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Familial visceral myopathy. Evidence of diffuse involvement of intestinal smooth muscle.
Am J Surg Pathol. 1987 Nov; 11(11):846-54.AJ

Abstract

We report the histologic and ultrastructural findings on two sisters with familial visceral myopathy who presented with acquired megacolon that necessitated subtotal colectomy. Both patients were mentally retarded and had repeated episodes of constipation and fecal impaction. Each presented near the age of 30 with massive dilatation of the colon and without clinical evidence of small intestinal involvement. Histologic abnormalities primarily involved smooth muscle and included marked nuclear enlargement and irregularity, interstitial fibrosis, and cytoplasmic vacuolation. These changes were most severe in the muscularis propria, but similar abnormalities were found in the muscularis mucosae and blood vessels. In the most advanced stages, collagen had completely replaced the muscularis propria, with extreme thinning of the intestinal wall. Abnormalities were noted in all segments of the colon and the appendix, but there was little correlation between severity of involvement and the segment examined. This study not only confirms the variable nature of morphologic changes in familial visceral myopathy, but also provides evidence of more extensive involvement of intestinal smooth muscle than has been previously reported.

Authors+Show Affiliations

Department of Surgical Pathology, L.A. County--USC Medical Center 90033.No affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

3674282

Citation

Fitzgibbons, P L., and P T. Chandrasoma. "Familial Visceral Myopathy. Evidence of Diffuse Involvement of Intestinal Smooth Muscle." The American Journal of Surgical Pathology, vol. 11, no. 11, 1987, pp. 846-54.
Fitzgibbons PL, Chandrasoma PT. Familial visceral myopathy. Evidence of diffuse involvement of intestinal smooth muscle. Am J Surg Pathol. 1987;11(11):846-54.
Fitzgibbons, P. L., & Chandrasoma, P. T. (1987). Familial visceral myopathy. Evidence of diffuse involvement of intestinal smooth muscle. The American Journal of Surgical Pathology, 11(11), 846-54.
Fitzgibbons PL, Chandrasoma PT. Familial Visceral Myopathy. Evidence of Diffuse Involvement of Intestinal Smooth Muscle. Am J Surg Pathol. 1987;11(11):846-54. PubMed PMID: 3674282.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Familial visceral myopathy. Evidence of diffuse involvement of intestinal smooth muscle. AU - Fitzgibbons,P L, AU - Chandrasoma,P T, PY - 1987/11/1/pubmed PY - 1987/11/1/medline PY - 1987/11/1/entrez SP - 846 EP - 54 JF - The American journal of surgical pathology JO - Am. J. Surg. Pathol. VL - 11 IS - 11 N2 - We report the histologic and ultrastructural findings on two sisters with familial visceral myopathy who presented with acquired megacolon that necessitated subtotal colectomy. Both patients were mentally retarded and had repeated episodes of constipation and fecal impaction. Each presented near the age of 30 with massive dilatation of the colon and without clinical evidence of small intestinal involvement. Histologic abnormalities primarily involved smooth muscle and included marked nuclear enlargement and irregularity, interstitial fibrosis, and cytoplasmic vacuolation. These changes were most severe in the muscularis propria, but similar abnormalities were found in the muscularis mucosae and blood vessels. In the most advanced stages, collagen had completely replaced the muscularis propria, with extreme thinning of the intestinal wall. Abnormalities were noted in all segments of the colon and the appendix, but there was little correlation between severity of involvement and the segment examined. This study not only confirms the variable nature of morphologic changes in familial visceral myopathy, but also provides evidence of more extensive involvement of intestinal smooth muscle than has been previously reported. SN - 0147-5185 UR - https://www.unboundmedicine.com/medline/citation/3674282/Familial_visceral_myopathy__Evidence_of_diffuse_involvement_of_intestinal_smooth_muscle_ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=3674282.ui DB - PRIME DP - Unbound Medicine ER -