Surgical strategies in paediatric inflammatory bowel disease.

Inflammatory bowel disease (IBD) comprises two distinct but related chronic relapsing inflammatory conditions affecting different parts of the gastrointestinal tract. Crohn's disease is characterised by a patchy transmural inflammation affecting both small and large bowel segments with several distinct phenotypic presentations. Ulcerative colitis classically presents as mucosal inflammation of the rectosigmoid (distal colitis), variably extending in a contiguous manner more proximally through the colon but not beyond the caecum (pancolitis). This article highlights aspects of the presentation, diagnosis, and management of IBD that have relevance for paediatric practice with particular emphasis on surgical considerations. Since 25% of IBD cases present in childhood or teenage years, the unique considerations and challenges of paediatric management should be widely appreciated. Conversely, we argue that the organizational separation of the paediatric and adult healthcare worlds has often resulted in late adoption of new approaches particularly in paediatric surgical practice.

Gastrointestinal surgery in cystic fibrosis: a 20-year review.

OBJECTIVES: The purpose of this study was to evaluate outcomes of the surgical management for meconium ileus (MI) and Distal Intestinal Obstruction Syndrome (DIOS) in Cystic Fibrosis (CF). METHODS: Children born between 1990 and 2010 were identified using a regional CF database. Retrospective case note analysis was performed. Outcome measures for MI were mortality, relaparotomy rate, length of stay (LOS), time on parental nutrition (TP), and time to full feeds (TFF). Outcome measures for DIOS were: age of onset, number of episodes, and need for laparotomy. RESULTS: Seventy-five of 376 neonates presented with MI. Fifty-four (92%) required laparotomy. Contrast enema decompression was attempted in nineteen. There were no post-operative deaths. Thirty-nine (72%) neonates with MI were managed with stomas. LOS was longer in those managed with stomas (p=0.001) and in complex MI (p=0.002). Thirty-five patients were treated for DIOS. Twenty-five patients were managed with gastrograffin. Ten patients underwent surgical management of DIOS. Overall, MI did not predispose to later development of DIOS. There was a significantly greater incidence of laparotomy for DIOS in children who had MI. CONCLUSION: The proportion of neonates with complex meconium ileus was high (49%) and may explain the infrequent utilisation of radiological decompression. Complex MI or management with stomas both significantly increase LOS. Re-laparotomy rate is high (22%) in MI irrespective of the type of management. DIOS is not a benign condition, particularly when the child has had previous abdominal surgery. Early referral to a surgical team is essential in these children.

Potential benefits of fast-track concepts in paediatric colorectal surgery.

BACKGROUND: Structured care pathways optimising peri-operative care have been shown to significantly enhance post-operative recovery. We aim to determine if enhanced recovery after surgery (ERAS) principles could provide benefit for paediatric patients undergoing major colorectal resection for inflammatory bowel disease (IBD). METHODS: Children undergoing elective bowel resection for IBD at a regional paediatric unit using standard methods of peri-operative care were matched to adult cases from an associated tertiary referral university hospital already using an ERAS program. Cases were matched for disease type, gender, operative procedure, and ASA grade. RESULTS: Forty-four children undergoing fifty procedures were identified. Thirty-four were matched to adult cases. Total length of stay in the paediatric group was significantly longer than in the adult group (6 vs. 9 days; P=0.001). Paediatric patients were slower to start solid diet (1 vs. 4 days; P<0.0001) and were slower to mobilize post-operatively (1 vs. 4 days; P<0.0001). No difference was seen in time to restoration of bowel function (2 vs. 3 days; P=0.49). Thirty day readmissions and total in-hospital morbidity were not significantly different between the groups. CONCLUSION: Potentially, application of ERAS in paediatric surgery could accelerate recovery and reduce length of post-operative stay thereby improving quality and efficiency of care.

Comparison of childhood appendicitis management in the regional paediatric surgery unit and the district general hospital.

