Microgastria-limb reduction anomaly with total amelia

The Microgastria-limb reduction anomaly is a rare disorder of unknown aetiology in which microgastria is associated with limb defects, along with splenic and other variable congenital abnormalities. In 15 previously reported cases, none have had the most extreme form of amelia. We report the clinical progress of a 3-year-old girl with severe microgastria and total amelia who has been managed conservatively with minimal surgical intervention.