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Imaging of total colonic Hirschsprung disease

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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.

Materials and 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.

Conclusion

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.

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Authors and Affiliations

  1. Section of Pediatric Radiology, University of Michigan Health System, 1500 E. Medical Center Drive, Ann Arbor, MI, 48109-0252, USA

    Enno Stranzinger, Michael A. DiPietro & Peter J. Strouse

  2. Section of Pediatric Surgery, University of Michigan Health System, Ann Arbor, MI, USA

    Daniel H. Teitelbaum

Authors
  1. Enno Stranzinger
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  2. Michael A. DiPietro
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  3. Daniel H. Teitelbaum
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  4. Peter J. Strouse
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Correspondence to Michael A. DiPietro.

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Stranzinger, E., DiPietro, M.A., Teitelbaum, D.H. et al. Imaging of total colonic Hirschsprung disease. Pediatr Radiol 38, 1162–1170 (2008). https://doi.org/10.1007/s00247-008-0952-4

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  • DOI: https://doi.org/10.1007/s00247-008-0952-4

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