It is uncommon and accounts for 2-13% of cases of Hirschsprung disease 3,7. Compared with Hirschsprung disease which has a marked male predominance, Zuelzer-Wilson syndrome only has a slight male predominance (M:F of 1-1.5:1) 7.
Similar to Hirschsprung disease, most patients present as neonates, or less commonly as infants 7. Very rarely do patients present beyond infancy.
The presentation can have either an acute or chronic course 7:
- acute (generally neonates): features of bowel obstruction (e.g. abdominal distension, not passing meconium, etc.)
- chronic (generally after the neonatal period): chronic constipation, failure to thrive
Zuelzer-Wilson syndrome is characterized by aganglionosis (absence of ganglion cells) throughout the entire colon 7. Additionally, variable amounts of the small intestine may also be involved 5.
Treatment and prognosis
Multiple procedures have been devised to treat the condition, including ileorectal anastomosis, ileum pouch anal anastomosis without a clearly superior method 4,8. If there is small bowel involvement, intestinal transplantation may be required 5.
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- 2. Zuelzer WW, Wilson JL. Neurogenic intestinal obstruction in infancy; a clinical and pathologic study. Am J Dis Child. 1947;73 (2): 241. Pubmed citation
- 3. Devos A. S., Johan G. Blickman and J G Blickman. Radiological Imaging of the Digestive Tract in Infants and Children. Springer, 2007. ISBN: 3540407332, 9783540407331.
- 4. Marquez TT, Acton RD, Hess DJ et-al. Comprehensive review of procedures for total colonic aganglionosis. J. Pediatr. Surg. 2009;44 (1): 257-65. doi:10.1016/j.jpedsurg.2008.10.055 - Pubmed citation
- 5. Hukkinen M, Koivusalo A, Merras-Salmio L et-al. Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis. J. Pediatr. Surg. 2015; . doi:10.1016/j.jpedsurg.2015.05.017 - Pubmed citation
- 6. O'Dell K, Staren E, Bassuk A. Total colonic aganglionosis (Zuelzer-Wilson syndrome) and congenital failure of automatic control of ventilation (Ondine's curse). J. Pediatr. Surg. 1988;22 (11): 1019-20. Pubmed citation
- 7. Sweatt III JL, Spencer JR, Wierman WH. Aganglionosis of the entire colon. The American Journal of Surgery. 1970 Dec 1;120(6):764-8. https://doi.org/10.1016/S0002-9610(70)80045-9
- 8. J Wenskus, G Burmester, C Staude, T Krebs, K ReinshagenspanIleumpouch bei Patienten mit Zuelzer-Wilson-Syndrom–Bedeutung für die Patienten? class="ref_v4">