Primary pull-through for Hirschsprung's disease: comparison of open and laparoscopic-assisted procedures.

Rectosigmoid Hirschsprung's disease is usually amenable to minimally invasive primary neonatal pull-through. This may be performed either entirely transanally or with laparoscopic assistance for biopsies with or without colonic mobilization. In our center, all dissection is performed transanally; laparoscopy is used for obtaining colonic biopsies and orientation of the pulled-through bowel segment. In this paper, we describe our initial experience of a consecutive cohort of 20 one-stage laparoscopic-assisted endorectal pull-through (LAEPT) procedures. A historic consecutive cohort of 22 infants who underwent the same open endorectal pull-through (OPT) with open transabdominal mobilization was used for comparison. Age at operation and mean theater time were not significantly different. The mean postoperative stay was significantly reduced in the laparoscopic group (LAEPT 3.8 days vs. OPT 9.5 days; P = 0.0002). Readmission and enterocolitis rates in the first postoperative year did not differ significantly. LAEPT permits early intraoperative biopsies with a visualization of the pull-through to prevent twisting of the bowel.

After the honeymoon comes divorce: long-term use of the antegrade continence enema procedure.

BACKGROUND: Having reported that 18% of children discontinue use of the antegrade continence enema (ACE) after 5 years, we aimed to determine long-term use after an ACE procedure. METHODS: A postal/telephone questionnaire was conducted. Subjects were consecutive children undergoing an ACE between 1993 and 1999. Outcome measures were use of ACE, reasons for nonuse, complications, and overall satisfaction. RESULTS: Of 84 eligible subjects, data were available on 61 (73%) aged 22.4 years (15.5-35.1 years). Underlying diagnoses included spina bifida (n = 27), anorectal malformations (n = 18), constipation (n = 11), Hirschsprung's disease (n = 1), sacral agenesis (n = 2), and trauma/tumor (n = 2). Follow-up was 11.02 years (8.34-14.39 years). Thirty-six (59%) of 61 patients were still using their ACE. Reasons for nonuse were lack of effectiveness (n = 14), complications (n = 5), psychologic issues (n = 2), and poor compliance (n = 2). There was no association between diagnosis and nonuse (chi(2), P = .63). In those still using ACE, the overall satisfaction score was 4.1 (1-5). Several individuals reported feeling abandoned on becoming adults and losing the support they had in childhood. CONCLUSION: There is a late "failure" rate for the ACE procedure. However, satisfaction was high among those still using the ACE. This study further emphasizes the need for robust transitional care arrangements.

Gastroschisis associated with cleft lip and palate.

Gastroschisis is a common congenital abdominal wall defect. Rarely, it is associated with extraabdominal midline congenital anomalies. Oral clefts are the commonest craniofacial congenital midline defects. They can be associated with other midline defects like omphalocele. We believe these are the first 3 case reports of gastroschisis and cleft palate occurring in the same patient.

BAPS UK inflammatory bowel disease surgical practice survey.

AIMS: A recent survey of children with inflammatory bowel disease (IBD) identified wide regional variations of care within the UK. The present study was designed to analyse paediatric surgical provision for children with ulcerative colitis and Crohn's disease. METHODS: All UK paediatric surgical centres were contacted to identify surgeons with a subspecialist interest in IBD. A questionnaire was designed to probe specific areas including team working, caseload, and transitional care. Annual consultant caseload was requested for colonoscopy, J-pouch ileoanal anastomosis (IPAA) for ulcerative colitis, and strictureplasty (Crohn's disease). The questionnaire and the accompanying letter were approved by the BAPS Research and Clinical Effectiveness Committee. RESULTS: The response rate from individual centres was 86% (25/29). In 11% of centres, care was shared between 2 consultants. A transitional care clinic was provided by 77% of centres. The median experience with IPAA was 0.9 cases per year of consultant practice (range, 0-3.7), and 12.5% of surgeons had limited experience of revision pouch surgery. The majority have arrangements for joint operating with adult surgeons for IPAA. Forty percent of surgeons reported experience with strictureplasty. Surgical preference for recalcitrant left-side Crohn's colitis favoured segmental resection (60%), compared to subtotal/panproctocolectomy. CONCLUSIONS: Paediatric surgeons use a diversity of surgical management options in IBD. Experience with IPAA is limited for most surgeons. Whether children should undergo elective IPAA independent of experienced adult practitioners, who naturally assume responsibility after transition, requires careful debate.

