title: Abnormal layering of muscularis propria as a cause of chronic intestinal pseudo-obstruction: A case report and literature review.
abstract: Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscularis propria is extremely rare. We report a case of a 9-mo-old Thai male baby who presented with chronic intestinal pseudo-obstruction. Histologic findings showed abnormal layering of small intestinal muscularis propria with an additional oblique layer and aberrant muscularization in serosa. The patient also had a short small bowel without malrotation, brachydactyly, and absence of the 2(nd) to 4(th) middle phalanges of both hands. The patient was treated with cisapride and combined parenteral and enteral nutritional support. He had gradual clinical improvement and gained body weight. Subsequently, the parenteral nutrition was discontinued. The previously reported cases are reviewed and discussed.
authors.affiliation: Napat Angkathunyakul, Department of Pathology, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.
authors.forename: Napat
authors.initials: N
authors.lastname: Angkathunyakul
authors.affiliation: Napat Angkathunyakul, Department of Pathology, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.
authors.forename: Suporn
authors.initials: S
authors.lastname: Treepongkaruna
authors.affiliation: Napat Angkathunyakul, Department of Pathology, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.
authors.forename: Sani
authors.initials: S
authors.lastname: Molagool
authors.affiliation: Napat Angkathunyakul, Department of Pathology, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.
authors.forename: Nichanan
authors.initials: N
authors.lastname: Ruangwattanapaisarn
mesh_terms: D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D059327:Brachydactyly / Q000209:etiology; D002908:Chronic Disease; D020117:Cisapride / Q000627:therapeutic use; D004065:Digestive System Abnormalities / Q000150:complications* / Q000175:diagnosis; D004750:Enteral Nutrition; D005385:Fingers / Q000002:abnormalities; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D007421:Intestine, Small / Q000002:abnormalities* / Q000737:chemistry; D008297:Male; D009130:Muscle, Smooth / Q000002:abnormalities* / Q000737:chemistry; D010288:Parenteral Nutrition; D012778:Short Bowel Syndrome / Q000209:etiology; D016896:Treatment Outcome; D015430:Weight Gain
chemical_list: D015415:Biomarkers; D005765:Gastrointestinal Agents; D020117:Cisapride
keywords: Abnormal layering of muscularis propria; Brachydactyly; Chronic intestinal pseudo-obstruction; Serosal muscularization; Short small bowel; Visceral myopathy