identified. Case notes were reviewed. Hospital course and long-term outcome were documented. Ten patients were identified. Eight had acute lymphoblastic leukaemia, one child had acute myeloid leukaemia and another had rhabdomyosarcoma. Lesions occurred in the perineal region (n = 5), buttocks (n = 2), thigh (n = 2) and the face (n = 1). Seven children had positive blood cultures for Pseudomonas aeruginosa. Surgery included (1) radical debridement, and (2) debridement with covering colostomy for four of those with perianal lesions. Ecthyma gangrenosum is a rapidly spreading and potentially lethal condition. Paediatric oncology patients with neutropenia are at a high risk. Surgical excision is crucial for progressive lesions to prevent mortality.

Less is best? The impact of urokinase as the first line management of empyema thoracis.

To assess the impact of intrapleural urokinase and small tube thoracostomy on the management of childhood empyema thoracis. The study population included 38 children presenting consecutively to a regional surgical unit with empyema thoracis from January 2001 to December 2003. Children with malignancy, immunodeficiency and complex intercurrent illness were excluded. Primary outcome variables were the need for second intervention and duration of stay, with other variables including duration of antibiotics, serial CRP and amelioration of pyrexia. Interventions were: tube thoracostomy (16-20 Fr) alone (n=2), tube thoracostomy (6-10 Fr)+urokinase (n=17), thoracoscopy (tube: 20-24 Fr) (n=9), thoracotomy (tube: 16-24 Fr) (n=10). There were no differences in age, weight or length of prodromal symptoms, between treatment groups. There were no differences in primary outcome variables, although no child undergoing thoracotomy required further intervention. The duration of intravenous antibiotics was similar in all groups. Amelioration of pyrexia was more rapid in children undergoing thoracotomy. There were no differences seen with regard to decline in CRP level. Small tube thoracostomy and intrapleural urokinase had a similar outcome to more invasive therapies such as thoracotomy or thoracoscopy thereby supporting the evidence base for urokinase and tube drainage as first line intervention.

Laparoscopic repair of late presenting congenital diaphragmatic hernia.

Congenital diaphragmatic hernia usually presents in the neonatal period, with delayed presentation being uncommon. Traditionally repair was performed by laparotomy or thoracotomy. We have performed laparoscopic repair of a previously undiagnosed congenital diaphragmatic hernia that presented acutely in a 10-year-old male. Laparoscopic repair of late-presenting congenital diaphragmatic hernia is a safe and effective approach even in an emergency. The laparoscopic approach has advantages including reduced hospital stay, excellent visualisation of the defect even for obese patients, and improved cosmesis.

Trends in paediatric circumcision and its complications in England between 1997 and 2003.

BACKGROUND: It has been suggested that too many English boys undergo circumcision. This report describes how circumcision rates have changed in England between 1997 and 2003, including data on complication rates and on how age, medical indication and surgical specialty affect postoperative haemorrhage rates. METHODS: Data were extracted from the Hospital Episode Statistics database of admissions to National Health Service hospitals in England. Patients were included in the study if an Office of Population Censuses and Surveys version 4 code for circumcision was present in any of the operative procedure fields of the database; 75 868 boys below 15 years of age were included in the study. RESULTS: Circumcision rates declined by about 20 per cent, from 2.6 per 1000 boys per year in 1997 to 2.1 in 2003. Between 2000 and 2003, circumcision rates remained static at 2.1 per 1000 boys per year. Circumcision rates fell by 31.2 per cent for boys aged 0-4 years, 9.3 per cent for boys aged 5-9 years and increased by 7.7 per cent in boys aged 10-14 years; 90.2 per cent of circumcisions were done for phimosis and 1.2 per cent of boys experienced a complication. CONCLUSION: Circumcision rates in England continued to fall up until 2000, particularly in those aged under 5 years, in whom pathological phimosis is rare. The circumcision rate remains five times higher than the reported incidence of Phimosis.

