All issues > Volume 47(1); 2004

Case Report

Korean J Pediatr. 2004;47(1):95-99. Published online January 15, 2004.

A Case of Congenital Megacystis Due to Non-Obstructive Urinary Retention Associated with Intestinal Malrotation and Hypoperistalsis

Tai Young TY Ham¹, Jeong Hoon JH Kim¹, Ji Hong JH Kim¹, Jae Seung JS Lee¹, Seok Ju SJ Han², Choon Sik CS Yoon³, Soon Won SW Hong⁴

¹Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea
²Department of General Surgery, College of Medicine, Yonsei University, Seoul, Korea
³Department of Diagnostic Radiology, College of Medicine, Yonsei University, Seoul, Korea
⁴Department of Pathology, College of Medicine, Yonsei University, Seoul, Korea

Correspondence Ji Hong JH Kim ,Email: kkkjhd@yumc.yonsei.ac.kr

Abstract

Congenital megacystis with bilateral hydroureteronephrosis is most commonly associated with posterior urethral valves, prune-belly syndrome, vesicoureteral reflux, or nonrefluxing, nonobstructive megaureters. Among the other cause of congenital megacystis, megacystis-microcolon-intestinal hypoperistalsis syndrome(MMIHS), which is characterized by megacystis, microcolon and hypoperistalsis of the intestines with resultant abdominal distension, is a rare cause of functional obstruction of urinary tracts in childhood. It was first reported by Berdon, et al. in 1976, and only 89 cases have been reported until the present. There has been no report in Korea. We report a 2 month old female patient who exhibited intestinal malrotation, megacystitis, abdominal distension and hypoperistalsis. She did not show any evidence of microcolon, but her biopsy result exhibited degenerative changes of intestinal and cystic smooth muscle, pointing to a syndrome very similar to MMIHS.

Keywords :Megacystis, MMIH syndrome, Non obstructive urinary retention