DOI: 10.36347/sjams.2021.v09i09.022

Pages: 1427-1430

Hirschsprung's disease (HD) is a rare congenital disease characterized by the absence of ganglion cells in the distal rectum and which is usually diagnosed in early childhood. We speak of Hirschsprung’s disease of adults in patients where the diagnosis was made after the age of 10 years, it is a rare but not exceptional condition. This should be borne in mind in young adults with a history of chronic constipation. Due to the rarity of Hirschsprung’s disease in adults, patients are rarely referred for evaluation, including rectal biopsy, further delaying diagnosis. The elements of the diagnosis are primarily clinical. The basic treatment for Hirschsprung's disease recognized in adulthood is surgical resection of the lymph node segment of the intestine with the intention of creating an anastomosis with healthy and properly innervated tissue. In this regard, we present the case of a 22-year-old young man, followed in the gastro-entero-hepatology department at the Arrazi hospital of the Mohammed VI University Hospital in Marrakech for aetiological assessment of a chronic constipation of terminal appearance, complicated by 2 episodes of sigmoid volvulus resolved by rectal probe detorsion, the rectal biopsy of which revealed aganglion in favor of Hirschsprung’s disease.