Marc A. Levitt

Bowel management for fecal incontinence in patients with anorectal malformations

BACKGROUND/PURPOSE Fecal incontinence is common in patients operated on for anorectal malformations. Treatment with enemas, laxatives, and medications are often given by clinicians in an indiscriminate manner and without a demonstrated benefit. A systematic diagnostic approach and bowel management program was developed for patients suffering from fecal incontinence, and a retrospective analysis of the results is presented. METHODS Three hundred forty-eight patients were seen in consultation for fecal incontinence after repair of imperforate anus at other institutions. Clinical and radiological evaluation helped determine different types of patients. Group I consisted of 147 patients who were considered candidates for reoperation and forms the basis of a future report. Group II included 172 patients who had no potential for bowel control and were therefore candidates for bowel management. These patients fell into two categories; group IIA included 44 patients with incontinence and constipation. The bowel management involved the use of daily large enemas only. Group IIB included 128 patients with incontinence and a tendency to diarrhea. Group III consisted of 29 patients who had pseudoincontinence. They had an original defect with good prognosis, good sphincters, good sacrum, and a well-located rectum. They suffered from severe constipation, megasigmoid, chronic fecal impaction, and overflow pseudoincontinence and were treated with laxatives or sigmoid resection. RESULTS Bowel management was successful in 93% of patients in the constipation group (IIA) and 88% in the diarrhea group (IIB). Ninety-seven percent of patients in group III became fecally continent. CONCLUSION Bowel management consisting of enemas, laxatives, and medications is successful when administered in an organized manner. It is vital to determine the type of fecal incontinence from which the patients suffer and to target their treatment accordingly.

Continent appendicostomy in the bowel management of fecally incontinent children

BACKGROUND Fecal incontinence is common in children who have anorectal malformations, Hirschsprungs Disease, and spina bifida and can negatively impact their emotional and social development. Enemas have been used as an artificial way to keep children clean and to improve their quality of life. This method is unpleasant for many children, particularly when they reach adolescence. Malone in 1990 described an alternative method in which the appendix is used as a conduit to administer an antegrade enema. METHODS The authors describe their experience with this new procedure, modified by them, and used in 20 patients. In the original procedure, the base of the appendix is divided, inverted, and reimplanted into the cecum with an antireflux technique. The authors simplify this by plicating the cecum around the appendix to create a one-way valve mechanism but leaving the appendix in its original position. The authors also mobilize the cecum and exteriorize the appendix at the umbilicus to create an inconspicuous stoma. If the native appendix is absent a neoappendix was created from a flap of cecum. RESULTS Nineteen of 20 patients (95%) are now completely clean 24 hours a day. Stricture of the stoma occurred in two patients and required revision. Leakage at the appendicostomy site occurred in three patients, and two required a tighter plication. CONCLUSIONS The technique is used to change the route of enema administration, and is only used in patients for whom bowel management with rectal enemas has been proven successful. The appendix must be preserved whenever possible in patients at risk for fecal incontinence.

