Megacystis Microcolon Intestinal Hypoperistalsis Syndrome 4 C5543519
A rare congenital disorder affecting bladder and intestinal function, causing an enlarged bladder and impaired digestive movement. It typically presents in newborns with urinary and feeding difficulties. Management involves surgical interventions and long-term supportive care.
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 distension
(gastrointestinal)Urinary retention
(gastrointestinal)Feeding intolerance
(gastrointestinal)Bacterial overgrowth
(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
GI disease management
Requires immediate emergency medical intervention. Do not delay treatment.
Parenteral Nutrition
Complete intravenous nutrition providing calories, amino acids, lipids, vitamins and minerals when enteral feeding is insufficient or contraindicated due to intestinal dysmotility.
Intestinal Stoma Creation
Surgical creation of ileostomy or colostomy to bypass non-functional intestinal segments and decompress the GI tract in cases of severe hypoperistalsis.
Additional treatment options
Bladder Catheterization and Drainage
Intermittent or continuous catheterization to manage urinary retention from megacystis. May include suprapubic catheter placement for long-term drainage.
procedureMultidisciplinary Supportive Care
Coordinated care involving gastroenterology, urology, nutrition and developmental specialists to manage complex needs. Regular monitoring of growth, hydration and organ function.
monitoringMobility and Rehabilitation Support
Physical therapy to maintain muscle tone, prevent deconditioning and support developmental milestones. Includes gentle range-of-motion exercises appropriate for severely ill patients.
physical_therapyWhen 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 4 C5543519?
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.
MMIHS Consortium. Consensus Guidelines for the Management of Megacystis Microcolon Intestinal Hypoperistalsis Syndrome. Orphanet J Rare Dis. 2022.
Tallapaneni V, Nakashima A, Kawaguchi M. ACTG2-Related Megacystis Microcolon Intestinal Hypoperistalsis Syndrome: Clinical Features and Genetic Insights. Pediatr Surg Int. 2021.
Matsumoto K, Rodriguez-Fuentes M, Garcia-Dios D. Multicenter Observational Study of Intestinal Rehabilitation in MMIHS Patients. J Pediatr Gastroenterol Nutr. 2023.
Wang H, Schreiber C, Kapor A. Molecular Pathogenesis of ACTG2 Mutations in Visceral Myopathy: A Systematic Review. Neuromuscular Disord. 2020.
Medication Comparisons
Compare medications used to treat Megacystis Microcolon Intestinal Hypoperistalsis Syndrome 4 C5543519. 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