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Treatment and functional results of anorectal continence in children with imperforate anus.

The results of 172 patients with imperforate anus are reported. In these patients 215 operations where performed. The complications, lethality and the results concerning anorectal continence are analysed. The lethality was 58% in the risk group A (high risk), 27% in group B and 3% in C group (low risk). The overall lethality was 16.6%. Specific complications were anal stenosis (15.5%), relapse of anorectal or anovaginal fistulas (7%) and mucosa prolapse (5%). Anorectal continence was evaluated according to the Kelly score of continence and to our own electromanometric and clinical score. Five years after the operation 50% of the patients with high type, and 14% of those with low type imperforate anus remained incontinent. Only 15% of the high anal atresias and 43% of the low forms became continent. Thirty-five percent of the children with a high anorectal malformation and 43% with a low type acquired a partial continence with small amounts of soiling. The current surgical techniques to improve anorectal continence are discussed: Pickrell's gracilis transplantation and its modifications, the free muscle transplantation according to Hakelius and Grotte, the free smooth muscle transplantation according to Schmidt and the reverse smooth muscle transplantation according to Holschneider and Hecker. Clinical and electromanometrical as well as electromyographical results are presented.

Anus, Imperforate↗
PubMed1989

Ultrasound of the distal pouch in infants with imperforate anus.

Optimal surgical management of the newborn with imperforate anus depends on accurate determination of the level of the rectal pouch. Eighteen children with imperforate anus were evaluated with ultrasound. The distance from the end of the pouch to the perineum was measured. Ultrasound correctly predicted the level of the distal pouch in all 12 children who had confirmation of the pouch level by surgery or by distal contrast stomagrams. Six children have not yet had definitive surgery. Five children with a pouch to perineum (P-P) distance of less than or equal to 10 mm and three of six children with a P-P distance of 10 to 15 mm had successful correction by a simple perineal anoplasty. Three of six children with a P-P distance of 10 to 15 mm and all of those (seven) with a P-P distance greater than 15 mm were diverted with colostomies.

Anus, Imperforate↗
PubMed2001

Folic acid supplementation and risk for imperforate anus in China.

Maternal consumption of folic acid before pregnancy and during early pregnancy is associated with a reduced risk for some birth defects. Whether folic acid can reduce the risk for imperforate anus is unknown. As part of a public health campaign conducted in China from 1993 through 1995, the outcomes of pregnancies of > or =20 weeks' gestation were evaluated among women using folic acid supplements. The women were asked to take one pill containing 400 microg of folic acid (without other vitamins) every day from the time of their premarital examination until the end of their first trimester of pregnancy. Rates of imperforate anus and risk ratios for imperforate anus among the offspring of these women were calculated according to folic acid use. Among the offspring of women who took folic acid and women who did not take folic acid, 20 and 30 infants with imperforate anus were identified, respectively. The rate of imperforate anus was 3.1 per 10,000 among the offspring of women who did not take folic acid and 1.6 per 10,000 among the offspring of women who took folic acid; adjusted for maternal age, the risk ratio was 0.59 (95% confidence interval: 0.33, 1.07). Daily maternal consumption of 400 microg of folic acid before and during early pregnancy may reduce the risk for imperforate anus.

Adult↗
PubMed2001

Three-flap anoplasty for imperforate anus: results for primary procedure or for redoes.

UNLABELLED: Three-flap anoplasty was first described in 1987 by Yazbeck for the treatment of rectal prolapse after pull-through operations for imperforate anus, and in 1992 in a case of anterior perineal approach. It is intended to reproduce the normal anatomy of a sensitive anal canal. The purpose of this study is to evaluate 14 children (9 boys and 5 girls) operated for imperforate anus. METHOD: Eleven children had an intermediate or high imperforate anus and 3 had a low imperforate anus. Nine were operated for the first time with a three-flap anoplasty (GrA), and 5 were reoperated with this technique because of fecal incontinence and/or rectal prolapse (GrB). Seven patients had associated malformations: one Hirschsprung's disease, one cloacal defect with renal insufficiency, three complex caudal malformations with tethered cord, one Down syndrome, and two psychological and psychomotor troubles. The patients (average age = 6 years) have been evaluated every year since 1997, with a questionnaire based on a clinical score validated with 30 healthy children, used as a control group. Ten anal manometries were carried out. RESULTS: In 1998, the GrA score was 16.1 (control = 22.5) and the GrB score was 11.5 (p = 0.25). In 1999, GrA and GrB score were approximately the same. The score of those without associated anomalies was 19.6 whereas the score of the children with other malformations or anomalies was 10 (p = 0.02). Anal manometry is almost normal in patients with low or intermediate imperforate anus (rectoanal relaxation reflex for 10 cm H2O, and basal resting pressure more than 40 cm H2O). Even though anal manometry is subnormal in patients with Down syndrome or psychomotor troubles, the clinical score remains low (score = 10). In cases of complex caudal malformations or high imperforate anus, the results of anal manometry and clinical score are bad (score 9.7). CONCLUSION: The three-flap anoplasty can be carried out in newborns without colostomy and often represents the only possible surgical approach to avoid a laparotomy. This plasty, proposed secondarily to correct a defect of continence, can be performed without colostomy, with satisfactory results.

