An Adult Man with Refractory Constipation that is Diagnosed as ‘Adult-type Hypoganglionosis’

The Hypoganglinosis is a rare and resembles Hirschsprung’s disease in the male-to-female ratio, etiology and clinical presentation. Although chronic constipation is a common complaint in general population, the patients with constipation can accompany with fatal complication if the patient with megacolon which is related with hypoganglinosis. Up to 90.5% of patients with Hirschsprung’s disease are diagnosed in the newborn period and the median age at diagnosis of the patient with hypoganglinosis was 4.85 years old. We report a healthy 43 year-old male with severe constipation who is diagnosed as adult type hypoganglinosis. A 43-year-old man presented with chronic constipation, abdominal discomfort, and flatulence, and had been passing intestinal gas with unusual odors for 18 months. Computed tomography showed a severely dilated feces-filled proximal segment of the descending colon and transverse colon. The patient underwent subtotal colectomy with ileocolic anastomosis for a clinical impression of intractable megacolon. Histopathology confirmed a diagnosis of hypoganglionosis of the colon. The hypoganglinosis is rare disease in population, especially in adult group. However, the early and accurate diagnosis of adult hypoganglionosis is important for the physician to avoid fatal complication. We hope that adult intestinal innervation disorders will be considered readily in the differential diagnoses of chronic constipation. (J Intern Med Taiwan 2016; 27: 44-48)