Hirschsprung's disease, associated rare congenital anomalies.

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dc.contributor.authorDas, Ken_US
dc.contributor.authorAlladi, Aen_US
dc.contributor.authorKini, Uen_US
dc.contributor.authorBabu, M Ken_US
dc.contributor.authorD'Cruz, A Jen_US
dc.date.accessioned2001-09-24en_US
dc.date.accessioned2009-05-30T11:10:39Z
dc.date.available2001-09-24en_US
dc.date.available2009-05-30T11:10:39Z
dc.date.issued2001-09-24en_US
dc.description.abstractOBJECTIVE: Hirschsprung's disease may be associated with a number of congenital anomalies of which Down's syndrome and intestinal atresias are commonly encountered. The study aimed to assess the impact of rare associated anomalies on the diagnosis and management of Hirschsprung's disease. METHODS: A retrospective review of the clinical presentation, diagnosis and outcome of thirty five consecutive newly diagnosed cases of Hirschsprung's disease encountered over two years was performed. RESULTS: Besides Down's syndrome (two), intestinal atresia (one) and pigmentary ocular defects (two), three rare anomalies (Occipital meningocele, Calcific meconium cyst with anal stenosis, Malrotation) were encountered in four of thirty five cases. The clinical features, radiologic anatomy and gross morphology of the bowel were unconventional and the diagnosis was supported by intraoperative acetylcholinesterase staining of biopsies. Though the diagnosis was relatively delayed in these cases, the outcome has been comparable to the rest. The dilemma in their diagnosis and management and their possible pathoembryology is discussed. CONCLUSION: Awareness of such associations and a specific investigative protocol is imperative for timely diagnosis and minimal morbidity in complex presentations of Hirschsprung's disease.en_US
dc.description.affiliationDepartment of Pediatric Surgery, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bangalore-560034, India.en_US
dc.identifier.citationDas K, Alladi A, Kini U, Babu MK, D'Cruz AJ. Hirschsprung's disease, associated rare congenital anomalies. Indian Journal of Pediatrics. 2001 Sep; 68(9): 835-7en_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/79407
dc.language.isoengen_US
dc.source.urihttps://medind.nic.in/icb/icbai.shtmlen_US
dc.subject.meshAbnormalities, Multiple --diagnosisen_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshFemaleen_US
dc.subject.meshHirschsprung Disease --complicationsen_US
dc.subject.meshHumansen_US
dc.subject.meshInfanten_US
dc.subject.meshInfant, Newbornen_US
dc.subject.meshMaleen_US
dc.subject.meshRetrospective Studiesen_US
dc.titleHirschsprung's disease, associated rare congenital anomalies.en_US
dc.typeCase Reportsen_US
dc.typeJournal Articleen_US
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