| A Case of Congenital Megacystis Due to Non-Obstructive Urinary Retention Associated with Intestinal Malrotation and Hypoperistalsis |
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Tai Young Ham1, Jeong Hoon Kim1, Ji Hong Kim1, Jae Seung Lee1, Seok Ju Han2, Choon Sik Yoon3, Soon Won 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 |
| 장관 회전이상과 장관 연동운동 저하를 동반한 비폐쇄성 소변 저류에 의한 선천성 거대 방광증 1례 |
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함태영1, 김정훈1, 김지홍1, 이재승1, 한석주2, 윤춘식3, 홍순원4 |
1연세대학교 의과대학 소아과학교실
2연세대학교 의과대학 외과학교실
3연세대학교 의과대학 방사선학교실
4연세대학교 의과대학 진단병리학교실 |
Correspondence:
Ji Hong Kim, Email: kkkjhd@yumc.yonsei.ac.kr
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| Abstract |
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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. |
| Key Words:
Megacystis, MMIH syndrome, Non obstructive urinary retention |
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