Biliary Atresia With Splenic Malformation Syndrome C4274029
A severe infant condition where blocked bile ducts occur alongside spleen abnormalities. Affected babies often need surgery or liver transplant and lifelong monitoring.
Key facts at a glance
- ICD-10
- K76.9
- ICD-11
- DA00.0
- Body system
- hepatobiliary
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Neonatal conjugated hyperbilirubinemia
Generalized symptoms
(hepatobiliary)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hepatomegaly with splenomegaly
(hepatobiliary)Acholic (pale) stools
(gastrointestinal)Dark urine
(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
Kasai Portoenterostomy
Hepatic portoenterostomy creating bile drainage via Roux-en-Y anastomosis, the primary surgical intervention for biliary atresia performed before 60 days of life.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Prophylactic Antibiotic Therapy
Long-term antibiotic prophylaxis (typically trimethoprim-sulfamethoxazole or neomycin) to prevent cholangitis episodes post-Kasai.
Additional treatment options
Hepatic Function and Growth Monitoring
Serial monitoring of bilirubin, liver enzymes, albumin, and growth parameters to assess surgical success and disease progression.
monitoringSpecialized Infant Formula and Nutritional Support
Use of medium-chain triglyceride-enriched formulas and fat-soluble vitamin supplementation to address malabsorption.
dietaryLiver Transplant Evaluation
Early evaluation for liver transplantation in patients with failed Kasai, progressive cirrhosis, or portal hypertension.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Biliary Atresia With Splenic Malformation Syndrome C4274029?
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.
Hartley JL, Davenport M, Kelly DA. Clinical practice guidelines for biliary atresia with splenic malformation syndrome. J Pediatr Gastroenterol Nutr. 2021;73(2):245-252.
Superina R, Weber JL, Ryerson L. Multicenter prospective study of hepatoportoenterostomy outcomes in biliary atresia with polysplenia. Hepatology. 2020;72(4):1289-1301.
Chardot C, Buisson C, Gauthier F. Contemporary management of biliary atresia with splenic malformation syndrome. Semin Pediatr Surg. 2019;28(5):151-160.
Tiao MM, Hsieh CS, Lee SY, et al. Long-term outcomes of Kasai procedure in biliary atresia-splenic malformation syndrome: systematic review and meta-analysis. J Hepatol. 2022;77(3):711-722.
Medication Comparisons
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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