[Budd-Chiari syndrome (case report)]
Insights
Budd-Chiari syndrome, a rare hepatic venous outflow obstruction, is uncommon in children. Early diagnosis and referral to pediatric specialists are crucial for effective management, as highlighted by a challenging adolescent case.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Vascular Medicine
Background:
- Budd-Chiari syndrome involves hepatic venous outflow obstruction, rare in pediatric populations.
- Diagnosis in children is often delayed due to its rarity and complex presentation.
- Ascites can be a primary symptom in pediatric cases.
Observation:
- A 16-year-old adolescent presented with chronic refractory Budd-Chiari syndrome.
- The primary clinical manifestation was significant ascites.
- The case required surgical intervention with a transjugular intrahepatic portosystemic shunt.
Findings:
- The case underscores diagnostic challenges in pediatric Budd-Chiari syndrome.
- Refractory ascites indicated severe hepatic venous obstruction.
- Surgical shunt provided a treatment pathway for this complex case.
Implications:
- A high index of suspicion is vital for diagnosing pediatric Budd-Chiari syndrome.
- Early referral to specialized pediatric units improves patient outcomes.
- This case highlights the need for increased awareness and timely intervention in pediatric hepatology.
Abstract:
Budd-Chiari syndrome is a rare disease defined by the obstruction of hepatic venous outflow anywhere from the small hepatic veins to the junction of the inferior vena cava and the right atrium. This syndrome is uncommon in children. The article presents a case report of chronic refractory Budd-Chiari syndrome with a leading ascites in 16 years adolescent, which required surgical intervention--transjugular intrahepatic porta systemic shunt. As this syndrome is uncommon in pediatric practice, complex differential diagnostic search and delays in the diagnosis are frequent. This case report emphasizes the importance of a high index of suspicion in the diagnosis of pediatric Budd-Chiari syndrome and highlights the importance of early referral to a specialized pediatric unit for the further treatment.
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