Role of Routine Dilatations after Anorectal Reconstruction-Comparison of Two Tertiary Centers.

Aim:  Regular anal dilatations are commonly recommended in the postoperative management following posterior sagittal anorectoplasty (PSARP) in anorectal malformations (ARM). We hypothesized that routine postoperative dilatations may not affect surgical outcomes following PSARP. We compare surgical outcomes of routine postoperative dilatations versus no routine postoperative dilatations from two United Kingdom tertiary pediatric surgical centers.

Malrotation: Age-Related Differences in Reoperation Rate.

Objective: Intestinal malrotation classically presents in the neonatal period with bilious vomiting. However, population studies suggest that up to two-thirds of these patients are diagnosed later in childhood or in adulthood. Increased morbidity in the adult population has been reported.

Kabuki Syndrome and Anorectal Malformations: Implications for Diagnosis and Treatment.

Kabuki syndrome (KS) is a rare genetic condition characterized by a distinctive facies, intellectual disability, growth delay, and a variety of skeletal, visceral, and other anomalies, including anorectal malformations (ARMs). We present two cases of female patients with KS, diagnosed and successfully managed at our institution, one with a perineal fistula and one with a rectovestibular fistula. Our report, along with a literature review, shows that the syndrome is usually associated with "low" anomalies, with a potential for a good prognosis.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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).