ISSN : 2146-3123
E-ISSN : 2146-3131

Hirschsprung's Diseases Complicated by Sigmoid Volvulus: A Systematic Review
Ufuk Uylas1, Orgun Gunes2, Cüneyt Kayaalp3
1Department of Gastroenterology Surgery, Gaziantep Dr. Ersin Arslan Tranining and Research Hospital, Gaziantep, Turkey
2Department of Gastroenterology Surgery, Inonu University Faculty of Medicine, Malatya, Turkey
3Department of Gastrointestinal Surgery, Inonu Universitesi Faculty of Medicine, Malatya, Turkey
DOI : 10.4274/balkanmedj.galenos.2020.2020.4.131

Background: Hirschsprung's disease and sigmoid volvulus can sometimes be seen in the same patient.
Aims: A systematic review of the literature was conducted to find out conditions suggesting underlying Hirschsprung's disease in patients with sigmoid volvulus and to discuss the diagnostic and treatment modalities.
Study Design: Systematic Review
Methods: This systematic review has been reported in line with PRISMA (preferred reporting items for systematic reviews and meta-analyses) and AMSTAR (assessing the methodological quality of systematic reviews) guidelines. PubMed and Scopus databases were scanned using the keywords ''Hirschsprung* volvulus*'' and “congenital aganglionic megacolon volvulus*”. The reference list of selected studies was reviewed for cross-checking. Two reviewers independently screened the available literature. Only Hirschsprung’s disease cases involving sigmoid volvulus were accepted, and volvulus in other sites was excluded. No restriction was used in the publication language and type of writing. The primary outcome was morbidity and mortality.
Results: A total of 31 cases were analyzed in 22 articles. Ninety-seven percent of the patients were under 40 years of age. Ninety percent of the patients were male. In patients suspected to have Hirschsprung's disease accompanying sigmoid volvulus and treated appropriately, a postoperative emergency relaparotomy was not performed, whereas this ratio was 37.5% in patients whose Hirschsprung's disease was misdiagnosed (p = 0.02). Likewise, in patients with sigmoid volvulus with missed Hirschsprung's disease, the post-treatment mortality rate was 25%, while in patients whom Hirschsprung's disease was suspected, mortality was not observed (p = 0.08).
Conclusion: If a sigmoid volvulus patient is under 40 years of age and complaints of constipation occurring since childhood, Hirschsprung's disease should be excluded with rectal biopsy.

Keywords : colon, ileus, acute abdomen, anastomosis, bowel, constipation
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