Voluntary Bowel Movements Research Articles

One‐stage Correction of Rectovestibular Fistula by Transfistula Anorectoplasty (TFARP)

The present article details a new technique for the repair of recto-vestibular fistula. Twenty-five patients with recto-vestibular fistula, between 13 days and 4 years of age underwent surgical correction by trans-fistula ano-recto-plasty (TFARP). The technique, described in detail, involves mobilization of the fistula and the rectum through the fistula and creation of a new anus in the anatomically normal site by preserving both the perineal skin bridge (skin between the neo-anus and the posterior fourchette) and the levator muscle. The mean operating time was 85 min, and the mean hospital stay was 5 days. Moderate anal stenosis developed in 1 patient and was treated successfully by anal dilatations using Hegar dilators. A diverting colostomy was not required in any patient, and none of the patients developed rectal prolapse. Eleven patients who are now 3 years of age or older have voluntary bowel movements with good fecal continence scores. The 14 neonates and infants, who are still too young to be evaluated for continence, have symmetric anal contraction to stimulation and strong squeeze on digital rectal examination. The average number of bowel movements per day was three to five, without the need for any laxative or enema. Trans-fistula ano-recto-plasty is a simple surgical procedure that does not divide the levator muscle or the perineal body. Preservation of these structures contributes significantly toward improvement of the aesthetic appearance of the perineum and of fecal continence.

Reoperations in Hirschsprung disease

Background We sought to identify causes of preventable complications related to operations for Hirschsprung disease. Methods We reviewed the cases of 51 patients with Hirschsprung disease who underwent a primary procedure elsewhere, had a complication, and were referred for reoperation. Results Thirty-five patients had 1 failed operation, 10 had 2, and 6 had 3. Initial operations were Soave (20), Duhamel (15), Swenson (5), transanal endorectal (4), myectomy (3), unknown (3), and laparoscopic Swenson (1). Thirty-one patients presented with a stoma. Patients without a stoma (20) had fecal impaction (8), recurrent enterocolitis (6), and fecal incontinence (6). None had both enterocolitis and incontinence. Reoperation was performed posterior sagittally (40) or transanally (5). Indications included stricture (21), megarectal Duhamel pouches (12), fistulae (11 [8 rectocutaneous, 2 rectourethral, and 1 rectovaginal]), pouchitis (2), and retained aganglionic bowel (8). After reoperation, 14 were continent, 11 had a stoma (8 permanent), 6 had voluntary bowel movements but soiled occasionally, 6 received rectal irrigations to avoid enterocolitis, 6 were incontinent but clean with bowel management, and 2 were lost to follow-up. Conclusion Stricture, megarectal pouch, fistula, and retained aganglionic bowel are preventable complications. Enterocolitis is partially preventable but can occur after a technically correct procedure. Fecal incontinence is a preventable complication likely because of anal canal damage.

Primary anorectoplasty in females with common anorectal malformations without colostomy

Background/Purpose The objective of this study is to assess the feasibility of primary posterior sagittal anorectoplasty in vestibular fistula without a covering colostomy. Methods Girls presenting from July 1997 to July 2005 with vestibular fistula were included prospectively in the study, in a nonrandomized manner, after excluding those with megarectosigmoid and pouch colon. All underwent primary posterior sagittal anorectoplasty after total gut irrigation with normal saline. They were kept nil per oral until the fifth postoperative day. No patient was started on anal dilatation. Patients were assessed for immediate and delayed complications as well as voluntary bowel movements and continence. Results A total of 72 patients with an age range of 1.5 months to 8 years (median, 9 months) were studied after excluding 7 with pouch colon and 3 with megarectosigmoid. Of the 72, 3 had undergone previous surgery. Follow-up ranged from 7 months to 8 years. No wound dehiscence or recurrence of fistula was noted. There were 5 mild wound infections. At 1 month postoperative, all patients had 1 to 3 stools per day with no episodes of soiling. None required anal dilatations, laxatives, or enemas. Conclusions Primary posterior sagittal anorectoplasty in vestibular fistula can be performed without a covering colostomy provided fecal contamination of the wound can be kept to the minimum in the first postoperative week. We achieve this by thorough total gut irrigation preoperatively and keeping the child nil per oral for the first 5 postoperative days. Continence rates are excellent and postoperative constipation is unlikely if megarectosigmoid and pouch colon are ruled out before surgery. Anal dilatation is not required after surgery.

