A Case of Acquired Zonal Aganglionosis in Adult

ABSTRACT

A chronic intestinal pseudo-obstruction is a rare disorder and a severe digestive syndrome. It is characterized by deranged gut propulsive motility that resembles a mechanical obstruction, but no obstructive process is present. An intestinal pseudo-obstruction may be classified as acute or chronic; the chronic form may also be classified as idiopathic or secondary to a variety of diseases. Treatment of intestinal pseudo-obstruction involves nutritional, pharmacological, and surgical therapies. Surgery should be limited to patients who are refractory to medical therapy and show a deteriorating course. Despite available medical and surgical interventions, the outcome remains poor. Here, we describe a case of a 54-year-old female with chronic constipation and abdominal distension, who was subsequently found to have segmental aganglionosis. The patient was treated with a subtotal colectomy and ileosigmoidostomy without sequelae.