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Management of imperforate anus at the Lagos University Teaching Hospital, Nigeria: a review of ten years' experience.

Sixty cases of imperforate anus treated at the Lagos University Teaching Hospital, Nigeria between 1969 and 1979 are reviewed. Late presentation at our hospital was a remarkable feature. Although there were only 18 attempts at Wangensteen-Rice (Wangensteen and Rice 1930) inversion radiograms using all the information available, it was possible to categorize 27 children as having high and 17 as having a low-type imperforate anus. Only 16 of the children with high lesions have had abdominoperineal pull-through procedures with an operative mortality of 31%. Eleven definite procedures for low lesions were carried out in newborn infants. The rest were decompressed either by a colostomy or a congenital fistula and are waiting for definite surgery. One child died before any surgical treatment. Some of the problems related to the outcome of treatment of this disease in our hospital are late presentation, inadequate facilities and shortage of specialized personnel, problems peculiar to a developing country. We suggest an urgent colostomy performed under local anaesthesia as a safe and expedient treatment for babies with obstruction and in whom the presence of a translevator anomaly cannot be immediately verified.

Anus, Imperforate↗

The split notochord syndrome with dorsal enteric fistula, meningomyelocele and imperforate anus.

A male infant was referred to our department because of lumbosacral meningomyelocele, dorsal enteric fistula and imperforate anus. The mother had received a parenteral drug containing estradiol benzoate and progesterone for inducing abortion in the first trimester. She also used an anal pomade containing triamcinolone and lidocaine-HCl during the pregnancy for hemorrhoids. Sigmoid end colostomy was performed after meningomyelocele repair. On abdominal exploration a wandering spleen was detected but no other anomalies. Two months later, an abdominoperineal pullthrough was performed, and the patient was discharged well after three weeks. Our case is the sixth that had split notochord syndrome associated with dorsal enteric fistula and imperforate anus. Additionally, penoscrotal transposition and wandering spleen were present in this case. To our knowledge, these associated anomalies have been extremely rare.

Abnormalities, Multiple↗

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.

Abnormalities, Multiple↗

Cryptorchidism in boys with imperforate anus.

In a retrospective study of the case reports of 136 boys who were operated on for an imperforate anus and who survived at least 18 months, cryptorchidism was the most common associated anomaly, involving 26 cases (19%). The higher the level of the anorectal malformations, the higher was the incidence of cryptorchidism. The incidence of renal and ureteric malformations and dysplasias showed a parallel tendency. The incidence of vertebral malformations and dysplasias in the T10-S5 area was low among patients with a covered anus or a perineal fistula. Cryptorchidism was found associated with urological and with T10-S5 vertebral malformations and dysplasias. Recognition of this association is probably new. The histopathological findings of testicular biopsy specimens and the location of the undescended testes in patients with an imperforate anus showed the same pattern as seen in undescended testes from patients with cryptorchidism only. These findings, together with the existing literature on the subject, indicate that further studies on the association of cryptorchidism, urological, and T10-S5 vertebral malformations and dysplasias may be very helpful toward a better understanding of cryptorchidism in general.

Abnormalities, Multiple↗

Familial imperforate anus.

A boy, 2 years 5 months of age, was admitted for examination and was found to be mentally retarded. He had a history of low birth weight, covered anus, and perineal fistula. Investigation revealed ventricular septal defect and persistent left superior vena cava, left upper lobe atelectasis, hand and foot anomalies, agenesis or nonfunctioning right kidney and large left kidney with double pelves and double ureters, and large sigmoid colon. Family history revealed consanguineous parents and a mentally retarded elder sister who had anorectal atresia with introital fistula and perineal ectopic stenotic anus. This family demonstrates two extremes of the same syndrome, most probably inherited as an autosomal-recessive entity.

Abnormalities, Multiple↗

Imperforate anus.

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Age Factors↗

Congenital hypothalamic hamartoblastoma, hypopituitarism, imperforate anus, and postaxial polydactyly--a new syndrome? Part II: Neuropathological considerations.

Unusual and virtually identical hypothalamic tumors were recently studied in three unrelated neonates with a similar complex malformation syndrome. Previous reports of either the hypothalamic tumors or the syndrome as a whole have not been found. Each tumor was apparent on the inferior surface of the cerebrum and extended from the optic chiasma to the interpeduncular fossa. The tumor replaced the hypothalamus and other nuclei which originate in the embryonic hypothalamic plate; it was principally composed of cells resembling primitive, undifferentiated germinal cells. The term "hamartoblastoma" is used to designate these tumors in order to emphasize the malformational and neoplastic aspects. In addition, short olfactory tracts suggest a relation to the arrhinencephaly field defect.

Anus, Imperforate↗