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KMID : 0812020060120020170
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Korean Journal of Neurogastroenterology and Motility
2006 Volume.12 No. 2 p.170 ~ p.176
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A Case of Zonal Adult Hirschsprung`s Disease
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Kim Yun-Soo
Lee Joon-Seong
Kim Kyung-Min
Lee Bo-Young
Kim Yong-Dae
Jung In-Seop
Ko Bong-Min
Hong Su-Jin
Ryu Chang-Beom
Kim Jin-Oh
Cho Joo-Young
Lee Moon Sung
Jin So-Young
Kim Jae-Joon
Shim Chan-Sup
Kim Boo-Sung
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Abstract
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Hirschsprung’s disease is associated with congenital megacolon of the colon proximal to a segment that lacks ganglion cells in the myenteric plexus and submucosal plexus. Hirschsprung’s disease is usually considered as a pediatric disease entity. In the typical cases, the aganglionosis arises from the anal verge and this extends proximally for varying distances; the lesion mainly involves the rectum or rectosigmoid colon. Zonal segmental involvement is very rare even in children. We experienced a 45-year-old male who suffered from acute intestinal obstruction with a collapsed luminal portion in the distal descending colon and proximal sigmoid colon. The patient underwent surgical resection for segmental constriction. We present here a case of zonal adult Hirschsprung’s disease along with a review of the literature. (Kor J Neuro-gastroenterol Motil 2006;12:170-176)
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KEYWORD
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Hirschsprung`s disease, Megacolon, Aganglionosis
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