Work

An atypical case of megacystis microcolon intestinal hypoperistalsis syndrome with extended survival and consistent bowel function

Year: 2017

Type: article

Abstract: • MMIHS is a very rare condition that is associated with high neonatal mortality. Management is mainly supportive. • MMIHS is diagnosed on prenatal ultrasound that shows megacystis and is associated with mutations in MYH11, LMOD1, and ACTG2. • MMIHS patients who survive long-term usually require total parenteral nutrition and multiple GI and GU surgeries. • This MMIHS patient with mutated ACTG2 is unique--she survived past 1 year without TPN and with consistent bowel function.

Source: Journal of Pediatric Surgery Case Reports

DOI: 10.1016/j.epsc.2017.11.010