DOI: 10.36347/sjmcr.2020.v08i06.006

Pages: 617-619

Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a quite rare congenital and fatal disease which was firstly defined by Berdon and colleagues in 1976 [1] and is often underdiagnosed and confused with other disorders. The clinicians priority is to be make a correct diagnosis. It predominantly affects females (4:1 ratio) and is characterized by the presence of distended bladder (without distal urinary tract obstruction), microcolon, and decreased or absent intestinal peristalsis. We present a case of female newborn with antenatal ultrasound revealing a large cystic mass in pelvis of urinary tract origin, abdominal distension, aperistalsis of the intestine and micro colon. Gastrostomy and vesicostomy was done. Pathological findings of small bowel biopsy revealed normal ganglion cells. Child died of septicaemia at age of 20 days.