title: Imaging of total colonic Hirschsprung disease.
abstract: BACKGROUND
  Hirschsprung disease (HD) is a functional obstruction of the bowel caused by the absence of intrinsic enteric ganglion cells. The diagnosis of total colonic HD (TCHD) based on contrast enemas is difficult in newborns because radiological findings vary.
  
  
  OBJECTIVE
  To evaluate the radiographic and contrast enema findings in patients with pathologically proven TCHD.
  
  
  METHODS
  From 1966 to 2007, 17 records from a total of 31 patients with TCHD were retrospectively evaluated for diameter and shape of the colon, diameter of the small bowel, bowel wall contour, ileal reflux, abdominal calcifications, pneumoperitoneum, filling defects, transitional zones and rectosigmoid index.
  
  
  RESULTS
  Three colonic patterns of TCHD were found: microcolon, question-mark-shape colon and normal caliber colon. Additional findings included spasmodic colon, ileal reflux, delayed evacuation and abdominal calcifications. Colonic transitional zones were found in eight patients with TCHD.
  
  
  CONCLUSIONS
  The diagnosis of TCHD is difficult to establish by contrast enema studies. The length of the aganglionic small bowel and the age of the patient can influence the radiological findings in TCHD. The transitional zone and the rectosigmoid index can be false-positive in TCHD. The colon can appear normal. Consider TCHD if the contrast enema study is normal but the patient remains symptomatic and other causes of distal bowel obstruction have been excluded.
authors.affiliation: Section of Pediatric Radiology, University of Michigan Health System, Ann Arbor, MI 48109-0252, USA.
authors.forename: Enno
authors.initials: E
authors.lastname: Stranzinger
authors.forename: Michael A
authors.initials: MA
authors.lastname: DiPietro
authors.forename: Daniel H
authors.initials: DH
authors.lastname: Teitelbaum
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Strouse
mesh_terms: D001466:Barium Sulfate; D003108:Colonic Diseases / Q000000981:diagnostic imaging*; D003287:Contrast Media; D004733:Enema; D005260:Female; D006627:Hirschsprung Disease / Q000000981:diagnostic imaging*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011859:Radiography; D012189:Retrospective Studies
chemical_list: D003287:Contrast Media; D001466:Barium Sulfate