Zonal colonic aganglionosis, a variant of Hirschsprung's disease.
نویسندگان
چکیده
منابع مشابه
Segmental aganglionosis in Hirschsprung's disease in newborns - a case report.
Segmental aganglionosis Hirschsprung's is an extremely rare condition. Described as a segment of normally ganglionated bowel surrounded proximally and distally by aganglionosis, zonal aganglionosis is of interest because it may cause confusion in interpreting surgical margins. Diagnosis of segmental lesions in Hirschsprung's disease may be missed as it is rarely suspected at initial surgery. We...
متن کاملZonal adult Hirschsprung's disease.
BACKGROUND Hirschsprung's disease is a congenital disorder which is rare in adulthood. In typical cases the aganglionosis involves mainly the rectum or rectosigmoid colon and the lesion starts from the anal valve. Zonal segmental aganglionosis is a very rare type even in children. PATIENT A 54 year old women with zonal segmental aganglionosis had an aganglionic segment 18 cm in length located...
متن کاملHirschsprung's disease - Postsurgical intestinal dysmotility
OBJECTIVE To describe the case of an infant with Hirschsprung's disease presenting as total colonic aganglionosis, which, after surgical resection of the aganglionic segment persisted with irreversible functional intestinal obstruction; discuss the difficulties in managing this form of congenital aganglionosis and discuss a plausible pathogenetic mechanism for this case. CASE DESCRIPTION The ...
متن کاملHirschsprung's disease associated with a deletion of chromosome 10 (q11.2q21.2): a further link with the neurocristopathies?
We report a patient with total colonic aganglionosis in association with a deletion of part of the long arm of chromosome 10: (del(10)(q11.2q21.2)). This deletion includes the ret proto-oncogene, which has recently been implicated in multiple endocrine neoplasia type 2A (MEN 2A). The possible links between Hirschsprung's disease and the neurocristopathies and the aetiological role of abnormalit...
متن کاملOutcomes of Hirschsprung's disease associated with Mowat-Wilson syndrome.
PURPOSE Mowat-Wilson syndrome (MWS) is a developmental disorder presenting with mental retardation, delayed motor development, and a wide spectrum of clinical features. Hirschsprung's disease (HD) is associated in almost 50% of cases. This report aims to analyze the course of HD and to evaluate the clinical outcomes of these patients. PATIENTS AND METHODS Between 1997 and 2007, 110 patients p...
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ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 47 252 شماره
صفحات -
تاریخ انتشار 1972