The tethered spinal cord in patients with anorectal malformations

The aims of this study were to find the prevalence of tethered cord in patients with anorectal malformations; to determine if the presence of tethered cord relates to the severity of the anorectal defect, and to certain symptoms, signs, radiologic findings, and associated anomalies; and finally to determine whether tethered cord impacted on a patients functional prognosis and whether surgical untethering improved the patient. The authors studied 934 patients with anorectal malformations, 111 of whom had magnetic resonance imaging (MRI) of the spine. We compared patients with and without tethered cord by using parametric and nonparametric statistical tests. Tethered cord occurred in 24% of the patients. The prevalence varied according to the type of anorectal defect from 43% in the complex group to 11% in patients with rectovestibular fistula. Patients with tethered cord had a lateral sacral ratio lower than that of patients without tethered cord (0.410 versus 0.702). Tethered cord was present in 90% of patients with myelodysplasia, 60% of patients with a presacral mass, 57% of patients with sacral hemivertebrae, and 56% of patients with a single kidney. The greater number of associated anomalies a patient had, the greater the risk of having tethered cord (P < .05 for all differences). The authors noted differences between patients with and without tethered cord in the presence of voluntary bowel movements (46% versus 70%), fecal soiling (91% versus 63%), constipation (21% versus 43%), and urinary incontinence (86% versus 42%). The data indicate that patients with tethered cord have a worse functional prognosis than patients without tethered cord. However, the incontinence in our patients was also predictable based on the type of anorectal defect and the character of the sacrum irrespective of the presence of tethered cord. Eighteen patients underwent surgical untethering of the cord, and none had any significant change in bowel or urinary function postoperatively. No patient with tethered cord experienced incontinence that could be attributed to the cord defect alone. This study suggests that tethered cord occurs more frequently in patients with severe anorectal defects, sacral hypodevelopment, myelodysplasia, presacral mass, sacral hemivertebrae, or a single kidney, or in those with an anorectal defect with poor functional prognosis. At present no solid evidence supports the concept that tethered cord by itself affects the functional prognosis of patients with anorectal malformations. Also, there is no good evidence demonstrating that surgical untethering improves the prognosis.

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.

Gynecologic concerns in the treatment of teenagers with cloaca

Abstract Background/Purpose: Gynecologic anomalies are common in patients with persistent cloaca, but, except for hydrocolpos, these patients remain asymptomatic in the neonatal period. Anatomic abnormalities may become manifest in puberty when menses occurs. The authors sought to describe the sequelae of these anatomic defects in the teenage cloaca patient and to determine recommendations for prevention and treatment in the neonatal period. Methods: From a series of 198 patients operated on for persistent cloaca, the authors report a group of 22 patients who have reached puberty. Results: Seven patients are menstruating normally. Six patients have primary amenorrhea because of absent or atretic uteri. Nine patients presented with abdominal pain and cystic abdominal masses and required surgical resection of inflamed collections of old blood in uteri, hemiuteri, tubes, blind vaginas, hemivaginas, or in the peritoneum. The common denominator of this last group was an obstruction of one or more Mullerian structure that interfered with the drainage of menstrual blood. Asymmetric gynecologic anatomy or a vaginal atresia in the neonatal period seemed to correlate with future obstruction to menstrual flow. Conclusions: To prevent future problems, the management of the neonatal cloaca must include the early clarification of the gynecologic anatomy, specifically the patency of Mullerian structures. Unilateral atretic structures and tubes connected to atretic uteri should be resected. Also, clinicians must be suspicious of menstrual problems in teenagers operated on early in life for persistent cloaca.

Anorectal malformations and Down's syndrome

BACKGROUND/PURPOSE Downs syndrome is a common association in patients with anorectal malformations. The purpose of this study was to determine whether the anorectal defect in patients with Downs syndrome had specific characteristics and whether the presence of Downs syndrome represented a serious detriment to the patients functional prognosis. METHODS Nine hundred eighty-seven patients with anorectal malformations were studied retrospectively. Twenty patients (2%) had Downs syndrome. Nineteen of these (95%) had the same specific type of anorectal defect: imperforate anus with no fistula. This defect has a good prognosis, the rectum is located about 2 cm above the perineal skin, the sacrum is normal, and the sphincter mechanism is good. For comparison, a group of 34 patients with the same defect but without Downs syndrome was also studied. All patients were operated on via posterior sagittal approach by the same surgeon. RESULTS Imperforate anus without fistula occurs in 5% of all patients with anorectal malformations and in 95% of those patients who also suffer from Downs syndrome. The characteristics of the defect were the same in both groups of patients, and surprisingly, the prognosis was good in both groups (80% to 96% of patients had voluntary bowel movement, 100% had urinary continence). CONCLUSIONS The association of Downs syndrome with imperforate anus without fistula is not coincidental. This particular benign defect can be predicted to occur in most patients with Downs syndrome. The presence of Downs syndrome in cases of anorectal malformations should not be a contraindication to repairing the imperforate anus and to closing the colostomy.

