Hirschsprung Disease at Children: Report of One Case

Hirschsprung disease is a rare motor disorder of the gut that is characterized by absence of ganglia in distal colon resulting in a functional obstruction. Most cases of Hirschsprung disease are the diagnosted in the newborn period and is characterized by the total absence of intramural ganglion cells of the submucosal (Meissner) and myenteric (Aurbach) neural plexus in the affected segment of the bowel. Hirschsprung disease should be considered in any newborn that fails to pass meconium within 24-48 hours of birth 94 % of cases are diagnosted before the patient reaches 5 years of age, mild cases of Hirschsprung disease may go underdiagnosted until he or she reached adulthood. In this study, we describe one case of infant with Hirschsprung disease, 5months old, presenting to the University Hospital Center of Tirana. He had a history of longlasting recurrent constipation, realived by laxatives, progressive abdominal distendion, colicky pain, emesis. Barium Enema reveal a grossly distended descendent colon, sigma and rectum. HD should be considered in the differential diagnosis of cases where patients present with constipation, acute megacolon, hypothyroidism, intestinal motility disorder, irritable bowel sindromy, toxic megacolon. The treatment of HD is surgery.