Early adult outcome of the Duhamel procedure for left-sided Hirschsprung disease--a prospective serial assessment study.

PURPOSE: To assess both early adult functional outcome and change in long-term functional outcome over time after the Duhamel procedure (DP) for left-sided Hirschsprung disease (HSCR). METHODS: The study population consisted of 78 children (aged 19.9 +/- 3.6 years) who previously underwent objective outcome assessment after DP was performed for HSCR during the period of 1980 to 1991. Inclusion criteria were previous evaluation of functional outcome and either rectosigmoid or left-sided HSCR. Outcome measures were assessed twice within the cohort, in 1997 and in 2005. The primary outcome measure was the Rintala (J Ped Surg. 1995;30:491-494) functional outcome score (FOS; maximum, 20). Controls consisted of 20 age-matched healthy children. Satisfactory functional score was defined as an FOS at or above the 10th percentile of controls (FOS, > or = 17). Secondary outcome measures were the operation failure rate (defined by requirement for a stoma or major reoperative surgery), and enterocolitis rates (defined by intention to treat). Consecutive outcome scores were compared by paired t test. Data were expressed as mean +/- SD, and P < .05 was considered significant. RESULTS: Operation failure occurred in 9 (11.5%) of 78. Consecutive FOSs were obtained in 40 (57%) of 69. A satisfactory functional score was observed in 23 (58%) of 40 adults as opposed to 33 (47%) of 70 children 8 years previously (P = .02). Satisfactory outcome (defined by satisfactory functional score and lack of enterostomy or major revision pull-through procedure) was observed in 23 (47%) of 49. Previously, this figure was 34 (44%) of 78. Individual paired FOSs showed a significant improvement with time (1997: 14.9 +/- 4.1; 2005: 16.4 +/- 2.8; P = .02). CONCLUSIONS: At early adult follow-up, the operation failure rate has not changed from that of the same cohort 8 years earlier. However, a significant improvement in individual FOSs was demonstrated.

Functional outcome and quality of life in anorectal malformations.

BACKGROUND/PURPOSE: The aim of this study was to assess the early functional outcome and quality of life (QOL) in children with anorectal malformations. METHODS: Children treated for anorectal malformations (ARMs) from 1994 to 2000 were evaluated if 4 years or older. Primary outcome measures were bowel function score, assessed by functional outcome questionnaire, and QOL using the Pediatric Quality of Life Inventory (PedsQL 4). The secondary outcome measure was age at potty training. Twenty healthy children were used as controls for functional outcome and age at potty training. Data are reported as mean (SD) unless otherwise stated. RESULTS: Eighty children were evaluated during the study period. The mean age at follow-up was 82 months (18.7). The response rate was 76.3% (58/76) for bowel function and 77.5% (62/80) for QOL questionnaires. Functional outcome score (maximum 20) decreased significantly with increasing severity of the ARM (male perineal fistula, 16 [3]; female perineal fistula, 15 [3]; rectourethral fistula, 12 [4]; vestibular fistula, 13 [3.5]; bladder neck fistula, 6 [2]; analysis of variance, P = .001). However, there was no difference in QOL between patients with ARM and controls. There was no correlation between age and either bowel function score (Pearson r2 = 0.06) or QOL (Pearson r(2) = 0.12). Affected children took significantly longer to achieve potty training for bladder (35 [13.8] months vs 26 [8.7] months for controls [t test, P = .005]) and bowels (38 [16] months vs 25 [7] months [t test, P = .001]). CONCLUSION: Children with ARMs have significantly worse bowel function than their peers, depending on the type of lesion. Despite these findings, QOL was not significantly impaired. No correlation was demonstrated between age and either functional outcome or QOL.