Hirschsprung disease and fecal incontinence: diagnostic and management strategies.

PURPOSE Ideally, fecal incontinence after operative management for Hirschsprung disease should not occur. If it does, it presents a formidable challenge. The purpose of this study was to describe the causes of fecal incontinence and present our algorithm for its treatment. METHODS We reviewed 68 patients with Hirschsprung disease and fecal incontinence referred to us after surgery at other institutions. Patients were evaluated by contrast enema and by an examination under anesthesia to look specifically for the integrity of the anal canal. They were designated as having a dilated colon and constipation or a nondilated colon and a tendency to diarrhea based on their stooling pattern and the appearance of the contrast enema. Medical management was started that included laxatives for those patients with a dilated colon and constipation. For those with a nondilated colon and tendency to diarrhea, the management included loperamide, pectin, and a special dietary regimen (constipating diet, 3 meals per day, and no snacks). Those patients who responded to medical management were retrospectively considered to have been pseudoincontinent. Those who did not respond were considered truly incontinent. The truly incontinent group was treated with enemas alone for those with a dilated colon, or enemas, loperamide, pectin, and a constipating diet for those with a nondilated colon and tendency to diarrhea. RESULTS Fifty-six patients had true incontinence and 12 had pseudoincontinence. Of the true incontinent group, 27 had a dilated colon and 29 had a nondilated colon. Five of these patients had a damaged or absent anal canal (anastomosis at the anal skin) and all of them had true incontinence. In the dilated colon group with true incontinence, 23 (85%) patients were clean after treatment. In the nondilated colon group with true incontinence, 23 (79%) were successfully treated. All patients in the pseudoincontinent groups had no soiling after treatment. Of 55 in the truly incontinent group, 39 (70%) had had an endorectal (Soave type) pull-through. CONCLUSION Fecal incontinence after operative management of Hirschsprung disease represents a serious problem. Poor surgical technique may be a contributing factor in some of the cases. Successful management depends on the appropriate evaluation, which determines whether the incontinence is true or pseudo, and the type of colon the patient has. Each category can be well treated, leading most of the time to a clean child.

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.

Pitfalls in the management of newborn cloacas

Clinicians caring for newborns with persistent cloaca face significant challenges in the newborn period. Avoiding key pitfalls during this time can have dramatic implications. We reviewed the medical records of 361 patients with cloaca operated on at our institution and analyzed sequelae that resulted from incorrect management in the newborn period. Of 361 patients, 282 underwent primary operations at our institution, and 79 patients were referred to us after a failed repair at other institutions. Pitfalls in management during the newborn period included the following: (1) Failure to recognize and manage hydrocolpos, which occurred in 46 patients. Of these, three patients developed pyocolpos (two progressed to vaginal perforation), and 43 suffered from persistent bilateral hydronephrosis, megaureters, recurrent urinary tract infections, persistent acidosis, or failure to thrive due to undrained hydrocolpos. They underwent unnecessary urinary drainage procedures (nephrostomy, ureterostomy, cystostomy, or vesicostomy) in the newborn period. When the vagina was finally decompressed, all of these symptoms disappeared. (2) Colostomy or vesicostomy problems, which occurred in 50 patients. These included incorrect placement of the colostomy (too distal, which interfered with the pull-through) in 24 and colostomy prolapse in 23. Incompletely diverting loop colostomies led to urinary tract infections in 49 patients. Vesicostomy prolapse occurred in three patients. (3) Clinical misdiagnosis, which occurred in 42 patients. Six were incorrectly diagnosed as “intersex” and 36 as “rectovaginal fistula.” In this group only the rectum was repaired, and the patients were left with a urogenital sinus that required reoperation. Proper management of a newborn with cloaca includes drainage of a hydrocolpos, which avoids unnecessary urinary diversions and pyocolpos. Our preferred colostomy is one with separated stomas, adequate distal bowel for the pull-through, and use of a proper technique to avoid prolapse. Correct clinical diagnosis of cloaca avoids problems during the definitive repair.

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.