Comparison of functional outcomes of Duhamel and transanal endorectal coloanal anastomosis for Hirschsprung's disease.

PURPOSE: The aim of this study was to determine the morbidity and medium-term functional outcome of the Duhamel operation and laparotomy and transanal endorectal coloanal anastomosis (TECA) for Hirschsprung's disease (HSCR). METHODS: The study populations were 34 consecutive children who underwent the Duhamel operation (or Lester Martin modification) and 37 who had the TECA. Demographic details were obtained by case note review, and functional outcome was determined by a combination of outpatient interview, questionnaire, and telephone enquiry. RESULTS: There was no difference between the groups with respect to age, gender, and length of aganglionic segment. Seventy percent presented as neonates (Duhamel, 24 of 34; TECA, 26 of 37). A single-stage primary pull-through was performed in 17 of 37 children in the TECA group, and in 1 of 34 from the Duhamel group. There was a single perioperative death in the Duhamel group and an unrelated, late death in the TECA group. Postoperative enterocolitis was seen in 13 of 37 TECA children and in a single child from the Duhamel group. A stricture of the pull-through segment was seen in 7 of 37 children after TECA and required temporary diversion in 2 of 9. Late division of a rectal spur was required in 6 of 33 Duhamel children. Requirement for late myectomy was the same in both groups (Duhamel 3 of 33, TECA 4 of 37). Complications requiring stoma formation occurred in 5 of 37 after TECA and 2 of 33 after the Duhamel operation. Two children from the TECA group and 1 from the Duhamel group remain diverted. One child from each group required a re-pull-through procedure. Two patients were lost to follow-up in the TECA group, leaving 34 children in this group and 33 in the Duhamel group in whom functional outcome could be assessed. Functional outcome was similar in the 2 groups. CONCLUSIONS: TECA and Duhamel procedures have similar medium-term functional outcomes. TECA has a high incidence of postoperative enterocolitis and transient stricture formation but is suitable for single-stage neonatal treatment of HSCR.

After the honeymoon--medium-term outcome of antegrade continence enema procedure.

PURPOSE: The aim of this study was to determine medium-term outcomes of the antegrade continence enema (ACE) procedure. METHODS: A retrospective casenote review plus telephone questionnaire was conducted. The study was performed at a regional paediatric surgical centre. The subjects were consecutive children undergoing the ACE procedure over a 5 year period. Main outcome measures were use of the ACE; reversal rates; complications, ease of use, effectiveness, and satisfaction scores. Data are expressed as median (range). RESULTS: Thirty-two (52%) of 62 children undergoing the ACE procedure were girls. The age at the time of operation was 11.5 (3.8 to 17.6) years. Underlying diagnoses included spina bifida (n = 31), anorectal malformations (n = 15), slow-transit constipation (n = 9), Hirschsprung's disease (n = 2), sacral agenesis (n = 2), and trauma/tumour (n = 2). Median follow-up was 5.4 (3.25 to 8.25) years. Eleven of 62 (18%) children were no longer using the ACE (n = 5) or had it surgically reversed (n = 6; 14.1 +/- 9.3 months postprocedure). Reasons for disuse/reversal were lack of effectiveness (n = 4), complications (n = 2), noncompliance (n = 3), independent continence (n = 1), and pain (n = 1). Five (8%) children currently have a colostomy. Gender (P =.31; Fisher's Exact), age (Pearson), and underlying diagnoses (P =.07, Chi2) were not predictors of failure. Overall, stomal stenosis was the most common complication, affecting 26 of 62 (41%) children. Of 32 questionnaire respondents to linear scores, ease of use was rated as 2 (0 to 8, 0, very easy; 10, very difficult), discomfort on use as 3 (0 to 9; 0, no pain; 10, very painful), overall satisfaction as 9 (0 to 10; 0, completely dissatisfied; 10, completely satisfied). Eighty-four percent were completely continent or had soiling less than once a month. There was a significant correlation between the level of continence and satisfaction with the procedure (P =.04; Pearson). CONCLUSIONS: The ACE procedure offers significant benefits to some children with incontinence or intractable constipation. However, it is not universally successful, and other continence promoting strategies may need to be considered.

Location of stem cells for the enteric nervous system.

Hirschsprung disease is the result of aganglionosis of a variable length of the terminal bowel, which arises from the incomplete colonisation of the embryonic gut by vagal neural crest-derived cells (NCC) that migrate caudally from the pharyngeal gut to the rectum. We have previously shown that a very small group of NCC, at the leading edge of this wave of migration, can proliferate and differentiate to innervate the entire distal gut. It remains unknown if this capability is unique to those cells at the leading edge of NCC migration. The hypothesis tested was that NCC capable of acting as stem cells are found throughout the developing enteric nervous system (ENS). Gut was taken from mice at embryonic day 11.5 as the leading edge of NCC migration enters the colon. Terminal colon was separated as aganglionic recipient gut and its rostral end juxtaposed to the caudal end of the small intestine or caecum. The explants were cultured on nitrocellulose filters for up to 120 h, after which time the apposed segments had fused. The gut was then fixed and examined by immunohistochemistry to detect the neuronal markers PGP9.5 and nitric-oxide synthase (NOS) to assess development of enteric ganglia. NCC migrated from the proximal gut into the terminal colon, colonising it along its entire length. The pattern of NCC colonisation and differentiation of NOS-positive neurons was the same, regardless of whether the NCC were derived from the leading edge of migration in the caecum or from more proximal regions of the small intestine. Vagal NCC have the capacity to migrate into separated aganglionic terminal colon and differentiate into neurons. NCC at the leading edge of migration and those located more proximally within the gut demonstrate equivalent ability to migrate to and differentiate in the terminal rectum. Further studies are required to confirm which of these migrating NCC have the properties of ENS stem cells.

Double blind randomised controlled trial of topical glyceryl trinitrate in anal fissure.

AIMS: To determine the effectiveness and safety of topical glyceryl trinitrate (GTN) in the management of acute anal fissure in children. METHODS: Individual children were randomised to receive GTN paste or placebo for six weeks in addition to oral senna and lactulose. Patients took laxatives alone for a further 10 weeks. Each week a research nurse telephoned families to assess pain scores and give advice. Main outcome measures were validated standardised pain scores and time to painless defaecation. RESULTS: Forty subjects were recruited from 46 eligible children; 31 children completed the trial (13 in the GTN group and 18 in the placebo group). No differences in the proportion of those achieving pain free defaecation with relation to time were seen between the two groups. Similarly, there were no significant differences in pain scores between the two groups over the 16 week study period. However, in both groups pain scores had decreased significantly. There were no differences in the incidence of rectal bleeding, faecal soiling, presence of visible fissure, skin tag, or faecal loading at outpatient review at the time of recruitment, or at 6 weeks and 16 weeks. No serious adverse effects were observed. CONCLUSIONS: This study suggests that 0.2% GTN paste is ineffective in the treatment of acute anal fissures in childhood. However the overall fissure healing rate is high (84%) with associated reduction in pain scores, suggesting that a nurse based treatment programme can achieve a high rate of fissure healing.

Assessment of protein gene product 9.5 as a marker of neural crest-derived precursor cells in the developing enteric nervous system.

The neurons and glial cells of the enteric nervous system (ENS) are derived from the neural crest. To study the developmental events involved in congenital abnormalities of the ENS, it is essential to identify all neural-crest cells (NCC) in the prenatal gut. The low-affinity neurotrophin receptor p75 is currently considered to be a gold-standard marker, but because it is a membrane protein, it is lost during procedures that permeabilise cells that are necessary to identify intracellular components and in apoptosis and cell-proliferation assays. We have therefore assessed the potential of the intracellular neuronal marker protein gene product (PGP) 9.5 as a label for neural-crest-derived precursor cells during gut development. Gut was taken from mouse embryos at 11.5 days post-coitum, at which time NCC had reached the proximal colon. Cellular p75 and PGP9.5 expression was determined by double-labelling immunofluorescence. The leading edge of neural-crest migration was defined as the 10 most distal p75-labelled cells. The neuronal marker PGP9.5 labelled NCC as they migrated along the gut at day 11.5. At the leading edge of migration, over 95% of p75-positive cells also expressed PGP9.5, and all PGP9.5-positive cells were also labelled for p75. PGP9.5 is expressed by at least 95% of neural-crest-derived precursor cells at the leading edge of migration along the gut. Thus, it can be used as a robust marker for developing NCC in the gut.

Evolving experience in the management of empyema thoracis.

The optimal management of paediatric empyema thoracis remains controversial. The objective of the study was to analyse evolving experience in clinical presentation, management, outcome and factors contributing to adverse morbidity in thoracic empyema. Forty-seven patients presenting to a paediatric surgical centre were studied in three consecutive 6-y periods during 1980-97 to compare any change in the pattern of disease influencing diagnosis and management. Patients were categorized into two treatment groups: (i) conservative management (antibiotics and/or tube thoracostomy), (ii) thoracotomy. The median duration of illness prior to hospital admission was 10 d (range 1-42 d). Ultrasound was increasingly utilized in the diagnosis and staging of empyema and played an important role in directing definitive management. The presence of loculated pleural fluid determined the need for thoracotomy. Sixteen of 20 patients (80%) who were initially treated with thoracocentesis or tube thoracostomy eventually needed thoracotomy. There was a positive shift in management towards early thoracotomy resulting in prompt symptomatic recovery. Significant complications were noted in seven children who had delayed thoracotomy. These included recurrent empyema with lung abscess (n = 2), scoliosis (n = 2), restrictive lung disease (n = 1), bronchopleural fistula (n = 1) and sympathetic pericardial effusion (n = 1). An unfavourable experience with delayed thoracotomy during the study period has led us to adopt a more aggressive early operative approach to empyema thoracis. The decision to undertake thoracotomy has been influenced by the ultrasound findings of organized loculated pleural fluid. Delayed surgery was associated with adverse outcome. Whilst fibrinolytics and thoracoscopy may provide attractive options for early empyema, thoracotomy can hasten patient recovery regardless of the stage of disease. Prospective randomized trials are required to assess the ideal therapy for childhood empyema.

Reduced endothelin-3 expression in sporadic Hirschsprung disease.

BACKGROUND: Enteric aganglionosis in Hirschsprung disease has been linked to genes coding for endothelin-3 (EDN3) and the endothelin B receptor (EDNRB), but there is no such linkage in most patients with sporadic Hirschsprung disease. However, the similarity between the distal colonic aganglionosis in Hirschsprung disease and that due to EDN3 or EDNRB mutations led to the hypothesis that levels of expression of these genes might be affected in the absence of mutation, thus causing the Hirschsprung disease phenotype. The aim of this study was to determine EDN3 and EDNRB messenger RNA (mRNA) levels in tissue samples from patients with sporadic Hirschsprung disease. METHODS: RNA and DNA were isolated from the ganglionic and aganglionic colonic segments of ten children with sporadic Hirschsprung disease, and from the colon of ten age-matched controls. The DNA was analysed for mutations in the genes coding for endothelin-3 (ET-3) and endothelin B receptor (ET-B) proteins. Relative levels of EDN3 and EDNRB mRNA were determined by semi-quantitative transcriptase-polymerase chain reaction. RESULTS: Three children had sequence variants in EDN3 and EDNRB. In the remaining seven patients, EDN3 mRNA levels were reduced in both the ganglionic and aganglionic colon compared with levels in controls; there was no difference in expression of EDNRB between groups. CONCLUSION: In the absence of mutation, EDN3 is downregulated in short-segment Hirschsprung disease, suggesting that this may be a common step leading to aganglionosis.

Time-dependent effects of endothelin-3 on enteric nervous system development in an organ culture model of Hirschsprung's disease.

BACKGROUND/PURPOSE: Terminal colonic aganglionosis (Hirschsprung disease) results from incomplete rostrocaudal colonisation of the embryonic gut by neural crest cells (NCC). Mutations in the genes encoding endothelin-3 (EDN3) or its receptor (EDNRB) have been shown to result in a similar aganglionosis. This article describes the development of an organ culture model using embryonic murine gut to determine how endothelin-3 regulates development of the enteric nervous system. METHODS: Gut explants from mice of different gestational ages were cultured for up to 3 days in the presence or absence of 5 micromol/L of the specific endothelin-B receptor antagonist BQ788. EDN3 and EDNRB mRNA expression were analysed by reverse-transcription polymerase chain reaction (RT-PCR) and whole-mount in situ hybridisation. NCC were localised using immunoreactivity for PGP 9.5, a specific neuronal marker. RESULTS: EDN3 mRNA continued to be expressed by caecal mesenchymal cells and EDNRB mRNA by the migrating NCC in culture. Embryonic day (E)11.5 explants were already colonised by NCC up to the terminal ileum. Complete colonisation occurred in organ culture over the next 72 hours (equivalent to E 14.5). Explants of E 12.5 and E 13.5 showed complete colonisation after 48 and 24 hours culture, respectively. Terminal aganglionosis resulted from treatment of E 11.5 and E 12.5 gut explants with 5 micromol/L BQ788, whereas there was no inhibitory effect on E 13.5 explants. CONCLUSIONS: An organ culture model has been developed in which NCC colonisation of embryonic gut mirrors that described in vivo. Blockade of the EDN3/EDNRB receptor pathway shows that the interaction of endothelin-3 with its receptor is only necessary for NCC colonisation at early time-points, despite the continued expression of endothelin-3 mRNA in the gut.

Ontogeny of interstitial cells of Cajal in the human intestine.

BACKGROUND/PURPOSE: Interstitial cells of Cajal (ICC) recently have been identified as intestinal pacemaker cells. Abnormalities in ICC are increasingly recognized in a number of neonatal disorders such as infantile hypertrophic pyloric stenosis, Hirschsprung's disease, and transient intestinal pseudo-obstruction. The aim of this study was to determine the fetal and postnatal differentiation and development of ICC in the human gastrointestinal tract to aid interpretation of pathological specimens. METHODS: Specimens of human gastrointestinal tract from (1) fetuses (9 to 17 weeks' gestation; n = 12), (2) premature and full-term neonates with non-gut motility-related disorders, (age 26 to 59 weeks' gestation; n = 13), and (3) children (age 4 months to 13 years; n = 7) were immunohistochemically stained with antibodies to c-kit(a marker for ICC) and protein gene product 9.5 (PGP9.5, a marker for neural tissue). RESULTS: (1) C-kit-positive ICC were present throughout the gut in all specimens including those from the earliest gestational ages. C-kit and PGP9.5 immunoreactivities were present in different cell populations. (2) The distribution of ICC varied with gestational age and with region of the gut. (3) Maturation of ICC networks continues postnatally in a region-specific manner. CONCLUSIONS: ICC are present from an early stage in human gut development. Interpretation of apparent abnormalities in ICC distribution as being of pathological significance should be tempered by the knowledge that ICC networks continue to develop postnatally and that ICC development varies throughout the gut.

Long-term outcome and colonic motility after the Duhamel procedure for Hirschsprung's disease.

BACKGROUND/PURPOSE: The aim of this study was to investigate long-term functional outcome and colonic motility in children who had undergone the Duhamel-type operation for Hirschsprung's disease (HSCR). METHODS: All patients (n = 91) who underwent the Duhamel or Lester Martin Modified Duhamel operation for HSCR from 1980 to 1991 were included in the study. Twenty-two healthy age-matched children were used as controls. Functional outcome was assessed by questionnaire (response rate 100%). Total and segmental colonic transit time (CTT) was determined using the saturation method (80% participation rate). RESULTS: Outcome scores were significantly worse in the study group for patients with rectosigmoid (RS, P < .001), long segment (LS, P < .001), and total colonic (TC) aganglionosis (P < .05), when compared with controls. The CTT was significantly prolonged in the RS group (P = .01) compared with LS, TC, and control groups; this was caused by prolonged "rectosigmoid" transit in the RS group compared with controls (P = .012). There was a positive linear correlation (P = .0002) between age and outcome score in patients with RS disease unrelated to CTT. Nine patients required a late long-term enterostomy. A satisfactory outcome (defined as outcome score > or = 10th percentile of the control group, and absence of stoma or requirement for major revisional surgery) was seen in only 42% of patients overall and in 79% of patients over 14 years of age. CONCLUSIONS: The Duhamel procedure, in common with other pull-through procedures, is associated with significant long-term morbidity, the aetiology of which is poorly understood.

Expression of endothelin 3 by mesenchymal cells of embryonic mouse caecum.

BACKGROUND: Mutations in endothelin 3 (EDN3) and endothelin B receptor (EDNRB) genes cause terminal colonic aganglionosis in mice, and mutations in these genes have also been linked to the terminal aganglionosis seen in human Hirschsprung's disease. However, details of EDN3 expression during embryogenesis are lacking, and consequently the cellular mechanism by which EDN3 regulates innervation of the terminal gut is unclear. AIMS: To localise the expression of EDN3 and EDNRB in the embryonic mouse gut. METHODS: Expression of EDN3 and EDNRB mRNA was analysed by reverse transcription polymerase chain reaction and in situ hybridisation. RESULTS: High levels of EDN3 mRNA expression were restricted to mesenchymal cells of the caecum before and after the arrival of neural crest cells. In contrast, EDNRB expression along the gut displayed a time dependent pattern similar to those of the protein tyrosine kinase ret and the neural crest cell marker PGP9.5. CONCLUSIONS: Mesenchymal cells of the caecum express high levels of EDN3 mRNA during embryogenesis and hence the production of EDN3 at the caecum is likely to act on neural crest cells as a paracrine factor necessary for subsequent innervation of the terminal gut.

Functional results following the antegrade continence enema procedure.

BACKGROUND: Forty children who underwent the antegrade continence enema (ACE) procedure for faecal soiling were studied to determine factors predictive of outcome. METHODS: There were four patient groups: (1) ambulant with spinal dysraphism (n = 13), (2) wheelchair bound with spinal dysraphism (n = 14), (3) ambulant with miscellaneous disorders (n = 11) and (4) wheelchair bound with miscellaneous disorders (n = 2). Effectiveness of the procedure was assessed using technical evaluation and quality-of-life improvement (QOLI) scores (0-5). Objective assessment included colonic transit time (CTT) and anorectal manometry. Median follow-up was 21 (range 5-37) months. RESULTS: Some 28 of 40 children achieved continence. The procedure was reversed in four of 40 children. Of the other 36 children with a functioning ACE stoma, all reported improvement in quality of life (mean QOLI score 3.5). There were no significant differences in technical evaluation score, QOLI score, CTT, manometry findings or continence between ambulant groups and the wheelchair-bound group with miscellaneous disorders. QOLI score, anorectal squeeze pressure and continence were significantly poorer in those who were wheelchair bound with spinal dysraphism. Absent squeeze pressure was associated with poor outcome. CONCLUSION: Wheelchair-bound children with spinal neuropathy have a poorer outcome following the ACE procedure. Although ACE is an effective method of promoting faecal continence, it is essential to determine the aetiology of incontinence and sphincter function before operation.