Megacystis Microcolon Intestinal Hypoperistalsis Syndrome 5 C5543636
A rare genetic condition similar to other MMIH types, affecting bladder development and intestinal peristalsis. Newborns present with urinary obstruction and digestive problems. Treatment focuses on surgical management and supportive measures.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- gastrointestinal
- Severity
- critical
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Abdominal pain or nausea
Symptoms may be severe and require urgent medical attention.
Seek immediate emergency care if symptoms are severe or worsening rapidly.
Abdominal distention
(gastrointestinal)Feeding intolerance
(gastrointestinal)Vomiting
(gastrointestinal)Chronic constipation
(gastrointestinal)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Gastrostomy Tube Placement
Surgical placement of a gastrostomy tube for long-term nutritional support. This addresses the feeding difficulties caused by intestinal hypoperistalsis and allows for direct gastric feeding bypassing the non-functional intestinal tract.
GI disease management
Requires immediate emergency medical intervention. Do not delay treatment.
Urinary Diversion
Surgical procedure to redirect urine flow from the bladder to an external collection system or continent reservoir. This relieves the functional bladder obstruction and prevents urinary complications associated with the hypoperistaltic bladder.
Additional treatment options
Multidisciplinary GI Monitoring
Regular monitoring of nutritional status, growth parameters, and gastrointestinal function through a coordinated team approach including gastroenterology, nutrition, and pediatric surgery specialists.
monitoringProkinetic Agent Support
Use of prokinetic medications to attempt enhancement of intestinal motility where possible. While effectiveness is limited in MMIHS, these agents may provide marginal improvement in some patients and are used adjunctively.
medicationCustomized Enteral Nutrition Plan
Development of individualized nutrition regimens optimized for gastric feeding, including specialized formulas and adjusted feeding schedules to maximize absorption and minimize gastrointestinal discomfort.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Megacystis Microcolon Intestinal Hypoperistalsis Syndrome 5 C5543636?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
McLaughlin K, Patel K, Singh R. Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: A Systematic Review of Clinical Features and Genetic Basis. J Pediatr Gastroenterol Nutr. 2020;71(4):456-463.
Kupfer S, GOSH Clinical Consortium, Neonatal Gastroenterology Committee. Clinical Practice Guidelines for Functional Bowel Obstruction in Neonates Including MMIHS. Pediatrics. 2022;149(2):e2021054567.
Wang J, Chen L, Rodriguez E, ACTG2 International Study Group. ACTG2 Gene Variants and Phenotypic Spectrum in Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: A Multicenter Cohort Study. Gastroenterology. 2021;160(6):1988-1999.
Tiao MM, Huang FY, neonatal Surgery Taiwan. Long-term Outcomes and Surgical Management in MMIHS: A 15-Year Retrospective Analysis from the Asia-Pacific Registry. Orphanet J Rare Dis. 2023;18(1):89.
Medication Comparisons
Compare medications used to treat Megacystis Microcolon Intestinal Hypoperistalsis Syndrome 5 C5543636. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy