Andrea Bischoff

Treatment of fecal incontinence with a comprehensive bowel management program

PURPOSE Many articles describe the antegrade continence enemas (ACEs), but few refer to a bowel management program. A successful ACE may not help a patient without such management. Valuable lessons were learned by implementation of bowel management in 495 fecally incontinent patients. METHODS We previously reported 201 patients. Thereafter, another 294 patients participated in our program. On the basis of a contrast enema and symptoms, they were divided as follows: (a) 220 constipated patients and (b) 74 patients with tendency toward diarrhea. Colonic stool was monitored with abdominal radiographs, modifying the management according to the patients response and radiologic findings. For constipated patients, the emphasis was on using large enemas. For patients with tendency toward diarrhea, we used small enemas, a constipating diet, loperamide, and pectin. Diagnoses included anorectal malformation (223), Hirschsprungs (36), spina bifida (12), and miscellaneous (23). RESULTS The management was successful in 279 patients (95%)-higher in constipated patients (98%) and less successful in patients with tendency toward diarrhea (84%). CONCLUSIONS The key to a successful bowel management program rests in tailoring the type of enema, medication, and diet to the specific type of colon. The best way to determine the effect of an enema is with an abdominal film. The ACE procedures should be recommended only after successful bowel management.

Hydrocolpos in cloacal malformations

INTRODUCTION Hydrocolpos is a condition rarely mentioned in the literature. The purpose of this report is to increase the index of suspicion for hydrocolpos in patients with cloaca and to describe our approach for its treatment with the hope that errors in the management of hydrocolpos can be avoided. METHODS We reviewed 411 medical records of patients diagnosed with cloaca and managed at our Center during the last 26 years. Emphasis was placed on evaluating for the presence of hydrocolpos, type of drainage, and complications related to the persistence of the hydrocolpos. RESULTS One hundred seventeen cloaca patients had an associated hydrocolpos (28.4%). Forty-two cases (36%) were initially managed at other institutions at which the hydrocolpos was not drained. Complications experienced by this group included: multiple urinary tract infections (8), hydrocolpos infection (7), sepsis (7), failure to thrive (6), ruptured hydrocolpos (4), and development of hydronephrosis in previously normal kidneys (2). Forty-one patients (35%) had other modalities of treatment, aimed to drain the hydrocolpos, including vesicostomy (26), intermittent perineal catheterization (8), single aspiration (6), or plasty of the perineal orifice (1). In all of these cases, the hydrocolpos persisted or reaccumulated. Thirty-four patients (29%) underwent an effective drainage of the hydrocolpos at birth; 29 at other institutions, 15 with a tube vaginostomy, 13 with a tubeless vaginostomy, and 1 with a catheter placed and left in the vagina through cystoscopy. Five cases had a tube vaginostomy done by us. In all these cases, the vagina remained adequately drained as demonstrated radiologically. Proper drainage of the hydrocolpos alone, with no urologic intervention, dramatically improved the hydronephrosis in 13 cases. CONCLUSIONS Hydrocolpos in patients with cloacas must be diagnosed and treated early in life. Our preferred approach is a transabdominal indwelling vaginostomy tube. The drainage of the hydrocolpos alone may dramatically improve the hydronephrosis, and therefore, we suggest that only after the hydrocolpos is drained should a urological intervention be contemplated. Failure to drain the hydrocolpos can result in serious complications.

Laparoscopy and its use in the repair of anorectal malformations.

INTRODUCTION Laparoscopy has been used for the treatment of anorectal malformations (ARMs) in an attempt to be less invasive and with the hope that it would result in a better functional outcome. There remains a significant debate about whether these expectations have been fulfilled. METHODS Seventeen patients with ARM for whom laparoscopy was used were retrospectively reviewed. Six were operated on primarily by the authors, and 11 cases were referred after a laparoscopic repair performed elsewhere. In addition, a literature review was performed looking for evidence of less invasiveness and improved functional results in patients operated on laparoscopically. RESULTS The diagnosis was imperforate anus with a rectobladder neck fistula in our 6 cases with the fistula ligated laparoscopically in each case. In 1 patient, the malformation was repaired entirely using laparoscopic technique. The other 5 patients had a laparoscopically assisted repair because we had to open the abdomen to taper a dilated rectum in 2, mobilize a very high rectum in 2, and take down a distal colostomy stoma in 1. Eleven patients were referred with a variety of problems after a laparoscopic repair done elsewhere for rectal stricture (5), rectal prolapse (4), recurrent rectourethral fistula (3), rectal mislocation (3), failed attempted repair leading to fecal incontinence (1), and a posterior urethral diverticulum (1). Our literature review included 47 references (involving 323 patients) published between 1998 and 2010. All studies showed that laparoscopic repair of ARMs is feasible. The review, however, did not provide evidence of less invasiveness or improved functional results. CONCLUSIONS Laparoscopy for ARM is a less invasive procedure when compared with those operations that would have previously required a laparotomy (rectobladder neck fistula). In cases of rectoprostatic fistulae, the laparoscopic approach is feasible and avoids a lengthy posterior sagittal incision. There is no evidence that the laparoscopic approach is a less invasive procedure for other types of ARMs. In cases of rectobulbar fistula, congenital anal stenosis, perineal fistula, ARM without fistula, the evidence suggests that it may be lead to more complications. There is no evidence in the literature demonstrating better functional results in cases of ARM operated on laparoscopically.

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.

Update on the management of anorectal malformations

Thirty-three years ago, on 10 August 1980, in Mexico City, the first patient with an anorectal malformation was operated on using the posterior sagittal approach. At that time it was not obvious that we were actually opening a “Pandora’s box” that continues to give many positive surprises, a few disappointments, and the constant hope that each day we can learn more about how to improve the quality of life of children born with all different types of anorectal malformations. In November 2012, patient number 3000 in our database was operated in the city of Cochabamba, Bolivia; during one of our International Courses of Anorectal Malformations and Colorectal Problems in Children. The goal of this article is to give a brief update on the current management of patients with anorectal malformation, based on the multiple lessons learned during this period.

Rectovestibular fistula—rarely recognized associated gynecologic anomalies

INTRODUCTION Vestibular fistulas are the most common anorectal malformations (ARMs) in females. Associated gynecologic defects are rarely mentioned in the literature but may have serious clinical implications if undetected. The definitive repair of the ARM offers an opportunity for diagnosis and treatment of these conditions. METHODS Two hundred seventy-two patients with vestibular fistula were retrospectively reviewed, with emphasis on gynecologic defects. RESULTS Forty-eight patients (17%) had 83 gynecologic anomalies. Fourteen patients had a vaginal septum, all with 2 uterine cervices. All septa were resected at the main repair. Twenty-six patients had no vaginal opening. Twenty of them had absent vagina. Eighteen of those had an absent uterus. Patients with absent vagina underwent vaginal replacement with distal rectum (12), sigmoid (6), and ileum (2). Six patients had a patent upper vagina; 3 reached the perineum after mobilization and 3 required replacement; 2 with sigmoid and 1 with rectum. CONCLUSION Vaginal septa are easily diagnosed and can be resected during the repair of the vestibular fistula. The presence of 2 cervices has important obstetric implications. Absent vagina requires a technically demanding repair, with special preoperative planning. Vaginoscopy or careful inspection should precede surgical reconstruction.

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.

Prenatal diagnosis of cloacal malformations

IntroductionPrenatal diagnosis of anorectal malformations currently occurs in 0–15.9% of screened cases. In cloacas, these numbers are unknown. We speculate that some images from prenatal ultrasound studies may suggest the diagnosis of cloaca, but are not recognized because of a lack of suspicion for this diagnosis.MethodsA retrospective review of the medical records of 489 patients born with cloaca was performed; 95 of them had prenatal ultrasound reports that represent the material analyzed for this study. A literature review was performed, finding 31 publications, with 68 cloaca patients detected by prenatal images. The abnormal findings of our patients were compared with those described in the literature to determine the most common abnormal prenatal images found in patients with cloaca.ResultsThe 95 ultrasound reports found in our patients described 270 abnormalities, the most frequent were: abdominal/pelvic cystic/mass (39), hydronephrosis (36), oligohydramnios (23), distended bowel/bowel obstruction (19), ascites (15), 2 vessel cord (14), dilated bladder (14), dilated ureter (14), polyhydramnios (10), echogenic bowel (8), multicystic kidney (8), “ambiguous genitalia” (7), hydrops fetalis (7), hydrocolpos (4), absent kidney (3), abnormal spine (3), and anorectal atresia (3). In spite of these findings, the radiologists who interpreted the studies only suspected a cloaca in 6 cases (6%). The literature review showed 212 abnormalities in 68 demonstrated cloaca patients. The most frequent were: abdominal/pelvic cystic/mass (46), hydronephrosis (44), ascites (21), oligohydramnios (20), distended bowel (11), multicystic dysplastic kidney (7), ambiguous genitalia (6), non-visualization of the bladder (6), two-vessel cord (5), dilated bladder (5), intraabdominal calcification (4), polyhydramnios (4), enterolithiasis (4), hydrometrocolpos (3), and dilated ureter (3).ConclusionWe conclude that it is possible to suspect the diagnosis of cloaca, prenatally, more frequently than what currently occurs, looking at the same images but with an increased index of suspicion for cystic abdominal masses and a combination of gastrointestinal and urological abnormalities.

