Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: Case Series and Updated Review of the Literature with an Emphasis on Urologic Management

• Published in: Journal of pediatric surgery 2016-09, Vol.51 (9), p.1565-1573
• Main Authors: Wymer, Kevin M, Anderson, Blake B, Wilkens, Ashley A, Gundeti, Mohan S
• Format: Article
• Language: English
• Subjects: Abnormalities, Multiple - diagnosis; Abnormalities, Multiple - mortality; Abnormalities, Multiple - therapy; Berdon; Colon - abnormalities; Combined Modality Therapy; Cystostomy; Female; Gastrostomy; Humans; Hypoperistalsis; Ileostomy; Infant; Infant, Newborn; Intermittent Urethral Catheterization; Intestinal Pseudo-Obstruction - diagnosis; Intestinal Pseudo-Obstruction - mortality; Intestinal Pseudo-Obstruction - therapy; Male; Megacystis; Microcolon; MMIHS; Parenteral Nutrition, Total; Pediatrics; Surgery; Treatment Outcome; Urinary Bladder - abnormalities
• ISSN: 0022-3468; 1531-5037
• DOI: 10.1016/j.jpedsurg.2016.06.011

Abstract Introduction Megacystis microcolon intestinal hypoperistalsis (MMIHS) is a rare disorder characterized by distended non-obstructed 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.