Anus, Imperforate↗
PubMed2003

Imperforate anus: US determination of the type with infracoccygeal approach.

PURPOSE: To assess the usefulness of infracoccygeal transperineal ultrasonography (US) in differentiation between high- and low-type imperforate anus. MATERIALS AND METHODS: Infracoccygeal US was prospectively performed with a 7-10-MHz linear-array transducer prior to corrective surgery in 14 neonates with imperforate anus. The approach site was just inferior to the coccyx and posterior to the anus. Transverse images of the anorectal area were obtained. The puborectalis muscle was identified, and the relationship between the puborectalis muscle and the distal rectal pouch was evaluated. US findings were compared with surgical findings. RESULTS: In 10 neonates, a low-type imperforate anus was correctly diagnosed at infracoccygeal US. In those with low-type imperforate anus, the puborectalis muscle was seen as a hypoechoic U-shaped band (n = 10), and the distal rectal pouch passed through the puborectalis muscle (n = 10). In four neonates with high-type imperforate anus, the puborectalis muscle was not identified (n = 4). CONCLUSION: Infracoccygeal transperineal US enables the determination of the type of imperforate anus.

Anus, Imperforate↗
PubMed1983

The anterior perineal approach for pull-through operations in high imperforate anus.

Recent widespread interest in a new approach to imperforate anus is ample testimony to the difficulty of the classical operations and to the unsatisfactory results. In the early seventies, Mollard began to use an anterior perineal approach to high imperforate anus as an alternative to the sacrococcygeal pull-through. It entails a semicircular retroscrotal incision allowing direct access and visualization of the urethra and puborectalis sling. Since 1976 we have switched to the anterior perineal approach. We now have experience with 30 patients, 27 males and 3 females. Two of the females had complicated cloacal anomalies, and three teenage patients were reoperated for incontinence following previous unsuccessful pull-throughs. Of the 27 primary operations, there were 17 with an uneventful early postoperative course. There were two major and eight moderate or minor complications. There was no mortality. A 3-year follow-up is available in 14 patients. The results are good in 8, fair in 4 and poor in 2. The advantages of this technique are threefold: it allows clear visualization and precise identification of the puborectalis sling; the dissection proceeds parallel to and with constant vision of the urethra, avoiding injury to it; and it obviates the need to change the position of the patient during the procedure. This operation is easier and safer than the sacral approach, it avoids cutting through the puborectalis sling as recently described, and, finally, it respects the criteria established by Stephens.

Anus, Imperforate↗
PubMed1991

Urodynamic evaluation of the patient with an imperforate anus: a prospective study.

Prospective urodynamic evaluation of 14 patients with an imperforate anus revealed detrusor-sphincter dyssynergia in 4. Subsequent contrast voiding cystourethrograms demonstrated radiographic changes in all 4 cases, consisting of bladder trabeculation, new onset of vesicoureteral reflux or hydroureteronephrosis. Of the 4 patients 3 had a high (supralevator) imperforate anus, while 1 had a low (infralevator) lesion. Two patients had bony vertebral abnormalities and 2 had normal plain radiographs and magnetic resonance imaging of the spine. Therefore, early urodynamic study is recommended for all patients with an imperforate anus and it should complement neonatal uroradiographic evaluation. The finding of detrusor-sphincter dyssynergia should alert the urologist to the need for adjunctive management.

Abnormalities, Multiple↗
PubMed1990

Association of imperforate anus with short colon: a report of eight cases.