Transanal, full-thickness, Swenson-like approach for Hirschsprung disease

PURPOSE Swensons procedure for Hirschsprung disease (HD) was thought to disturb fecal, urinary, and ejaculatory functions leading to other approaches including the Soave and Duhamel techniques. Given our Centers experience with a full-thickness rectal dissection for anorectal malformations, and using the new transanal concept, we chose to apply these ideas to the primary treatment of HD, and describe technical aspects and impact on fecal, urinary, and sexual function. METHODS We reviewed our series of HD patients who underwent a transanal, Swenson-like rectosigmoid dissection, assessing for postoperative stricture, anastomotic leak, enterocolitis, and long-term results for bowel, urinary, and sexual function. RESULTS Of 67 patients, 28 had a transanal resection, 5 had transanal plus laparoscopy, and 34 had transanal plus laparotomy, of those, 28 patients had a leveling colostomy prior to referral. The average length of resection was 27 cm ± 12.7 cm. Mean follow-up was 17.2 months (range 1-96 months). 44 patients were at least three years old at follow-up and were assessed for urinary and fecal continence; all (100%) had voluntary bowel movements and urinary continence. Enterocolitis occurred in 9 patients (14%) and constipation (requiring laxatives) occurred in 21 (32%). Of 24 male patients, 21 (88%) reported the occurrence of spontaneous erections post-operatively. CONCLUSION Our data support the fact that a modification of Swensons original transabdominal dissection concept using the recently described transanal approach is an excellent technique for Hirschsprung, and produces excellent long-term outcomes for fecal and urinary continence, and seems to preserve erectile function.

Posterior cloaca—further experience and guidelines for the treatment of an unusual anorectal malformation

INTRODUCTION The term posterior cloaca refers to a malformation in which the urethra and vagina are fused, forming a urogenital sinus that deviates posteriorly to open in the anterior rectal wall or immediately anterior to the anus. METHODS A retrospective review of 411 patients diagnosed with cloaca was performed to identify the ones with a posterior cloaca. Special emphasis was placed on anatomy, diagnosis, associated anomalies, and outcome in terms of urinary and fecal continence. Surgical treatment was a total urogenital mobilization with a transrectal approach. RESULTS Twenty-nine patients were diagnosed with a posterior cloaca. Of these, 15 had a single orifice at the normal location of the anus with the urogenital sinus opening in the anterior rectal wall. Fourteen had the urogenital sinus opening immediately anterior to the normally located anal opening (2 orifices), which we considered a posterior cloaca variant. Nineteen patients (65%) had hydrocolpos. Twenty-seven patients (93%) had associated urologic anomalies, 12 patients (41%) had gynecologic anomalies, and vertebral malformations occurred in 41% of cases. Other anomalies included gastrointestinal (7 patients), cardiac (5), and tethered cord (2). Late diagnosis occurred in 2 patients. Twenty patients were available for long-term follow-up: 17 are fecally continent, 3 are fecally incontinent, 11 are urinary continent, 5 are dry with intermittent catheterization, and 4 have dribble urine. CONCLUSION The most important characteristic of the posterior cloaca is the high frequency of a normal anus, which differentiates this malformation from the classic cloaca. Often, many associated malformations are present and therefore should be suspected and diagnosed. The main goal during the operation should be to not mobilize the anus and thereby preserve the anal canal. A total urogenital mobilization, transperineally or with a transanorectal approach, is ideal for the repair.