Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan

Soh, Hideki; Fukuzawa, Masahiro; Kubota, Akio et al.

Journal of Pediatric Surgery, 2015, 50(12), 2048-2050

Number of Access:628（2025-07-22 22:13 Counts）

Identifier to cite or link to this item: https://hdl.handle.net/11094/99830

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Title
Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan
Creator
NRID
1000000457011 1000000457011
CreatorName
Soh, Hideki
NRID
1000060165272 1000060165272
researchmap permalink
read0106298 read0106298
CreatorName
Fukuzawa, Masahiro
NRID
1000010161671 1000010161671
CreatorName
Kubota, Akio
NRID
1000020224826 1000020224826
CreatorName
Kawahara, Hisayoshi
NRID
1000010456957 1000010456957
ORCID
0000-0002-4639-8814 0000-0002-4639-8814
researchmap permalink
ueno-pedsurg ueno-pedsurg
Scopus Author ID
21743992300 21743992300
CreatorName
Ueno, Takehisa
NRID
1000020197247 1000020197247
CreatorName
Taguchi, Tomoaki
Subject
Distended bladder
Functional bowel obstruction
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS)
Microcolon
Description
Soh H., Fukuzawa M., Kubota A., et al. Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan. Journal of Pediatric Surgery 50, 2048 (2015); https://doi.org/10.1016/j.jpedsurg.2015.08.026.
Abstract
Background: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan. Methods: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. Results: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n = 11) and colon (n = 5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively. Conclusions: MMIHS patients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHS patients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.
Publisher
Elsevier Inc.
Source Title
Journal of Pediatric Surgery
Volume (Issue)
50(12)
Page
2048-2050
Date of Issued
2015-12-01
Language
English
Handle URL
https://hdl.handle.net/11094/99830
PISSN
00223468
eISSN
15315037
Relation.isVersionOf
DOI (Publisher Version)
https://doi.org/10.1016/j.jpedsurg.2015.08.026
Access Rights
open access
CopyRight
© 2015. This manuscript version is made available under the CC-BY-NC-ND 4.0 license https://creativecommons.org/licenses/by-nc-nd/4.0/
oaire:version
Accepted Manuscript
Category
会議発表論文 Conference Paper