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Sacral anomaly and pelvic floor muscle in imperforate anus: a clinical and experimental study.

Influences of sacral anomalies on the postoperative continence of imperforate anus were evaluated clinically and experimentally. Postoperative continence was clinically assessed by the quantitative scoring system for 77 patients (44 of high-type anomalies and 33 of low-type anomalies). Incidence of combined sacral anomalies was 28.6% (38.6% in high type anomalies and 15.2% in low type anomalies). Combined sacral anomalies had harmful effects on the postoperative defecational function, especially on the parameters of rectal sensation and soiling. Patients with more than four segments of sacral vertebrae had relatively satisfying results on the postoperative assessment. For a teratological study to obtain a model of sacral anomalies, Donryu rats were used. Trypan blue was injected as the teratogen intraperitoneally on the nineth day of gestation. Of the 198 live fetuses, 5 rats had tail defect and sacral anomalies. Two of the 5 anomalous rats also had imperforate anus. Four of 5 rats had vertebrae intact above the first sacral segment. In these 4 rats the pelvic floor muscles had developed to some degree. All the vertebrae were agenetic below the thoracic segments in the other rat fetus. This rat had no pelvic floor muscles. These studies showed that the severity of sacral anomalies had a relationship to postoperative continence. Intact sacral vertebrae above the 4th in humans and the 1st in rats correlated with fair development of pelvic floor muscles and satisfying postoperative continence.

Animals↗

Urologic implications of imperforate anus.

Recent clinical studies have documented the high association of genitourinary and lumbosacral spine anomalies with imperforate anus. Many of these associated anomalies may go undetected unless special diagnostic studies are performed, and these genitourinary anomalies can be the cause of significant clinical morbidity in later life if not managed properly initially. All neonates with imperforate anus should therefore undergo radiographic examination with lower spine films, intravenous pyelography and voiding cystourethrography, and cystoscopy should be reserved for those with cloaca deformity or those whose x-ray films demonstrate urinary anomalies requiring further workup. Significant morbidity involving the genitourinary tract may be encountered after surgical treatment of the anorectal anomaly, particularly if total anorectal reconstructions are performed in the neonatal period.

Allantois↗

Findings of pelvic musculature and efficacy of laparoscopic muscle stimulator in laparoscopy-assisted anorectal pull-through for high imperforate anus.

BACKGROUND: Laparoscopic findings of levator muscle and the efficacy of laparoscopic muscle stimulator (LMS) in infants with high imperforate anus have not been reported. METHODS: Twelve patients underwent laparoscopically assisted anorectoplasty for high imperforate anus. Following laparoscopic dissection of the distal rectum and division of the fistula, levator muscles in the pelvic floor were stimulated with a 5-mm-diameter LMS. Dilatation was done by inserting a guidewire and balloon catheter through the center of the levator muscle sling and muscle complex. Rectal pull-through and anastomosis between the rectum and anus were successfully completed. RESULTS: LMS showed good contraction of levator muscles and enhanced accurate midline placement of pull-through rectum. LMS was particularly useful in observing weak muscles in infants with rectovesical fistula. CONCLUSIONS: Laparoscopy and LMS offer excellent visualization of the pelvic musculature and precise tract of rectal pull-through. Fecal continence will be assessed by long-term follow-up.

Anal Canal↗

The posterior sagittal approach to repair of vaginal atresia and imperforate anus.

In 1982 deVries and Peña reintroduced the posterior sagittal approach for the repair of high imperforate anus, and it is already being utilized by many pediatric surgeons worldwide. This approach, the so-called "Kraske approach," has been used in the past for access to difficult rectal lesions, and has been applied to cloacal anomalies, again by Peña and deVries. We have recently applied this technique to repair a mid-vaginal atresia in a 12-year-old girl who also had chronic constipation and fecal incontinence following neonatal imperforate anus repair. The posterior sagittal approach allowed simultaneous reconstruction of the anus and vagina and may have application to the primary repair of ano-vaginal anomalies in infancy. The case history and a discussion of the technique form the basis of this report.

Abnormalities, Multiple↗

Results after posterior sagittal anorectoplasty: a new approach to high imperforate anus.

Six patients, 3 to 12 months of age have undergone posterior sagittal anorectoplasty for high imperforate anus. A rectourethral fistula was present in all patients. The operative procedure performed was the method of de Vries and Pena, utilizing electrostimulation and identification of the external sphincter muscles. One patient required an abdominal operation as well as the perineal approach. There were no complications. Serial assessment was carried out over a period of 4 months to 2 1/2 years. Normal continence was achieved in three patients. Anorectal manometry was performed in five patients. Four of five patients had normal rectal sensation and normal mean anal canal pressures. These results suggest that this procedure is applicable to the young infant with high imperforate anus, and a satisfactory result can be anticipated.

Anal Canal↗

A case of acrocephalosyndactyly with low imperforate anus.

The authors report a case of a female acrocephalosyndactyly with imperforate anus without fistula, which is rare in girls. Acrocephalosyndactyly is characterized by premature closure of the sutures (craniosynostosis) and fusion or webbing of hands and feet (syndactyly). The most general types of the syndrome are the Apert syndrome and the Pfeiffer syndrome. They usually have some fibroblast growth factor receptor (FGFR) gene mutations, so that acrocephalosyndactyly is thought to be involved in "FGFR-related craniosynostosis." To the authors' knowledge, only 4 cases of anorectal anomaly in acrocephalosyndactyly have been reported in the world. The relationship between anorectal anomaly and the FGFR gene is not clear now, but might be clarified in the future.

Acrocephalosyndactylia↗

Congenital short colon associated with imperforate anus (Zachary-Morgan syndrome).

Two cases of short colon with imperforate anus are presented. Terminal colostomy is the therapy of choice in the neonatal period. As the definitive final procedure, trimming into a tubular structure and abdomino-perineal pull-through operation is suggested. A survey is given of similar cases reported in the literature.

Anus, Imperforate↗