Taiwo A. Lawal

The appendix as a conduit for antegrade continence enemas in patients with anorectal malformations: lessons learned from 163 cases treated over 18 years

INTRODUCTION The antegrade continence enema (ACE) has been shown to be a safe and effective method for managing fecal incontinence in the pediatric population. The purpose of this study was to examine our experience with the ACE procedure using the appendix as a catheterizable conduit in children with anorectal malformations (ARMs). METHODS We reviewed the charts of all patients who underwent an ACE procedure using the appendix as a catheterizable conduit between January 1992 and January 2010. Preoperative diagnosis (ARM type), operative details, functional outcomes, and postoperative complications were assessed. Technical modifications over time included selective cecoplication, implementation of the umbilical V-V appendicoplasty technique, and laparoscopy for cecal mobilization. RESULTS Mean age was 9.9 ± 0.6 years, and 67% were male. The most common preoperative diagnosis was rectourethral fistula in boys (39%) and persistent cloaca in girls (61%). Forty-five complications occurred in 41 patients with an overall incidence of 25.6% (stricture, 18%; leakage, 6%; prolapse, 4%; intestinal obstruction, 0.6%). The incidence of stomal leakage was lower in patients when a cecoplication was performed (2.9% [4/138] vs 29.4% [5/17]; P < .01), and the incidence of stricture was lower in patients when the umbilical anastomosis was created using the V-V appendicoplasty technique (11% [11/100] vs 30% [18/60]; P < .01). Successful management of incontinence was reported by 96% of all patients. CONCLUSIONS The ACE procedure using the umbilical V-V appendicoplasty provides an effective and cosmetically superior means for bowel management in children with ARMs. The rate of late complications is not insignificant however, and preventative strategies should focus on careful operative technique and ensuring compliance with catheterization protocols well past the initial postoperative period.

Reoperation for Hirschsprung Disease: Pathology of the Resected Problematic Distal Pull-Through

Hirschsprung disease, which consists of aganglionosis of the rectum and sometimes more proximal bowel, requires surgical removal of the aganglionic bowel and creation of ganglionated neorectum using proximal normally innervated bowel. The border between aganglionic and ganglionic bowel is irregular; the transition zone features variable quantities of ganglion cells and numerous large nerves. We report the histopathology of pull-through bowel segments resected because of poor postoperative outcome from 30 patients (22 boys, 8 girls). The most common indication for reoperation was severe constipation/obstruction. Transition zone (bowel with at least two nerves ≥40 μm diameter per 400× high-power field, and ganglion cells) or aganglionic bowel (bowel with at least two nerves ≥40 μm per high-power field diameter, but without ganglion cells) was found in 19/30 (63%) resections. In colons resected because of familial adenomatous polyposis, rare high-power fields showed two enlarged nerves; the mean age of those patients (135 ± 49.4 months) was significantly higher than that of the patients undergoing redo pull-through surgery (67.9 ± 42.8 months). Additional pathology included stricture and enterocolitis. Although there are multiple causes for poor outcomes following surgical therapy for Hirschsprung disease, abnormal innervation of the bowel used for pull-through is common. We recommend that intraoperative consultation at primary pull-through procedure include frozen section evaluation of the circumference of the bowel to be used for pull-through to confirm histologically the presence of both ganglion cells and normal-caliber nerves. The criteria used in this study are most suitable for infants and young children.

Acquired posterior urethral diverticulum following surgery for anorectal malformations.

PURPOSE Despite significant advances in the surgical management of anorectal malformations (ARMs), many children still experience significant debilities from potentially avoidable complications. One complication, the posterior urethral diverticulum, may have untoward consequences if not recognized and treated. METHODS A retrospective cohort review was undertaken of male patients who presented to us with persistent problems after being operated on elsewhere for ARM. Twenty-nine patients presented with a urethral diverticulum. Their charts were reviewed for the type of malformation, prior repair, presentation, treatment, and postoperative follow-up. RESULTS Twenty-nine patients were identified that fit the criteria for this study. To date, 28 patients have been managed with reoperation. Urinary complaints were the most common presenting symptoms. All patients were repaired using a posterior sagittal approach. Pathology of the diverticulum in one patient revealed a well-differentiated mucinous adenocarcinoma. CONCLUSION The incidence of acquired posterior urethral diverticulum has decreased with the popularization of the posterior sagittal incision. There is a theoretical concern that the incidence may increase with the use of laparoscopy for the treatment of ARMs especially those where the fistula is below the peritoneal reflection. Once detected, the diverticulum should be excised.

