Visceral Myopathy of Intestinal Pseudoobstruction

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Song, Sang-Yong; Chi, Je G.; Seo, Jeong Kee; Choi, Jung-Hwan; Park, Kwi Won
Issue Date
Seoul National University College of Medicine
Seoul J Med 1994;35(2):113-122
Intestinal pseudoobstructionVisceral myopathyMegacystis microcolon intestinal hypoperistalsis syndromeChildhood
Intestinal pseudoobstruction is a syndrome complex caused by a variety
of disorders of various etiology. It can be classified pathologically as visceral myopathy
and visceral neuropathy. The sporadic form of visceral myopathy is characterized
histologically by vacuolar degeneration and fibrosis of smooth muscle but differs from
the familial form only by the absence of other affected family members. We studied 6
cases with intestinal pseudoobstruction classified as sporadic visceral myopathy. They
were four boys and two girls, and were two neonates, two infants and two children. The
duration of symptoms ranged from two days to two years. Two babies were dead from
pneumonia and sepsis. Others were alleviated after surgical resection of the bowel.
Both small and large intestines were found affected in autopsy cases. Histopathologic
features were vacuolar degeneration of muscularis propria, disproportionate hypoplasia
of outer muscle layer, abnormal muscle direction of muscularis propria, submucosal
and/or interstitial fibrosis and extra muscle layering. It is presumed that a variety of
histopathologic features accounts for visceral myopathy of intestinal pseudoobstruction.
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 35 No.2 (1994)
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