Sacroperineal mobilization versus posterior sagittal anorectoplasty: A study on outcome

The two main surgical procedures for high and intermediate anorectal malformations (ARM), namely, Stephens' and Peρa's procedure, are compared in terms of their outcome.<br><b> Materials and Methods:</b> Fifty-eight patients who had Stephens' procedure and 28 patients who had posterior sagittal anorectoplasty (PSARP) are clinically analyzed in terms of associated anomalies, procedural complications, anatomical reconstruction and functional results. Functional results were assessed by Kelly score, voluntary bowel movements and sensation. Statistical analysis of data was done by Chi-square test. <br><b> Results:</b> There were 34 high and 52 intermediate ARM. Associated anomalies were noted in 32%. Procedure-related complications of urethral injury, bladder injury, neurogenic bladder, anal stenosis and mucosal prolapse were seen in both procedures. Ectopic positioning of anus was seen in 25% of PSARP and 19% of sacroperineal mobilization (SPM). Good circular sphincter creation was seen in 43% of PSARP and 40% of Stephens'. Noncontractile sphincter was found more with SPM. In functional results, when assessed by Kelly score, VBM and sensation, there was no difference for high ARM, whereas results were better with SPM for intermediate anomalies. <br><b> Discussion:</b> A few reports are available in literature comparing PSARP and SPM. Procedural complications of urethral injury and neurogenic bladder are slightly more with PSARP. Ectopic positioning, poor contraction of sphincter are associated with poor results, and creation of good circular sphincter with good squeeze is associated with good results. Functional assessment by Kelly score, VBM and sensation doesn't reveal any difference between two procedures for high ARM, whereas for intermediate anomalies, Stephens' procedure seems to give better functional results.

Perineal skin bridge and levator muscle preservation in neutral sagittal anorectoplasty (NSARP) for vestibular fistula

The incision in limited posterior sagittal anorectoplasty for vestibular fistula (VF) extends from the coccyx to the fistula with the rectum identified by partial sagittal division of the levator muscle. In anterior sagittal anorectoplasty, the perineal incision extends from the fistula opening to the posterior margin of the external sphincter complex while preserving intact the levator muscle. We describe a modification of the operation for repair of VF, the neutral sagittal anorectoplasty (NSARP), which preserves both a perineal skin bridge between the neo-anus and the posterior fourchette and the levator muscle. Leaving the perineal skin bridge and the levator muscle intact could be important both from the aspects of perineal wound-healing and functional outcome. The skin incision in NSARP extends from the coccyx to the anterior limit of the external sphincter muscle complex as defined by muscle stimulation. An artery forceps passed through the VF facilitates the location and exposure of the rectum. The levator muscle is left intact. The dissection and closure of the VF is done entirely from within the opened rectum. There is an intact perineal skin bridge between the neo-anus and the posterior fourchette at the completion of the NSARP. In 12 consecutive patients with VF, NSARP prevented the risk of wound complications occurring between the neo-anus and the posterior fourchette. A diverting colostomy was safely avoided in our last five patients. NSARP does not involve any division of the levator muscle and it also retains an undisturbed wad of tissue that could function as the perineal body. All five patients who are 3 years or older demonstrate voluntary bowel movements. Preserving the perineal skin bridge and the levator muscle in NSARP have contributed to the improvement of aesthetic appearance of the perineum and faecal continence in our patients.

