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Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management.
J Pediatr Surg. 2016 Sep; 51(9):1565-73.JP

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.

Authors+Show Affiliations

Pritzker School of Medicine, University of Chicago.Section of Urology, University of Chicago Medicine. Electronic address: Blake.Anderson@uchospitals.edu.Section of Urology, University of Chicago Medicine.Section of Urology, University of Chicago Medicine.

Pub Type(s)

Case Reports
Journal Article
Review
Systematic Review

Language

eng

PubMed ID

27421821

Citation

Wymer, Kevin M., et al. "Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: Case Series and Updated Review of the Literature With an Emphasis On Urologic Management." Journal of Pediatric Surgery, vol. 51, no. 9, 2016, pp. 1565-73.
Wymer KM, Anderson BB, Wilkens AA, et al. Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management. J Pediatr Surg. 2016;51(9):1565-73.
Wymer, K. M., Anderson, B. B., Wilkens, A. A., & Gundeti, M. S. (2016). Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management. Journal of Pediatric Surgery, 51(9), 1565-73. https://doi.org/10.1016/j.jpedsurg.2016.06.011
Wymer KM, et al. Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: Case Series and Updated Review of the Literature With an Emphasis On Urologic Management. J Pediatr Surg. 2016;51(9):1565-73. PubMed PMID: 27421821.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management. AU - Wymer,Kevin M, AU - Anderson,Blake B, AU - Wilkens,Ashley A, AU - Gundeti,Mohan S, Y1 - 2016/06/26/ PY - 2016/03/21/received PY - 2016/06/14/revised PY - 2016/06/19/accepted PY - 2016/7/17/entrez PY - 2016/7/17/pubmed PY - 2017/2/10/medline KW - Berdon KW - Hypoperistalsis KW - MMIHS KW - Megacystis KW - Microcolon SP - 1565 EP - 73 JF - Journal of pediatric surgery JO - J Pediatr Surg VL - 51 IS - 9 N2 - 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. SN - 1531-5037 UR - https://www.unboundmedicine.com/medline/citation/27421821/Megacystis_microcolon_intestinal_hypoperistalsis_syndrome:_Case_series_and_updated_review_of_the_literature_with_an_emphasis_on_urologic_management_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0022-3468(16)30146-4 DB - PRIME DP - Unbound Medicine ER -