title: Enteric nervous system, interstitial cells of cajal, and smooth muscle vacuolization in segmental dilatation of jejunum.
abstract: OBJECTIVE
  The etiology of congenital segmental dilatation (CSD) of bowel remains elusive. Intestitial cell of Cajal plays a role in the pace making of the intestine. Its abnormality has been documented in a variety of conditions of abnormal intestinal motility. The current study attempts to evaluate the roles of intestitial cells of Cajal, enteric nervous system, and smooth muscle in segmental dilatation of small bowel.
  
  
  METHODS
  Resected specimen from a neonate with segmental dilatation of jejunum was stained with H&E, Alcian blue, Periodic Acid-Schiff (PAS), and immunostained with S100, Ret, MAP5, and c-kit antibodies using the standard immunohistochemical process.
  
  
  RESULTS
  The immunostaining of S100, Ret, MAP5 and c-kit of CSD specimen were positive. Localized vacuolization was, however, detected in the circular smooth muscle of the jejunum. The Alcian blue and PAS staining of the vacuolization were negative.
  
  
  CONCLUSIONS
  CSD shows no abnormality in the enteric nervous system and pace makers. Localized vacuolization suggests myopathy to be a contributing factor to the disease. J Pediatr Surg 36:930-935.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, The Hong Kong University Medical Centre, Queen Mary Hospital, Hong Kong, SAR, China.
authors.forename: W
authors.initials: W
authors.lastname: Cheng
authors.forename: V C
authors.initials: VC
authors.lastname: Lui
authors.forename: Q M
authors.initials: QM
authors.lastname: Chen
authors.forename: P K
authors.initials: PK
authors.lastname: Tam
mesh_terms: D004108:Dilatation, Pathologic; D004200:Diseases in Twins; D029721:Drosophila Proteins; D017615:Enteric Nervous System / Q000166:cytology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000196:embryology* / Q000453:epidemiology / Q000473:pathology; D007583:Jejunum / Q000002:abnormalities* / Q000196:embryology*; D008297:Male; D008869:Microtubule-Associated Proteins / Q000378:metabolism; D009130:Muscle, Smooth / Q000166:cytology*; D011518:Proto-Oncogene Proteins / Q000378:metabolism; D019009:Proto-Oncogene Proteins c-kit / Q000378:metabolism; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases / Q000378:metabolism; D009418:S100 Proteins / Q000378:metabolism; D014617:Vacuoles
chemical_list: D029721:Drosophila Proteins; D008869:Microtubule-Associated Proteins; D011518:Proto-Oncogene Proteins; D009418:S100 Proteins; C110294:S100A1 protein; D019009:Proto-Oncogene Proteins c-kit; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases; C416649:Ret protein, Drosophila