Zuelzer-Wilson syndrome

The Zuelzer-Wilson syndrome (total colonic aganglionosis) is a subset of Hirschsprung disease, in which the whole colon is aganglionic. It is uncommon and accounts for 2-13% of cases of Hirschsprung disease 3. Multiple procedures have been devised to treat the condition, including proctocolectomy, without a clearly superior method 4

Variable amounts of the small intestine may also be involved and may require intestinal transplantation 5.

The condition has been associated with other congenital syndromes, including congenital central hypoventilation syndrome (Ondine's curse) 6.

 

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Article Information

rID: 8459
Section: Syndromes
Synonyms or Alternate Spellings:
  • Total colonic aganglionosis

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