Laparoscopic-Assisted Malone Appendicostomy in the Management of Fecal Incontinence in Children

INTRODUCTION The antegrade continence enema procedure offers convenient enema administration for patients with fecal incontinence and can be performed by a minimally invasive approach that provides shorter hospital stay, less analgesia requirement, and better cosmesis. We present our experience using this approach for fecal incontinence patients and technical modifications to reduce complications. METHODS Following successful management of fecal incontinence through bowel management using a daily rectal enema, 44 patients underwent a laparoscopic-assisted Malone appendicostomy procedure. We reviewed the diagnosis underlying the fecal incontinence, operative technique, duration of surgery, length of hospital stay, and postoperative complications. RESULTS The mean age at surgery was 8.6 ± 1.0 years. The diagnoses included anorectal malformations (31), idiopathic constipation (6), Hirschsprung disease (3), and others (4). All the patients underwent a V-V umbilico-appendicoplasty. The cecum was plicated around the base of the appendix in 34 patients (77%); this step was omitted in 10 (23%). The median follow-up was 21 months (range: 3-51 months). Twelve complications were recorded in 9 patients (20.5%)--leakage from the stoma in 1 (2.3%), stomal stenosis in 5 (11.4%), and a combination of both in 3 (6.8%)--with an overall stricture rate of 18.2% (8/44) and leakage rate of 9.1% (4/44). Strictures were managed with minor operative revision. Plicating the cecum was associated with a lower leakage rate compared with the nonplicated group (0/34, 0% versus 4/10, 40%; P = .002). All patients were consistently clean between enemas following their antegrade continence enema procedure. CONCLUSIONS The umbilical appendicostomy provides a convenient and cosmetic location for enema administration. Cecal plication, which is feasible using a laparoscopic-assisted approach, significantly reduces the leakage rate. Stomal stenoses remains a problem, may be lessened by a V-to-V umbilical to appendix anastomosis, and are easily fixed with a revision.

The utility of routine pouchogram before ileostomy reversal in children and adolescents following ileal pouch anal anastomosis

INTRODUCTION Pouchograms are used to assess the integrity of the ileal pouch anal anastomosis (IPAA) in patients who have undergone restorative proctocolectomy. Its benefits have been questioned, and there are no data to support the routine use in children. METHODS We retrospectively reviewed the charts of 26 patients who had an IPAA and pouchogram at our institution between 2001 and 2009. Each patient also underwent an examination under anesthesia to assess the integrity of the IPAA on the day of the ileostomy closure. RESULTS The mean age of the patients was 13.8 (± 0.7) years. The pouchogram was performed at a median of 6 weeks after the IPAA (range, 4-20 weeks). The findings were normal in 26 (89.7%) and demonstrated stricture in 2 (6.9%) and leak in 1 (3.4%). History was suggestive and physical examination was confirmatory in these 3 problematic cases. CONCLUSIONS A contrast enema is not routinely required to evaluate the integrity of the IPAA before ileostomy reversal in pediatric patients. Complications can be detected by history and rectal examination before ileostomy closure. We recommend the use of contrast enema only in symptomatic patients where a leak is suspected, thereby limiting radiation exposure and inconvenience.

The Transanal Approach with Laparoscopy or Laparotomy for the Treatment of Rectal Strictures in Crohn's Disease

INTRODUCTION Rectal strictures in patients with Crohns colitis are common and options described for their treatment include direct steroid injection, injection of tumor necrosis factor (TNF) inhibitors, endoscopic balloon dilatation, use of Hegar dilators, stricturoplasty, and proctectomy. Adequate treatment is a challenge, especially with respect to the prevention of stricture recurrence. We present an option for the surgical treatment of these strictures using a transanal resection of the rectum with the addition of laparoscopy or laparotomy. METHODS Three patients who had medically refractory or chronic Crohns colitis with rectal strictures were referred to us after failed medical management, rectal dilation, and balloon dilation of the strictures. In each case, we performed a transanal sphincter preserving dissection in the prone position and used the lithotomy position for intraabdominal mobilization, completion of the rectosigmoid resection, pull-through of the left colon, and coloanal anastomosis. RESULTS We resected the rectal strictures transanally in all three cases. One case provided the opportunity to perform a laparoscopy-assisted procedure, whereas the other 2 patients had laparotomy-assisted rectosigmoid resections. We did a coloanal anastomosis in 2 patients with healthy left colon. In the third case, the anal canal was preserved, but the patient was left with a stoma. CONCLUSIONS Transanal resection is feasible in the surgical treatment of rectal strictures in patients with Crohns colitis. It preserves the anal sphincteric mechanism and may help in avoiding a permanent stoma in a subgroup of patients. We found the prone position very helpful in performing the transanal rectal dissection.