title: Congenital visceral myopathy with a predominantly hypertrophic pattern treated by multivisceral transplantation.
abstract: Chronic intestinal pseudoobstruction due to visceral myopathy may cause intestinal failure. The anatomical pathology of visceral myopathy is dominated by an atrophic muscularis propria. We present a case that is overwhelmingly dominated by a hypertrophic muscularis propria. The hypertrophic pattern was documented from ages 2 to 28 years. Most descriptions of visceral myopathy focus on the atrophic pattern of injury to the muscularis propria, typified by vacuolar degeneration of myocytes, loss of muscle fibers, and frequently with a honeycomb pattern of fibrosis. Hypertrophic changes can be observed in visceral myopathy, but the hypertrophy typically represents only a minor feature, typically limited to the internal layer of the muscularis propria. In our patient, the observed muscular hypertrophy differed from most descriptions because it (a) was the dominant pattern of injury and (b) involved both layers of muscularis propria rather than just the inner circular layer. The patient had intestinal failure and the complications induced cirrhosis, eventually treated by multivisceral transplantation to correct the intestinal failure and cirrhosis.
authors.affiliation: Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at the University of California at Los Angeles, Los Angeles, CA 90095, USA.
authors.forename: Stephen
authors.initials: S
authors.lastname: Koh
authors.forename: Robert F
authors.initials: RF
authors.lastname: Bradley
authors.forename: Samuel W
authors.initials: SW
authors.lastname: French
authors.forename: Douglas G
authors.initials: DG
authors.lastname: Farmer
authors.forename: Galen
authors.initials: G
authors.lastname: Cortina
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006984:Hypertrophy; D007418:Intestinal Pseudo-Obstruction / Q000151:congenital / Q000473:pathology* / Q000601:surgery; D007422:Intestines / Q000637:transplantation; D008103:Liver Cirrhosis / Q000209:etiology / Q000473:pathology / Q000601:surgery; D016031:Liver Transplantation; D009092:Mucous Membrane / Q000473:pathology; D009130:Muscle, Smooth / Q000473:pathology*; D009135:Muscular Diseases / Q000151:congenital / Q000473:pathology* / Q000601:surgery; D016035:Pancreas Transplantation; D016562:Parenteral Nutrition, Home Total; D014184:Transplantation, Homologous; D019737:Transplants