Surgical management of cloacal malformations: a review of 339 patients

Background/purpose The aim of this study was to describe lessons learned from the authors’ series of patients with cloaca and convey the improved understanding and surgical treatment of the condition’s wide spectrum of complexity. Methods The medical records of 339 patients with cloaca operated on by the authors were retrospectively reviewed. Results A total of 265 patients underwent primary operations, and 74 were secondary. All patients were approached posterior sagittally; 111 of them also required a laparotomy. The average length of the common channel was 4.7 cm for patients that required a laparotomy and 2.3 cm for those that did not. Vaginal reconstruction involved a vaginal pull-through in 196 patients, a vaginal flap in 38, vaginal switch in 30, and vaginal replacement in 75 (36 with rectum, 31 with ileum, and 8 with colon). One hundred twenty-two patients underwent a total urogenital mobilization. Complications included vaginal stricture or atresia in 17, urethral strictures in 6, and urethro-vaginal fistula in 19, all of which occurred before the introduction of the total urogenital mobilization. A total of 54% of all evaluated patients were continent of urine and 24% remain dry with intermittent catheterization through their native urethra and 22% through a Mitrofanoff-type of conduit. Seventy-eight percent of the patients with a common channel longer than 3 cm require intermittent catheterization compared with 28% when their common channel was shorter than 3 cm. Sixty percent of all cases have voluntary bowel movements (28% of them never soiled, and 72% soiled occasionally). Forty percent are fecally incontinent but remain clean when subjected to a bowel management program. Forty-eight patients born at other institutions with hydrocolpos were not treated correctly during the neonatal period. The surgeons failed to drain the dilated vaginas, which interfered with the drainage of the ureters and provoked urinary tract infections, pyocolpos, and/or vaginal perforation. In 24 patients, the colostomy was created too distally, and it interfered with the pull-through. Twenty-three patients suffered from colostomy prolapse. All of these patients required a colostomy, revision before the main repair. Thirty-six patients underwent reoperation because they had a persistent urogenital sinus after an operation done at another institution, and 38 patients underwent reoperation because they suffered from atresia or stenosis of the vagina or urethra. The series was divided into 2 distinct groups of patients: group A were those with a common channel shorter than 3 cm (62%) and group B had a common channel longer than 3 cm (38%). Conclusions The separation of these groups has important therapeutic and prognostic implications. Group A patients can be repaired posterior sagittally with a reproducible, relatively short operation. Because they represent the majority of patients, we believe that most well-trained pediatric surgeons can repair these type of malformations, and the prognosis is good. Group B patients (those with a common channel longer than 3 cm), usually require a laparotomy and have a much higher incidence of associated urologic problems. The surgeons who repair these malformations require special training in urology, and the operations are prolonged, technically demanding, and the functional results are not as good as in group A. It is extremely important to establish an accurate neonatal diagnosis, drain the hydrocolpos when present, and create an adequate, totally diverting colostomy, leaving enough distal colon available for the pull-through and fixing the colon to avoid prolapse. A correct diagnosis will allow the surgeon to repair the entire defect and avoid a persistent urogenital sinus. Cloacas comprise a spectrum of defects requiring a complex array of surgical decisions. The length of the common channel is an important determinant of the potential for urinary control, and predicts the extent of surgical repair.

Clinical evaluation of fecal continence after posterior sagittal anorectoplasty in anorectal abnormalities

Posterior sagittal anorectoplasty (PSARP) was introduced in 1982, by Pena and de Vries, as a new surgical procedure for patients with anorectal malformations. It was supposed to provide better chance for normal fecal continence. Between 1991 and 2000, 50 patients with anorectal abnormalities underwent PSARP. In 43 patients PSARP was primary operation and in 7 it was a secondary procedure. At the time of study patients were not younger than 3, and not older than 13 years. Patients and their parents were interviewed, and fecal continence was graded as follows: voluntary bowel contractions, soiling less than once a week, soiling more than once a week, daily soiling and constipation. Amongst patients who underwent primary surgical correction, 74% had voluntary bowel contractions. Babies with perineal fistula, rectal atresia and stenosis presented with best results in term of voluntary bowel contractions (100%), as with vestibular fistula, ten of eleven patients. Only 18% of patients (atresia without fistula, vestibular fistula and bulbourethral fistula) had soiling. Patients with perineal fistula and rectal atresia and stenosis 40% were totally continent (voluntary bowel movements without soiling). The most frequent sequel was constipation, which appeared in 48%, without coincidence with frequency of soiling. The problem of constipation was surprisingly more frequent in patients with expected better prognosis in fecal continence. Although PSARP offers a good esthetic result, only two third of patients have voluntary bowel movements and in about half there exists a problem with constipation, with necessary further treatment.

