A Case of Congenital Megacystis Due to Non-Obstructive Urinary Retention Associated with Intestinal Malrotation and Hypoperistalsis.
- Author:
Tai Young HAM
1
;
Jeong Hoon KIM
;
Ji Hong KIM
;
Jae Seung LEE
;
Seok Ju HAN
;
Choon Sik YOON
;
Soon Won HONG
Author Information
1. Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea. kkkjhd@yumc.yonsei.ac.kr
- Publication Type:Case Report
- Keywords:
Megacystis;
MMIH syndrome;
Non obstructive urinary retention
- MeSH:
Biopsy;
Female;
Humans;
Infant;
Intestines;
Korea;
Muscle, Smooth;
Prune Belly Syndrome;
Urinary Retention*;
Urinary Tract;
Vesico-Ureteral Reflux
- From:Korean Journal of Pediatrics
2004;47(1):95-99
- CountryRepublic of Korea
- Language:Korean
-
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.
