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Megasigmoid: a source of pseudoincontinence in children with repaired anorectal malformations.

Three children with a history of anorectal malformation repairs were referred to the authors for evaluation and management of fecal incontinence. Their ages ranged from 5 to 7 years. On examination, all the children had fecal impaction and localized dilatation of the rectosigmoid colon. Medical treatment was tried but failed to control the symptoms, and the patients frequently had to be hospitalized for disimpaction. To correct this problem, the authors resected the dilated sigmoid colon, anastomosing the nondilated descending colon to the rectal ampulla, which was preserved to serve as a reservoir. Postoperatively, constipation was cured in all patients. In addition the patients became fecally continent postoperatively, which was an unexpected bonus. The authors believe that localized dilatation of the rectosigmoid should always be considered whenever a child is having intractable constipation after repair of an anorectal malformation and that sigmoid resection may be considered as a therapeutic alternative. Segmental dilatation of the sigmoid colon may be a source of fecal pseudoincontinence and, therefore, should be ruled out when the surgeon is evaluating patients with fecal incontinence.

Adolescent↗

N-type anorectal malformations.

Three cases of N-type anorectal malformations have been encountered. Two girls with anorectal-vestibular fistulas (one with a normal anus and the other with mild stenosis) were typical of 15 other such cases in the literature. A boy with an anorectal-urethral fistula and covered anus had a lesser variant than most of the other 8 males in the literature. The frequently accompanying esophageal, renal, and skeletal anomalies were not present, nor did he have anterior urethral hypoplasia. The constellation of other major associated anomalies in the boys, but not in the girls, follows the pattern of other types of anorectal malformations and suggests an earlier and/or more severe teratogenic insult in the boys. Anterior perineal resection of the fistula is appropriate for girls with N-type fistulas and for those boys with normal anterior urethras. An additional urethroplasty is necessary for boys with anterior urethral hypoplasia. Our experience and that from the literature suggest that N-type anorectal malformations constitute a rare but real entity, akin to N-type tracheoesophageal fistulas. A plea is made that they be included in classifications of anorectal anomalies.

Anal Canal↗

Urogenital sinus and anorectal malformation: experience with 22 cases.

Urogenital sinus and anorectal malformation is a complex problem. Anatomy must be defined precisely in each to guide surgical reconstruction, details of which can vary greatly. Although primary reconstruction in the newborn has been accomplished, it may be safer in most to start with decompression colostomy and definition of the anatomy. If the vagina is very distended with urine, it should be drained by some means. Separate abdominal perineal pullthrough of the colon without vaginoplasty should never be performed, because it can cause serious obstructive uropathy when the rectal--urogenital sinus fistula is divided. Ureteral reimplantation is often necessary. Many of these children have undergone urinary diversion; most of those are suitable candidates for "undiversion."

Abnormalities, Multiple↗

Reappraisal of endorectal pull-through procedure. I. Anorectal malformations.

Postoperative continence in anorectalmalformations was assessed by clinical score, rectoanal manometry, and electromyography. The results in low deformities operated by perineal rectoplasty showed "good" clinical scores, good preservation of the rectoanal reflex, and good electrical activity of the external sphincter. High deformities, on the other hand, showed low clinical scores, poor recordings of the rectoanal reflex, and diminished electrical activity around the anus. Analysis of high deformities treated by operative procedures, however, indicated better clinical scores and better preservation of rectoanal reflex in those who had undergone the endorectal pull-through procedure (Rehbein) than those who had had an abdominoperineal pull-through procedure. Furthermore, dissections of newborns with a rectourethral fistula revealed the presence of the puborectalis sling around the terminal rectum and fistula in a hammock fashion. This finding supports our suggestion that the colon can be safely pulled-through within the sling by the original Rehbein procedure without sacral dissection.

Anal Canal↗

Complications at menarche of urogenital sinus with associated anorectal malformations.