BACKGROUND/PURPOSE: Ongoing debate surrounds the future provision of general paediatric surgery. The aim of this study was to compare outcomes for childhood appendicitis managed in a district general hospital (DGH) and a regional paediatric surgical unit (RU). METHODS: Data collected retrospectively for a 2-year period in a DGH were compared with data collected prospectively for 1 year in an RU, where appendicitis management is guided by a care pathway. Children aged 6 to 15 years were included. RESULTS: Four hundred and two patients were included (DGH ,196; RU, 206). There were more cases of gangrenous/perforated appendicitis in the RU (P < .0001). In the DGH, fewer patients received preoperative antibiotics (P < .0001) or underwent preoperative pain scoring (P < .0001). When adjusted for case mix, the relative risk of complications for a child managed at the DGH was 1.76 (95% confidence interval, 1.44-2.16; P < .0001) and that of readmission was 1.76 (95% confidence interval, 1.43-2.16; P < .0001) when compared with the RU. CONCLUSIONS: Patients with appendicitis managed in the DGH had a higher risk of complications and readmission. However, this appears to be related to the use of a care pathway at the RU. Introduction of a care pathway in the DGH may improve outcomes and thus support the ongoing provision of general paediatric surgery.

Vacuum drainage in the management of complicated abdominal wound dehiscence in children.

PURPOSE: The aim of the study was to report the outcomes of the vacuum dressing method (vacuum-assisted closure [VAC]) in the management of "complicated" abdominal wounds in a selected group of children including neonates. METHODS: All children with vacuum (VAC) dressing-assisted closure of a complex abdominal wound (defined as complete/partial wound dehiscence combined with at least one of stoma, anastomosis, tube enterostomy, or infected patch abdominoplasty) were included in a 2-year study that took place in a single tertiary referral hospital. Retrospective case note analysis was used to determine premorbid diagnosis, management, illness severity markers, morbidity, and outcome. RESULTS: Nine children (neonate to 16 years) required 11 continuous episodes of VAC therapy. Abdominal wall dehiscence was complete in 7 and partial in 4 episodes. These were complicated by stomas (8), anastomoses (3), enterocutaneous fistulae (3), tube enterostomy (1), and infected patch abdominoplasty (2). Illness severity was assessed by the following proxy physiologic markers: American Society of Anesthesiologists status 3 or more (10), intensive care unit (ICU) (7), inotropes (4), ventilation (7), septic (C-reactive protein >100 and blood culture-positive) (3), liver impairment (aspartate transaminase >58 and alanine transaminase >36) (4), coagulopathy (international normalized ratio >1.3) (4), proinflammatory state (platelet count >450) (5), and nutritional impairment (albumin <37) (9). The median VAC treatment time was 32 days (range, 9-101 days). Of the changes, 70% required a general anesthetic or sedation on ICU. Control of 10 of 11 complex abdominal wounds (including 3 established enterocutaneous fistulae) was achieved using VAC therapy. Complications included nonreduction of laparostomy (1), failure of anastomosis (1), and failure of tube enterostomy diversion (1). Four children died of unrelated causes, 2 of them more than 3 months after VAC therapy. CONCLUSIONS: In our experience with a small series of patients, VAC therapy is both safe and effective in complex pediatric abdominal wounds in severely ill children. It appears to promote wound closure, controls local sepsis, and can be used to manage established fistulae. However, our results suggest that recent bowel anastomoses may be compromised using VAC, which in this circumstance, should be used with caution.

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.

Diagnosis, incidence, and outcomes of suspected typhlitis in oncology patients--experience in a tertiary pediatric surgical center in the United Kingdom.

BACKGROUND: Typhlitis is clinically defined by the triad of neutropenia, abdominal pain, and fever. Radiologic evidence of colonic inflammation supports the diagnosis. We report a single United Kingdom tertiary center experience with management and outcome of typhlitis for 5 years. METHODS: Hospital computerized records were screened for ultrasound or computerized tomographic scan requests for abdominal pain for all oncology inpatients (2001-2005). Retrospective case note analysis was used to collect clinical data for patients with features of typhlitis. RESULTS: The incidence of typhlitis among oncology inpatients was 6.7% (40/596) among oncology inpatients and 11.6% (40/345) among those on chemotherapy. Eighteen children had radiologically confirmed typhlitis, and 22 had clinical features alone. Most (93%) patients responded to conservative management. Eighteen children had a variable period of bowel rest, including 12 patients who were supported with total parenteral nutrition. Three patients had laparotomy that revealed extensive colonic bowel necrosis (1), perforated gastric ulcer (1), and a perforated appendix (1). A single child died of fulminant gram-negative sepsis without surgical intervention. CONCLUSIONS: The diagnosis of typhlitis was based on clinical features, supported by radiologic evidence in almost half of the study group. Surgical intervention should be reserved for specific complications or where another surgical pathologic condition cannot reasonably be ruled out.

Negative exploration for pyloric stenosis--is it preventable?