Surgical management of cloacal malformations

Cloaca is defined as a defect in which the urinary tract, the vagina and the rectum are fused, creating a single common channel, and opening into an orifice at the site of the normal urethra. Correct management includes an early diagnosis and adequate treatment from the newborn period. We believe that these patients are frequently misdiagnosed and consequently mistreated. The analysis of our experience with the treatment of 330 cases allowed us to make specific recommendations to improve the management of these patients. During the first 24 h of life, emphasis is placed on the recognition and treatment of potentially lethal associated defects, mainly urologic, oesophageal or cardiac. The baby should not be taken to the operating room without ruling out these associated defects. The basic principles of the main repair are delineated. Patients suffering from cloacas with common channels shorter than 3 cm can be treated by a general paediatric surgeon, provided he or she is familiar with the procedure and observes a delicate and meticulous technique. Patients suffering from cloacas with common channels longer than 3 cm belong, by definition, to a more complex type of deformity that, in order to be repaired, requires a surgeon with more experience as well as knowledge of paediatric urology. Sixty percent of all our patients enjoy voluntary bowel movements (VBM). Seventy percent of all patients with a common channel longer than 3 cm require intermittent catheterization to empty their bladder, whereas 20% of those born with a common channel shorter than 3 cm require such a manoeuvre. All patients must be followed on a long-term basis in order to evaluate sexual function and care for obstetric issues.

Bowel function following primary repair of anorectal malformations at Kenyatta National Hospital.

To evaluate bowel function following primary repair of anorectal malformation. A ten-year retrospective study. Kenyatta National Hospital, Nairobi, Kenya. All patients with anorectal malformations attended to at Kenyatta National Hospital (KNH) within the study period who had posterior sagittal repair as a primary definitive procedure. All the children were over three years of age, toilet trained, and had their colostomies closed with an adaptation period of at least six months. Posterior sagittal repair was used to repair anorectal malformations in 352 patients. One hundred and ninety three patients were evaluated. Overall voluntary bowel movement (VBM) was achieved in 71.5% of the patients, soiling was present in 21.2% of the patients and constipation in 7.3% of the patients. More than seventy nine per cent of children who had their colostomy fashioned before the age of one month achieved VBM, while 61.1% of the patients achieved VBM when the colostomy was fashioned after five years. Overall, 77.0% of the females achieved VBM compared to 63.8% of males. Patients with a perineal fistula achieved VBM in 79.1% of males and 75.0% of females, 76.0% with vestibular fistula, 73.9% with a recto-urethral fistula, 56.0% of anorectal anomalies without a fistula, 25.0% of vaginal fistulae and 12.5% in vesical fistulae. Overall patients with sacral defects achieved VBM in 25.9% compared to 78.9% in patients with a normal sacrum. The patients with low anomalies achieved VBM in 75.4% compared to 46.1% with high anomalies. Posterior sagittal repair has been used to repair all anorectal malformations and has improved the quality of life of our patients, with better functional results expected in female patients, early colostomy fashioning and definitive repair, low or simple anomalies, and absence of sacral defects. The repair was associated with low morbidity and mortality.

Surgical treatment of cloacal anomalies.

From 1989-1998 14 patients were treated with cloacal anomalies: 5 typical cloacas (PC), 5 posterior cloacas, and 4 cloacal exstrophies (CE); 12 underwent surgery. Four typical cloacas were resolved with posterior sagittal anorectovagino-urethroplasty (PSARVUP), whereas in the 5th total urogenital mobilization (TUM) was used. Three PCs were managed with transanorectal TUM and 2 with anterior TUM without opening the anal canal and rectum (without a protective colostomy). Two CEs were treated with atypical procedures. Two patients with CE died without surgery and 2 died after surgery due to complex associated anomalies. During postoperative follow-up of 1-8 years, 5 children had voluntary bowel movements and no soiling while the others had soiling with or without enemas; 1 had stress incontinence; 3 were on clean intermittent catheterization due to neurogenic bladder and were dry. PSARVUP provides a satisfactory result if there is no sacral anomaly. TUM makes this operation easier to perform. In patients with a PC it is sometimes possible using TUM to separate the urinary from the genital tract and remove the accessory urethra without opening the anus and rectum.

