Hirschsprungs Disease in An Adult Male - An Unusual Presentation
International Journal of Science and Research (IJSR)

International Journal of Science and Research (IJSR)
www.ijsr.net | Open Access | Fully Refereed | Peer Reviewed International Journal

ISSN: 2319-7064



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Case Studies | Medical Surgical | India | Volume 6 Issue 12, December 2017

Hirschsprungs Disease in An Adult Male - An Unusual Presentation

Bhabani Sankar Sahoo, Rajat Karma, Shipra Singhal, Arun Chhetri

Hirschsprungs Disease (HD) is a rare congenital disease where aganglionosis of the submucosal & myenteric neural plexuses principally affects the rectosigmoid or rectal segments of varying length. The vast majority of cases of HD are recognized in newborns or infants but a few cases with a milder form elude surgery until adolescence or adulthood. HD in adult is a rare & misdiagnosed cause of long-standing refractory constipation. We report a case of HD in a 32 year old man who presented to the emergency department with absolute constipation, severe abdominal distension & features consistent with large bowel obstruction. Operative findings were suggestive of HD, recto-colonic resection & diversion colostomy was done and diagnosis was confirmed as colonic aganglionosis on biopsy. The diagnosis of HD in adult is difficult due to the rarity of the disease & the incidence of short & ultra short disease with mild symptoms. The patients with long standing history of undiagnosed constipation since infancy & childhood could be suffering from HD. HD should be suspected in adults with a chronic history of constipation. Whenever reasonable doubt is present, biopsies should be taken.

Keywords: Hirschsprungs disease, chronic constipation, aganglionosis, adult, Duhamel procedure, negative calretinin staining

Edition: Volume 6 Issue 12, December 2017

Pages: 872 - 878

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How to Cite this Article?

Bhabani Sankar Sahoo, Rajat Karma, Shipra Singhal, Arun Chhetri, "Hirschsprungs Disease in An Adult Male - An Unusual Presentation", International Journal of Science and Research (IJSR), https://www.ijsr.net/search_index_results_paperid.php?id=ART20178428, Volume 6 Issue 12, December 2017, 872 - 878

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