Urologic Phenotype and Patterns of Care in Patients With Megacystis Microcolon Intestinal Hypoperistalsis Syndrome Presenting to a Major Pediatric Transplantation Center

Urology
Lee A HugarHeidi A Stephany

Abstract

To describe the phenotype, referral pattern, and trends of urologic management for children with megacystis microcolon intestinal hypoperistalsis syndrome. We performed a retrospective review of all patients with megacystis microcolon intestinal hypoperistalsis syndrome treated at a large tertiary children's medical center over a 14-year period. Our primary outcomes included the rate of urology referral, urodynamic characteristics of bladder function, and the proportion of patients managed with clean intermittent catheterization. Twenty-six patients were evaluated at our institution with a median age at presentation of 1.3 years (73% female, 19 of 26). Most patients presented for intestinal transplantation evaluation and were dependent on parenteral nutrition. Megacystis was diagnosed prenatally in 15 patients and postnatally in 11 patients. Twenty patients (77%, 20 of 26) were evaluated by a pediatric urologist. Six patients (23%, 6 of 26) had varying degrees of vesicoureteral reflux. Early bladder management consisted of clean intermittent catheterization in 13 patients and vesicostomy in 8 patients. Megacystis microcolon intestinal hypoperistalsis syndrome is a severe form of intestinal failure and bladder atony with substan...Continue Reading

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