Advances in the management of anorectal malformations

Background: Anorectal malformations represent a continuing challenge for the pediatric surgeon. A significant number of these children suffer from fecal incontinence, urinary incontinence, and sexual inadequacy. However, major advances in the management of these children have occurred during the last 10 years. The posterior sagittal approach has led to a better understanding of the internal anatomy of these defects, and in turn, a more rational way to manage the patients. We present here our experience with the long-term follow-up of a large series of these patients operated on by us. Methods: Clinical information from our database of patients with anorectal malformations was retrospectively reviewed. A total of 1,192 patients operated on by the authors and observed for the last 19 years are included in the database. We searched for type of malformation, associated defects, results in terms of voluntary bowel movements, soiling, constipation, and urinary control. Results: Globally, 75% of all patients have voluntary bowel movements. Half of this group still soils their underwear occasionally. Therefore, about 37.5% of all cases are considered totally continent. Constipation was the most common sequelae. Urinary incontinence was relatively common after the repair of cloacas. Male patients rarely suffered from urinary incontinence. Twenty-five percent of all cases suffered from fecal incontinence but they significantly improved their quality of life when subjected to a bowel management program. An operation called “continent appendicostomy” further improved their quality of life. Conclusion: All patients born with anorectal malformations can be kept clean of stool and dry of urine, either because they achieve bowel control or because they are subjected to a bowel management program.

Posterior sagittal anorectoplasty for failed imperforate anus surgery: Lessons learned from secondary repairs

Purpose: The aim of this study was to evaluate secondary operations using a posterior sagittal approach in patients with fecal incontinence and impaction after primary repair of anorectal malformations. Methods: Twenty patients (14 boys, 6 girls) who had previous failed surgery for imperforate anus underwent secondary operations. The indications for surgery included fecal incontinence (n = 16) and fecal impaction (n = 4). Patients ranged in age from 2 to 30 years (mean, 11 years), with 4 over the age of 20 years. The primary procedures included abdominosacroperineal (n = 7), sacroperineal (n = 10), and perineal (n = 3) pull-throughs. At surgery, none of the patients underwent a diverting colostomy. The rectum was mobilized from the surrounding structures through a posterior sagittal approach. The surgical findings included anteriorly displaced anus (n = 17), laterally displaced anus (n = 3), mesenteric fat surrounding the rectum (n = 4), megarectosigmoid (n = 2), and others (n = 3). The rectum underwent reconstruction, which involved relocation of the rectum and anus to surround them with the muscle complex. Results: Patients underwent follow-up for periods ranging from 8 months to 6 years after surgery (mean, 3 years). To evaluate the functional results, fecal continence scores (Templeton and Ditesheim) were calculated for incontinent patients. Of the 16 incontinent patients, 12 achieved continence and 4 some improvement. Of the 4 patients with fecal impaction, 2 achieved daily voluntary bowel movement, whereas the other 2 have mild constipation and need occasional enemas. Conclusions: Our study suggests that (1) a secondary operation through a posterior sagittal approach can restore fecal continence and is efficacious even in adolescents and adults and (2) a posterior sagittal procedure can be safely performed without a diverting colostomy. J Pediatr Surg 35:1626-1629. Copyright © 2000 by W.B. Saunders Company.