Persistent urogenital sinus is frequently associated with a wide spectrum of complex anatomic abnormalities involving the urinary, genital, and gastrointestinal tracts. Failure to accurately define these abnormalities can result in serious complications. One such group of complications occurs relatively late in the clinical course of these children and has received little attention. These are the complications related to menarche. This report reviews the anatomy, complications, and management in five such patients. In this group the onset of menses was associated with hydrosalpinx, pyosalpinx, hematocolpos, hematometrocolpos tubo-ovarian abscess, ruptured ovarian endometrioma, endometriosis, and peritonitis. Anatomic abnormalities included double and septate vagina, vaginal atresia and stenosis, uterus didelphys, and uterus bicornis unicollis, all predisposing to inadequate menstrual flow. Each of these children required surgical intervention. These cases stress the need for an awareness of not only the urinary but the vaginal and uterine abnormalities. Careful early definition of the anatomy and long-term follow-up of children with urogenital sinus malformation is important in order to avoid potential future complications.

Abnormalities, Multiple↗

Posterior sagittal anorectoplasty.

From October 1980 to November 1981, 34 patients with anorectal anomalies have been operated upon by a sagittal midline approach. The skin incision extends from the sacrum to the perineum (ventral aspect of the anal dimple). The superficial and deep layers of the external sphincter are identified by electrostimulation and split, with the coccyx, in the midline. Ileo- and pubococcygeal portions of the levator dorsally and then the striated muscle complex of the external sphinctor, pubococcygeus and the presumed puborectalis are split ventrally to the urethra. In no cases has the ventral portion of the levators been separated from the thick ventral portion of the external sphincter, hence the term "striated muscle complex." When the terminal bowel is dilated (congenitally ectatic), the bowel is tailored prior to reconstruction of the sphinctors. The posterior sagittal approach provides an excellent exposure for evaluation and mobilization of the terminal bowel. It enables one to construct an anal canal, suture the bowel wall to the striated musculature and the mucosa to the skin, thereby reducing or avoiding the complications of prolapse and stenosis. In males with ectasia and a rectourethral fistula, transrectal closure of the mucosa at the fistula site, leaving the rectal longitudinal smooth muscle insertions on the prostatic capsule, avoids damage to the nerves and genital structures.

Anal Canal↗

Further experience in reconstructive surgery for cloacal anomalies.

This paper describes 13 more cases of urogenital sinus malformation with an anorectal anomaly, usually rectal atresia with rectovaginal fistula. It is vital to define the anatomy precisely in these cases to guide the operative approach best suited for repair. Neonatal loop colostomy is needed in most. Intermittent catheterization of the bladder and/or urine-filled vagina via the urogenital sinus can allow temporizing until the child is old enough for a definitive reconstruction. Various urinary diversions are best avoided if possible, except possibly a simple temporary suprapubic cystostomy. It is best to complete the reconstructive operation, in a single procedure which is often long and complex. Preliminary AP pullthrough of the colon without repair of the genitourinary aspect of the problem can lead to urinary tract stasis and serious obstructive uropathy. A satisfactory functional and anatomic solution is possible in most of these girls.

Adolescent↗

Posterior sagittal anorectoplasty: important technical considerations and new applications.

Posterior sagittal anorectoplasty (PSARP) is a new technique for the repair of high anorectal malformations. It is based upon complete exposure of the anorectal region by means of a median sagittal incision that runs from the sacrum to the anal dimple, cutting through all muscle structures behind the rectum. It was learned through this procedure that the external sphincter is a functionally useful prominent structure. No puborectalis sling, as such, could be identified. It was possible, however, to recognize a muscle continuity from the skin to the sacral insertion of the levator ani. Since it is impossible to pull the generally ectatic rectum through without destroying the muscle structures present, the rectum must be tapered to allow suturing the muscle behind it. In all these anomalies, the rectum and urethra (or vagina) are very closely joined, sharing a common wall, and their separation calls for extensive exposure. A number of technical details clarified in the course of applying the procedure in 54 patients are fully discussed. This approach also proved to be very successful in the management of two patients with rectocloacal fistula, two with rectal atresia and two with stenosis. Colostomy has been closed in 27 patients and fecal continence may be described as excellent, except in those patients with severe sacral anomalies, and unquestionably superior to that obtained by us with other techniques.

