A Case of Adult Type Colonic Hypoganglionosis / 대한소화기내시경학회지

ABSTRACT

Hypoganglionosis is a rare form of intestinal neuronal malformation, which is characterized by scarce ganglia and a reduced number of parasympathetic nerves in the intestinal wall. The pathogenesis of intestinal neuronal malformation is mainly attributed to developmental disorders of the enteric nervous system (ENS). Furthermore, the ENS can be damaged during the postnatal period by ischemic, inflammatory, autoimmunological process or neurotoxic agents. Subsequently abnormalties of the ENS may cause chronic constipation or ileus. Intestinal neuronal malformation should be suspected in patients with a history of chronic stool retention and appropriate radiographic findings. Hypoganglionosis is extremely difficult to establish the diagnosis histochemically unless full-thickness biopsies are performed. Recently we experienced a case of adult type hypoganglionosis, which is assumed to be an acquired type. It was treated with right hemicolectomy.