Posterior sagittal approach: Megasigmoid resection and anal reconstruction for severe constipation and fecal incontinence after anoplasty

Purpose: The aim of this study was to present the technique of megasigmoid resection and anal reconstruction by complete posterior sagittal approach for the children with severe constipation and fecal incontinence after anoplasty. Methods: Six patients (age, 2 to 18 years) born with imperforate anus and originally treated with perineal anoplasty suffered from intractable constipation and fecal incontinence. Contrast enema showed massive dilated and aperistaltic rectosigmoid colon with fecal impaction. Resection of the dilated bowel and anal reconstruction were completely performed by posterior sagittal approach. Results: The mean operating time was 205 minutes (range, 125 to 265 minutes) and the average length of resected colon was 23.3 cm (range, 10 to 40 cm). There were no intraoperative or postoperative complications. By 2 to 4 months after the operation, all patients obtained voluntary bowel movement. On follow-up at 6 to 24 months postoperative, no patient had constipation or required use of the laxatives again. Four of 6 patients suffered from grade 1 soiling, and the other 2 had grade greater than 1 soiling. None had urinary retention or incontinence after the procedure. Conclusion: Resection of dilated rectosigmoid colon and anal reconstruction for the patients with severe constipation and fecal incontinence after anoplasty can be performed successfully using a posterior sagittal approach. J Pediatr Surg 35:1058-1062. Copyright © 2000 by W.B. Saunders Company.

The treatment of imperforate anus: Experience with 108 patients

Background/Purpose: The authors present their experience and results in the treatment of infants with imperforate anus over a 10-year period. Differences between these and previously published western results are noted and discussed. Methods: One hundred eight patients with imperforate anus were treated from June 1988 to July 1998. Of these patients, 66 were boys and 42 were girls. Associated anomalies include congenital heart disease, anomalies of bone and cartilage, and Down's syndrome. Thirty-five patients with a low lesion received a limited posterior sagittal anorectoplasty. Seventy-one patients had a high lesion and received 3-staged operations including colostomy, posterior sagittal anorectoplasty, and takedown of colostomy. All patients underwent follow-up by the author. Postoperative anorectal function was evaluated based on the following criteria: ability to have voluntary bowel movement, soiling, and constipation. The duration of follow-up ranges from 6 months to 10 years. Results: One patient died of multiple congenital anomalies after colostomy. One patient died of hyaline membranous disease. All except 2 patients had voluntary bowel movement. Three patients had soiling, and 19 suffered from constipation after operation. The constipation improved with medical treatment and time. Four patients who received the first operation at another hospital (3 underwent posterior sagittal anorectoplasty and 1 had cutback anoplasty) had problems with soiling. In these patients, soiling improved after redo posterior sagittal anorectoplasty. Conclusions: Utilizing the posterior sagittal operation described by Peña, most patients were continent and able to have voluntary bowel movements. Constipation occurred in a substantial number of patients with high-type lesions, but few of these patients needed medication or enemas. There were significantly fewer sacral and urogenital anomalies than have been reported in most western series. This may explain the excellent results.

Rectovestibular Fistula With Absent Vagina: A Unique Anorectal Malformation

Abstract Background/Purpose: A very unusual anorectal defect consisting of a vestibular fistula and absent vagina is presented. Only two previous isolated reports were found in the literature dascribing a repair that included both abdominal and perineal approach. The authors present another technical alternative that allows the repair via posterior sagittal. Methods: Of 1,007 patients with anorectal malformations analyzed, eight female cases shared the common anatomic features of a vestibular fistula and absent vagina. What appeared to be the vagina was actually the rectal orifice ending in the vestibule, and the vagina was absent. With the posterior sagittal approach, the distal rectum was used to create the neovagina. The proximal rectum was mobilized and placed within the limits of the sphincter mechanism. Two of the eight patients were born with a patent upper third remnant of the vagina. In one case, the remnant was anastomosed to the neovagina at the time of colostomy closure; in the second one, the anastomosis was performed during the main repair. Results: Four patients are continent of urine and have voluntary bowel movements. One patient is younger than 3 years and therefore has not been evaluated. One patient has a very poor sacrum and therefore is incontinent. One patient had a primary repair elsewhere and therefore is excluded, and another patient is lost to follow-up. One patient is already sexually active. Conclusions: It is mandatory to perform a meticulous inspection of the perineum in female patients with anorectal malformations to detect unusual defects. Also, a high index of suspicion is necessary to establish the diagnosis of this defect and avoid an inadequate treatment. The posterior sagittal approach represents another alternative to treat these defects without the need for a laparotomy.

