Megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndrome
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital visceral myopathy. We experienced a case of an infant with MMIHS with prune belly syndrome (PBS). A pregnant woman was transferred at 16 gestational weeks for a cyst in the fetal bladder. Fetal bladder punctures a...
Main Authors: | , , , , , , , , , , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Elsevier
2018-07-01
|
Series: | Journal of Pediatric Surgery Case Reports |
Subjects: | |
Online Access: | http://www.sciencedirect.com/science/article/pii/S2213576618301076 |
id |
doaj-498ef4eefaef4476a34c05809010d530 |
---|---|
record_format |
Article |
spelling |
doaj-498ef4eefaef4476a34c05809010d5302020-11-25T00:31:17ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662018-07-0134C545710.1016/j.epsc.2018.05.008Megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndromeHidehiko Maruyama0Yuichi Hasegawa1Rika Sugibayashi2Yuka Iwasaki3Shuhei Fujino4Shoichiro Amari5Junko Nagasawa6Yuka Wada7Hideshi Fujinaga8Keiko Tsukamoto9Kazunori Tahara10Takako Yoshioka11Yushi Ito12Haruhiko Sago13Division of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Pediatric Urology, Department of Surgical Specialties, JapanDivision of Fetal Medicine, Center of Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Surgery, Department of Surgical Specialties, JapanDepartment of Pathology, National Center for Child Health and Development, JapanDivision of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, JapanDivision of Fetal Medicine, Center of Maternal-Fetal, Neonatal and Reproductive Medicine, JapanMegacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital visceral myopathy. We experienced a case of an infant with MMIHS with prune belly syndrome (PBS). A pregnant woman was transferred at 16 gestational weeks for a cyst in the fetal bladder. Fetal bladder punctures and a vesicoamniotic shunting were performed. At 31 gestational weeks, a male preterm infant weighing 2432 g was born. After birth, a urinary catheter was inserted through the hole left by the fetal shunt. Bilateral undescended testicles, a dilated urinary tract, and deficient abdominal wall musculature compatible with PBS were also found. On day 5, microcolon and non-obstructive urethra were diagnosed by radiography. An upper gastrointestinal examination showed no movement of the stomach or intestine. Based on these findings, MMIHS was diagnosed. On day 76, a gastrostomy and ileostomy were performed. The histological findings showed no abnormality of the nerve plexus. The infant died at age 4 months. Fatty degeneration of the liver was found at autopsy. MMIHS has a bad prognosis due to liver dysfunction. Our patient had severe progressive liver dysfunction and symptoms of PBS. We speculate that MMIHS and PBS might fall along the same disease spectrum.http://www.sciencedirect.com/science/article/pii/S2213576618301076Fetal vesicoamniotic shuntMegacystis microcolon intestinal hypoperistalsis syndromePrune belly syndrome |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Hidehiko Maruyama Yuichi Hasegawa Rika Sugibayashi Yuka Iwasaki Shuhei Fujino Shoichiro Amari Junko Nagasawa Yuka Wada Hideshi Fujinaga Keiko Tsukamoto Kazunori Tahara Takako Yoshioka Yushi Ito Haruhiko Sago |
spellingShingle |
Hidehiko Maruyama Yuichi Hasegawa Rika Sugibayashi Yuka Iwasaki Shuhei Fujino Shoichiro Amari Junko Nagasawa Yuka Wada Hideshi Fujinaga Keiko Tsukamoto Kazunori Tahara Takako Yoshioka Yushi Ito Haruhiko Sago Megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndrome Journal of Pediatric Surgery Case Reports Fetal vesicoamniotic shunt Megacystis microcolon intestinal hypoperistalsis syndrome Prune belly syndrome |
author_facet |
Hidehiko Maruyama Yuichi Hasegawa Rika Sugibayashi Yuka Iwasaki Shuhei Fujino Shoichiro Amari Junko Nagasawa Yuka Wada Hideshi Fujinaga Keiko Tsukamoto Kazunori Tahara Takako Yoshioka Yushi Ito Haruhiko Sago |
author_sort |
Hidehiko Maruyama |
title |
Megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndrome |
title_short |
Megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndrome |
title_full |
Megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndrome |
title_fullStr |
Megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndrome |
title_full_unstemmed |
Megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndrome |
title_sort |
megacystis microcolon intestinal hypoperistalsis syndrome overlapping prune belly syndrome |
publisher |
Elsevier |
series |
Journal of Pediatric Surgery Case Reports |
issn |
2213-5766 |
publishDate |
2018-07-01 |
description |
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital visceral myopathy. We experienced a case of an infant with MMIHS with prune belly syndrome (PBS).
A pregnant woman was transferred at 16 gestational weeks for a cyst in the fetal bladder. Fetal bladder punctures and a vesicoamniotic shunting were performed. At 31 gestational weeks, a male preterm infant weighing 2432 g was born. After birth, a urinary catheter was inserted through the hole left by the fetal shunt. Bilateral undescended testicles, a dilated urinary tract, and deficient abdominal wall musculature compatible with PBS were also found. On day 5, microcolon and non-obstructive urethra were diagnosed by radiography. An upper gastrointestinal examination showed no movement of the stomach or intestine. Based on these findings, MMIHS was diagnosed. On day 76, a gastrostomy and ileostomy were performed. The histological findings showed no abnormality of the nerve plexus. The infant died at age 4 months. Fatty degeneration of the liver was found at autopsy.
MMIHS has a bad prognosis due to liver dysfunction. Our patient had severe progressive liver dysfunction and symptoms of PBS. We speculate that MMIHS and PBS might fall along the same disease spectrum. |
topic |
Fetal vesicoamniotic shunt Megacystis microcolon intestinal hypoperistalsis syndrome Prune belly syndrome |
url |
http://www.sciencedirect.com/science/article/pii/S2213576618301076 |
work_keys_str_mv |
AT hidehikomaruyama megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT yuichihasegawa megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT rikasugibayashi megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT yukaiwasaki megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT shuheifujino megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT shoichiroamari megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT junkonagasawa megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT yukawada megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT hideshifujinaga megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT keikotsukamoto megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT kazunoritahara megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT takakoyoshioka megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT yushiito megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome AT haruhikosago megacystismicrocolonintestinalhypoperistalsissyndromeoverlappingprunebellysyndrome |
_version_ |
1725322722039300096 |