Systematic Review of Isolated Hypoganglionosis and Presentation of a New Case Complicated With Sigmoid Volvulus

Isolated hypoganglionosis is rare and constitutes 3-5% of all congenital neurological bowel diseases. Since hypoganglionosis is associated with symptoms such as chronic constipation or pseudo-obstruction, it can be confused with Hirschprung's disease and it is important to make a differential diagnosis before treatment. Our aim in this study; In addition to sharing the rarely isolated hypoganglionosis and sigmoid volvulus in a 45-year-old male patient with mental retardation, the investigators present a systematic comprehensive review of hypoganglionosis. Pubmed and Scopus databases were scanned. No restrictions were used in the written language. Only isolated cases of hypoganglionosis were added to the study. In the cases; author, year of publication, country of publication, age, gender, surgical or non-surgical treatments, treatment results, and mortalities were examined.

When sigmoid volvulus is seen in children and adolescents, congenital neurological bowel diseases should be considered in the differential diagnosis. Hirschprung's disease must be ruled out by rectal biopsy. The histopathological investigation should be carried out for the diagnosis and determination of surgical margins in isolated hypoganglionosis.