Anal transposition without colostomy: functional results and complications

Rectovestibular fistula (RVF) is the most common form of anorectal anomaly in female infants. In the surgical repair of these malformations, most pediatric surgeons use cutback, fistula transposition with or without colostomy, and lately, posterior anorectoplasty with colostomy. This is a retrospective evaluation of the functional results and complications in 47 patients who underwent fistula transposition without colostomy for the treatment of a RVF. We prefer to perform the operation when the rectovaginal septum is amenable to dissection (width >2 mm). All patients had voluntary bowel movements; 28 (60%) had completely normal bowel habits, 45 (96%) good and only 2 (4%) fair. We did not encounter serious surgical complications such as infection dehiscence, and fistula recurrence. We thus prefer anal transposition without colostomy to other modes of surgical therapy for RVF.

The failed anoplasty: Successful outcome after reoperative anoplasty and sigmoid resection

Background/Purpose: Children with anorectal malformations often have less than optimal results after repair. The authors report on five patients (ages 3 to 17 years) born with imperforate anus and treated with anoplasty as a newborn. At presentation, all patients were completely incontinent of stool. None had ever experienced voluntary bowel movements, and all wore diapers continuously. Methods: Perineal examination with the nerve stimulator showed the muscle complex was largely intact with good contraction, but the neoanus was outside of the muscle complex. Contrast enema showed massive dilation of the rectosigmoid colon and fecal impaction. A tethered spinal cord was excluded by magnetic resonance imaging (MRI). We treated these patients with a combined reoperative anoplasty via the posterior sagittal approach and sigmoid resection. Results: Within 6 months after the procedure, all patients had achieved complete continence. They had from one to three voluntary bowel movements per day without soiling. Conclusions: Children with severe constipation and fecal incontinence after anoplasty should undergo evaluation by a surgeon. If examination shows a reasonably intact muscle complex and correctable anatomic defects, an excellent result can be achieved with appropriate reoperation.

Rectovestibular fistula with absent vagina: A unique anorectal malformation

Background/Purpose: A very unusual anorectal defect consisting of a vestibular fistula and absent vagina is presented. Only two previous isolated reports were found in the literature dascribing a repair that included both abdominal and perineal approach. The authors present another technical alternative that allows the repair via posterior sagittal. Methods: Of 1,007 patients with anorectal malformations analyzed, eight female cases shared the common anatomic features of a vestibular fistula and absent vagina. What appeared to be the vagina was actually the rectal orifice ending in the vestibule, and the vagina was absent. With the posterior sagittal approach, the distal rectum was used to create the neovagina. The proximal rectum was mobilized and placed within the limits of the sphincter mechanism. Two of the eight patients were born with a patent upper third remnant of the vagina. In one case, the remnant was anastomosed to the neovagina at the time of colostomy closure; in the second one, the anastomosis was performed during the main repair. Results: Four patients are continent of urine and have voluntary bowel movements. One patient is younger than 3 years and therefore has not been evaluated. One patient has a very poor sacrum and therefore is incontinent. One patient had a primary repair elsewhere and therefore is excluded, and another patient is lost to follow-up. One patient is already sexually active. Conclusions: It is mandatory to perform a meticulous inspection of the perineum in female patients with anorectal malformations to detect unusual defects. Also, a high index of suspicion is necessary to establish the diagnosis of this defect and avoid an inadequate treatment. The posterior sagittal approach represents another alternative to treat these defects without the need for a laparotomy.

Anorectal malformations and Down's syndrome

Background/Purpose : Down's 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 Down's syndrome had specific characteristics and whether the presence of Down's syndrome represented a serious detriment to the patient's functional prognosis. Methods : Nine hundred eighty-seven patients with anorectal malformations were studied retrospectively. Twenty patients (2%) had Down's 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 Down's 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 Down's 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 Down's syndrome with imperforate anus without fistula is not coincidental. This particular benign defect can be predicted to occur in most patients with Down's syndrome. The presence of Down's syndrome in cases of anorectal malformations should not be a contraindication to repairing the imperforate anus and to closing the colostomy.

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 patient's 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 drognosis.