Eight cases of imperforate anus with short colon were seen in our hospital from April 1982 to December 1987. Five were boys, three were girls, and their ages ranged from 2 days to 2 years. The international literature about this kind of disease was reviewed. The disease's name, embryology, diagnoses, differential diagnosis, treatment, and prognosis are discussed herein. We suggest that this case, which was not combined with exstrophy of the bladder and/or intestine, be called association of imperforate anus with short colon (AIASC). Other cases, combined with exstrophy of the bladder and/or intestine could be called, association of imperforate anus with exstrophy splanchnica (AIAES). This distinction is necessary because each group differs in symptoms, signs, diagnosis, treatment, and prognosis.

Anal Canal↗
PubMed1991

Occult neurovesical dysfunction in children with imperforate anus and its variants.

Neurovesical dysfunction (NVD) is frequently encountered in children with imperforate anus and its variants. Such functional urologic problems are often compounded by associated anatomic urologic abnormalities that in combination may profoundly alter the course and prognosis of children with imperforate anus. Herein, we report 16 such cases. Management of NVD in children with imperforate anus offers several unique challenges that require important alterations in management. Specific recommendation are presented.

Anus, Imperforate↗
PubMed2002

Routine MRI evaluation of low imperforate anus reveals unexpected high incidence of tethered spinal cord.

BACKGROUND/PURPOSE: Previous clinical practice has included evaluation for the presence of tethered cord in those children who have imperforate anus with a high lesion. To define the incidence in children with low lesions, the authors reviewed their experience with a protocol employing routine magnetic resonance imaging (MRI), regardless of the level of the lesion, to determine the presence of a tethered cord in all children with imperforate anus. METHODS: A retrospective review of children with imperforate anus was conducted over the last 13 years at our institution. Lesions were categorized as high versus low based on the supralevator or infralevator position of the fistula. RESULTS: Sixty-three patients completed evaluation for a tethered cord. Twenty-two (34.9%) of these 63 patients had a tethered cord: 11 of 41 (26.8%) patients with high lesions and 11 of 22 (50.0%) of those with low lesions. Of those children with a low lesion, 83% of the boys had a tethered cord, whereas 38% of the girls had a tethered cord. Forty-five percent of the patients with low lesions and a tethered cord did not have any other lumbosacral anomalies. All 22 children with a tethered cord underwent surgical release. CONCLUSIONS: The incidence of tethered cord in children with low lesions of imperforate anus is not lower than those with high lesions. The authors advocate early evaluation of all children with imperforate anus for a tethered cord.

Abnormalities, Multiple↗
PubMed2006

Imperforate anus and colon atresia in a newborn.

Imperforate anus is an uncommon congenital anomaly. Colon atresia is even more infrequent. This report describes a newborn with the simultaneous occurrence of these 2 anomalies, a condition that is exceedingly rare.

Abnormalities, Multiple↗
PubMed1978

Imperforate anus: avoiding a colostomy.

Early assessement of imperforate anus, combining clinical and radiographic data, determines the course of treatment. "High" lesions most often require combined abdominal and sacroperineal repair, usually preceded by a decompression colostomy. "Low" lesions are repaired through a perineal or sacroperineal approach not requiring a previous colostomy. Patients who do not have an apparent fistulous tract through which accurate radiographic assessment can be made frequently undergo preliminary colostomy to avoid colonic perforation. This occurs in patients who have "low" lesions and in whom colostomy may be avoided. Combining a new procedure and an old procedure may avoid unnecessary colostomy. A 14 Medicut intravenous cannula is inserted in the perineum and under fluoroscopic control is advanced into the radiolucent area identified in the pelvis as the probable lower rectal pouch. The position is confirmed by aspiration of gas or meconium and injection of contrast material. A limited contrast enema will clearly identify the level of the lesion and identify a fistula if one is present. If a low lesion is identified and no fistula is present to allow temporary colonic decompression, a fistulous tract may be created mechanically. A No. 4 Fogarty catheter is inserted through the previously placed cannula, the catheter balloon is inflated, and the entire apparatus is extracted under tension. This creates a small fistulous tract to the perineal surface. Subsequent dilation of this tract allows colonic decompression, and definitive operative repair may be planned at the most appropriate time. By employing this technique, we have avoided colostomy in 4 of 6 patients.

Anus, Imperforate↗
PubMed1987

Mucocele after endorectal pull-through for imperforate anus.

This report documents a patient with imperforate anus in whom an endorectal pull-through with incomplete excision of the macroscopic mucosa led to the formation of a 1,500 mL mucosal lined pelvic mucocele, obstructing both the rectum and ureter. All macroscopic mucosa should be removed during endorectal pull-through.

Anus, Imperforate↗
PubMed1976

Imperforate anus.

Sucessful treatment of the child with imperforate anus yields an individual with satisfactory continence and a healthy urinary tract. These goals are achieved by accurate evaluation of the newborn, optimal technical results, and conscientious follow-up. The infant born with one of these malformations frequently has a serious associated anomaly which also requires early specific diagnosis and management. When the bowel has passed through the puborectalis in utero, the deformity can often be definitively treated in the neonate by dilations or anoplasty. However, if a urinary or high vaginal fistula is present, or if the anomaly is of the supralevator type, definitive surgery is best postponed some months and the neonate given a colostomy. We have found the sacroabdominoperitoneal approach to be the most satisfactory pullthrough procedure for the supralevator lesion. The children with high, supralevator anomalies have higher mortality rates and, in one out of four cases, have suboptimal fecal continence. They require the utmost in technical skill and continuing care if they are to lives free of social stigma. The mortality rate in all other types is low and the functional continence uniformly acceptable.

Abnormalities, Multiple↗
PubMed2006

Parental experiences: care of children with high and intermediate imperforate anus.

In this study parental experiences of care of children with high and intermediate imperforate anus were evaluated. A group of 45 parents of children with high and intermediate imperforate anus and two control groups participated. Data collection with individual questionnaires concerning the child's hospital care, information to the parent and the child, and involvement in the care of the child were performed. Parents of children with imperforate anus reported being less satisfied with the care of their child, and they were less content with information about their child's treatment compared with the control groups. The parents had been extremely involved in the follow-up treatment. Constipation and fecal incontinence are common and involve suffering for the children and their parents. Parents have to be motivated and supportive and have a great deal of patience to be able to put up with caring for these children, and it seems as if health care professionals have underestimated their problems.

Adaptation, Psychological↗
PubMed1987

Neuroradiologic evaluation of sacral abnormalities in imperforate anus complex.

Neurologic impairment and sacral spinal anomalies are a finding in children with imperforate anus. The neurologic deficits previously had been considered static rather than progressive. Recent experience at this institution has documented the frequent association of imperforate anus patients having bony sacral and other spinal deformities with progressive neurologic dysfunction. These deficits are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. To investigate the nature and frequency of such lesions, spinal cord imaging was done in these patients. From July 1976 to June 1985, 106 patients with imperforate anus were screened with plain radiographs of the sacral spine; 37 (35%) patients had abnormalities. Of these, 26 have been evaluated with a variety of spinal cord imaging techniques, including magnetic resonance imaging (18), digital metrizamide myelotomography (14), computed tomography (10), metrizamide myelography (5), or ultrasound (4). Four patients were lost to follow-up, and seven have not yet been studied. Fifteen of the 26 patients (53%) had one or more abnormal studies. Thirteen were considered to have progressive dysfunction with operable lesions and have undergone neurosurgical correction. At follow-up, there was improved leg function in five patients and improved bladder function in two patients. No patient had additional neurologic deficits after surgery. Because of the high incidence of progressive but potentially correctable myelodysplasias in patients with coexisting anorectal and sacral anomalies, routine radiographic screening of the spine is recommended, followed by spinal cord imaging and neurosurgical evaluation if warranted.

Abnormalities, Multiple↗
PubMed1977

Imperforate Anus: a five to thirty year follow-up perspective.

A twenty-five year experience with 284 patients with imperforate anus has been reviewed, with a 5 to 30 year assessment after surgical intervention. The following conclusions seem to be suggested by the study: 1. There was a 20% mortality overall, with the greatest number of these occurring in the Type III high lesions. Eighty per cent of the deaths were unrelated to imperforate anus and resulted from associated anomalies. 2. Eighty-eight per cent of the overall series achieved socially acceptable continence. Four out of five poor results occurred in the Type III high lesions. 3. Functional results achieved in imperforate anus surgery seem more closely related to the anatomy presented to the surgeon than by his choice of operative procedure. 4. Continence is more slowly achieved in an imperforate anus patient than in a normal child. It is an evolutionary process which seems to improve with age, being especially influenced by the social motivation that comes with puberty. 5. An appreciable number of patients with poor musculature for continence remain clean by promptly heeding the defection signals, by avoiding laxative foods, and by enforced constipation. 6. Secondary surgery when the levator sling muscles have been missed or partially bypassed seems to improve continence in a high percentage of patients.

Adolescent↗