BACKGROUND: The diagnosis of infantile hypertrophic pyloric stenosis (IHPS), although traditionally clinical, is now increasingly dependent on radiological corroboration. The rate of negative exploration in IHPS has been reported as 4%. The purpose of our study was to look at elements of supportive clinical evidence leading to positive diagnosis, and to review these with respect to misdiagnosed cases undergoing negative exploration. METHODS: All infants undergoing surgical exploration for IHPS between January 2000 and December 2004 were retrospectively analysed with regard to clinical symptoms, examination findings, investigations and operative findings. RESULTS: During the study period, 343 explorations were performed with a presumptive diagnosis of IHPS. Of these, 205 infants (60%) had a positive test feed, 269 (78%) had a positive ultrasound scan and 175 (55%) were alkalotic (pH >or=7.45 and/or base excess >or=2.5). The positive predictive value for an ultrasound (US) diagnosis was 99.1% for canal length >or=14 mm, and 98.7% for muscle thickness >or=4 mm. Four infants (1.1%) underwent a negative surgical exploration; Ultrasound was positive in 3, and negative in 1(who underwent surgery on the basis of a positive upper GI contrast). One US reported as positive had a muscle thickness <4 mm. Two false positive US were performed at peripheral hospitals. One infant had a false positive test feed following a positive ultrasound diagnosis. Two infants had negative test feeds. CONCLUSION: A 1% rate of negative exploration in IHPS compares favourably with other studies. However potential causes of error were identified in all 4 cases. Confident diagnosis comprises a combination of positive test feed and an 'in house US' in an alkalotic infant. UGI contrast study should not be used in isolation to diagnose IHPS. If the test feed is negative, strict diagnostic measurements should be observed on US and the pyloric 'tumour' palpated on table under anaesthetic before exploration.

It is not what you do, it is the way that you do it: impact of a care pathway for appendicitis.

BACKGROUND/PURPOSE: Appendicitis is the most common surgical emergency in children. However, management varies widely. The aim of this study was to assess the impact of introducing a care pathway on the management of childhood appendicitis. METHODS: Data were collected prospectively for 3 successive cohorts: All patients operated for suspected appendicitis were included. The pathway was modified after interim analysis of group B data. P < .05 was significant. RESULTS: Six hundred patients were included. When compared with group A, group C patients were more likely to receive preoperative antibiotics (P < .0001), undergo formal pain assessment (P < .0001), and be operated before midnight (P = .025). There was a significant decrease in readmission rates from 10.0% to 4.2% (P = .023) despite an increase in cases of gangrenous and perforated appendicitis (P = .010). CONCLUSIONS: The introduction of a care pathway resulted in improved compliance with antibiotic regimens, more frequent pain assessment, and fewer post-midnight operations. Postappendicectomy readmission rates were reduced despite an increase in disease severity. This was achieved by critical reevaluation of outcomes and pathway redesign where appropriate.

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.

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.

Umbilical pyloromyotomy: comparison of vertical linea alba and transverse muscle cutting incisions.

BACKGROUND: Pyloromyotomy is commonly performed through a supraumbilical skinfold incision. Entry into the peritoneal cavity can be achieved via a vertical linea alba incision or a transverse muscle cutting approach. The aim of this study was to compare the morbidity associated with these 2 operative techniques. METHODS: Case records of all babies undergoing umbilical pyloromyotomy between January 2000 and December 2004 were retrospectively compared for postoperative dehiscence, mucosal perforation, and wound infection (defined by the need for antibiotics or wound exploration). Data were analyzed with GraphPad Prism contingency tables and results were compared by Fisher exact test (P < .05). RESULTS: During the study period, 341 umbilical pyloromyotomies were performed at our institution. The surgeon was permitted choice of either operative approach (219 vertical linea alba, and 122 transverse muscle cutting). There were no significant differences between the 2 groups regarding age at presentation, sex, duration of symptoms, biochemical derangement, and operator seniority. No significant differences in morbidity were encountered with either of these 2 operative strategies. CONCLUSIONS: This study demonstrates that the vertical linea alba and transverse muscle cutting incisions have equivalent postoperative morbidity. These findings indicate that neither technique is demonstrably superior.

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.

Anorectal malformation with tubular hindgut duplication.

The association of hindgut duplication and anorectal malformation is rare. Published classifications of this association are confusing in respect of terminology. We report a case of blind-ending, Y-shaped tubular duplication of the distal hindgut, associated with an anorectal malformation (rectourethral fistula) affecting the colon proper. Surgical options at time of presentation and of reconstructive surgery are discussed. A review and suggested modification of the classifications is presented.

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.