Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management
- PMID: 27421821
- DOI: 10.1016/j.jpedsurg.2016.06.011
Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management
Abstract
Introduction: Megacystis microcolon intestinal hypoperistalsis (MMIHS) is a rare disorder characterized by distended nonobstructed bladder, microcolon, and decreased intestinal peristalsis. MMIHS has a particularly poor prognosis; however, when appropriately managed, survival can be prolonged.
Study design: A systematic review (1996-2016) was performed with the key words "megacystis microcolon intestinal hypoperistalsis syndrome." In addition, a case series of four patients is presented as well as algorithms for the diagnosis and treatment of MMIHS.
Results: 135 patients with MMIHS were identified in the literature. 73% (88/121) of the patients were female, 65% underwent diagnostic biopsy (64/99), and 63% (66/106) were identified with prenatal imaging. The majority of patients were treated with TPN as well as gastrostomy or ileostomy and CIC, however 15% (18/116) received multivisceral or intestinal transplant, and 30% (22/73) had a vesicostomy. The survival rate was 57% (68/121).
Conclusion: Appropriate management of MMIHS patients is crucial. An enlarged, acontractile bladder in a child with bowel motility problems should be considered diagnostic. Bladder distension can be managed with CIC or vesicostomy in addition to prophylactic antibiotics if frequent urinary tract infections are present. These patients often require gastrostomy or ileostomy as well as total parenteral nutrition. This management has led to significant improvement in survival rates.
Keywords: Berdon; Hypoperistalsis; MMIHS; Megacystis; Microcolon.
Copyright © 2016 Elsevier Inc. All rights reserved.
Similar articles
-
Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan.J Pediatr Surg. 2015 Dec;50(12):2048-50. doi: 10.1016/j.jpedsurg.2015.08.026. Epub 2015 Aug 28. J Pediatr Surg. 2015. PMID: 26413901
-
Megacystis microcolon intestinal hypoperistalsis syndrome: systematic review of outcome.Pediatr Surg Int. 2011 Oct;27(10):1041-6. doi: 10.1007/s00383-011-2954-9. Pediatr Surg Int. 2011. PMID: 21792650 Review.
-
Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS): challenges in diagnosis and management.BMJ Case Rep. 2024 Apr 16;17(4):e259983. doi: 10.1136/bcr-2024-259983. BMJ Case Rep. 2024. PMID: 38627049
-
Esophageal dysmotility: An intrinsic feature of megacystis, microcolon, hypoperistalsis syndrome (MMIHS).J Pediatr Surg. 2019 Jul;54(7):1303-1307. doi: 10.1016/j.jpedsurg.2018.08.051. Epub 2018 Sep 7. J Pediatr Surg. 2019. PMID: 30257810
-
Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS): report of a case with prolonged survival and literature review.J Pediatr Urol. 2013 Feb;9(1):e12-8. doi: 10.1016/j.jpurol.2012.05.017. Epub 2012 Jun 30. J Pediatr Urol. 2013. PMID: 22749573 Review.
Cited by
-
Imaging findings of a twin male neonate with megacystis microcolon intestinal hypoperistalsis syndrome.Radiol Case Rep. 2021 Jan 5;16(3):628-630. doi: 10.1016/j.radcr.2020.12.055. eCollection 2021 Mar. Radiol Case Rep. 2021. PMID: 33437344 Free PMC article.
-
Use of whole genome sequencing to determine the genetic basis of visceral myopathies including Prune Belly syndrome.J Rare Dis (Berlin). 2023;2(1):9. doi: 10.1007/s44162-023-00012-z. Epub 2023 Jun 5. J Rare Dis (Berlin). 2023. PMID: 37288276 Free PMC article.
-
Loss of LMOD1 impairs smooth muscle cytocontractility and causes megacystis microcolon intestinal hypoperistalsis syndrome in humans and mice.Proc Natl Acad Sci U S A. 2017 Mar 28;114(13):E2739-E2747. doi: 10.1073/pnas.1620507114. Epub 2017 Mar 14. Proc Natl Acad Sci U S A. 2017. PMID: 28292896 Free PMC article.
-
Smooth muscle motility disorder phenotypes: A systematic review of cases associated with seven pathogenic genes (ACTG2, MYH11, FLNA, MYLK, RAD21, MYL9 and LMOD1).Intractable Rare Dis Res. 2022 Aug;11(3):113-119. doi: 10.5582/irdr.2022.01060. Intractable Rare Dis Res. 2022. PMID: 36200034 Free PMC article. Review.
-
Fetal megacystis: a lot more than LUTO.Ultrasound Obstet Gynecol. 2019 Jun;53(6):779-787. doi: 10.1002/uog.19182. Ultrasound Obstet Gynecol. 2019. PMID: 30043466 Free PMC article.
Publication types
MeSH terms
Supplementary concepts
LinkOut - more resources
Full Text Sources
Other Literature Sources
Research Materials
Miscellaneous
