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Diseases

Genetic and Rare Diseases Information Center (GARD)

Hypoganglionosis


Other Names for this Disease
  • Intestinal hypoganglionosis
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Overview

Hypoganglionosis, also known as intestinal hypoganglionosis, is a disorder causing a reduced number of nerves in the intestinal wall. Intestinal hypoganglionosis can mimic Hirschsprung disease; patients with both conditions may present with chronic constipation, intestinal obstruction, and enterocolitis (inflammation of the intestines).[1][2] Patients with hypoganglionosis may also suffer from severe complications including fecaloma (hardening of the feces inside the colon), bleeding or perforation of the intestine, and breathing problems resulting from a distended colon.[1] An accurate diagnosis in adults is often difficult, requiring one or more biopsies of the intestinal wall.[1][3] The exact cause of hypoganglionoisis is not known. In some cases, it is due to factors present at birth (congenital), while other times it is believed to be an acquired condition. The management of isolated hypoganglionosis generally involves surgery to remove the affected bowel segment.[2]
Last updated: 8/7/2015

References

  1. Kim, Hye Jin, et al.. Hirschsprung Disease and Hypoganglionosis in Adults: Radiologic Findings and Differentiation. RSNA Radiology. May, 2008; 247(2):http://pubs.rsna.org/doi/full/10.1148/radiol.2472070182. Accessed 8/7/2015.
  2. Friedmacher, F and Puri, P.. Classification and diagnostic criteria of variants of Hirschsprung's disease. Pediatr Surg Int.. Sept, 2013; 29(9):855-72. http://www.ncbi.nlm.nih.gov/pubmed/?term=23943250. Accessed 8/7/2015.
  3. Dingemann, Jens and Puri, Prem. Isolated hypoganglionosis: systematic review of a rare intestinal innervation defect. Pediatr Surg Int. 2010; 26:1111-1115. http://www.ncbi.nlm.nih.gov/pubmed/?term=20721562. Accessed 8/7/2015.
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In Depth Information

  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hypoganglionosis. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Intestinal hypoganglionosis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.