Anal Canal↗

Loop colon rectovaginoplasty for high cloacal anomaly.

We describe a simple technique for simultaneous reconstruction of all three perineal openings, including a functional vagina, in a case of high cloacal anomaly. End-to-end stapled colo-vaginal anastomosis allows creation of both vagina and rectum from one loop of bowel.

Anus, Imperforate↗

The use of computerized tomography to evaluate anorectal anomalies.

Computerized tomography (CT) was applied to various types of anorectal anomalies to directly image the anal sphincters. In normal cases and low type anomalies, CT demonstrated clear images of the puborectal muscle and external sphincters. Among high type anomalies, the distribution of sphincter muscle in patients with rectovesical fistula is totally different from that seen in patients with rectourethral fistula. In the latter, the puborectal muscle is attached not only around urethra, but also around the distal part of the blind rectum; external sphincters are present as a mass beneath the perineum. In the rectovesical fistula, however, the puborectal muscle cannot be identified and the external sphincters exist only as a string-like structure. CT done postoperatively identified two different causes for incontinence. In one type, the pull-through colon missed the sphincteric musculature, and in the other, sphincters were markedly hypoplastic. CT, therefore, provides adequate imaging to determine the type of operation needed to correct the abnormality.

Anal Canal↗

A technique for temporary control of colostomy prolapse in children.

Stomal prolapse is a common complication of colostomies and, if severe, may affect the timing of the definitive procedure. A simple technique for postreduction bowel fixation without stomal revision is presented. The reduced bowel is attached to the parietal peritoneum using a "U" type suture a few centimeters from the stoma. Two short parallel segments of latex tubing prevent the thread from cutting through. Suture and bolsters are removed when adhesions are established. In one of the four children, a percutaneous cecostomy was employed to connect a prolapse of the entire proximal colon.

Abdominal Muscles↗

Complications of posterior sagittal anorectoplasty.

From 1982 to 1985, 23 patients underwent posterior sagittal anorectoplasty procedures: 12 as primary treatment for congenital anorectal malformations, 9 for treatment of fecal incontinence following a prior pull-through procedure, and 2 for treatment of fecal incontinence following trauma. Six patients (26%) developed seven complications specifically related to the procedure. One patient with a cloacal anomaly had partial dehiscence of the sacroperineal incision following total reconstruction. This resulted in retraction of vaginal and anal openings, which, however, have remained separate and patent. Two patients developed temporary femoral nerve palsies, unilateral in one patient lasting one week, and bilateral in one patient lasting four months. Four patients developed leaks from the suture line of the tailored ectatic rectum, which was pulled through to the perineum. In one male patient, the suture line was placed anteriorly, resulting in a rectourethral fistula, which required a repeat posterior sagittal dissection. One male, who had a redo procedure, developed a posterior diverticulum comparable to a large anal crypt. This was repaired prior to closure of the colostomy. One seven-year-old girl developed multiple rectocutaneous fistulae, which closed with conservative management in five months. One male infant developed a single supralevator rectocutaneous fistula, which closed after rediversion of feces with a colostomy and has remained so after colostomy closure. The majority of the complications encountered were probably preventable if careful attention to certain details of technique had been observed: careful padding of the groin areas when patients are prone, especially in older patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Anal Canal↗

Covered anus with anocutaneous fistula: the muscular sphincters.

A rare specimen of a covered anus with anocutaneous fistula obtained from a newborn female baby was subjected to macroexamination and microexamination to determine the anatomy of the sphincters and the levatores musculature. The internal sphincter and the deep voluntary sphincters, albeit slightly modified, were well developed but the sphincters in the perineum were absent or rudimentary. The pelvic muscles were present even in the absence of the sacrum up to and including the second sacral vertebra.

Anal Canal↗