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[Budd-Chiari syndrome in children. Apropos of 7 cases]
T Boudhina1, N Ghram, S Ben Becher
1Service de Pédiatrie, Hôpital d'Enfants, Tunis, Tunisie.
Insights
This study reports seven pediatric Budd-Chiari syndrome cases, highlighting varied onset and constant hepatomegaly. Surgical shunts improved outcomes in two children, demonstrating potential treatment benefits for this rare liver condition.
Area of Science:
- Pediatric Hepatology
- Vascular Liver Diseases
- Gastroenterology
Background:
- Budd-Chiari syndrome is a rare condition involving hepatic vein obstruction.
- Pediatric presentation often differs from adults, necessitating specific diagnostic and management approaches.
- Understanding the varied clinical spectrum and potential etiologies in children is crucial for timely intervention.
Purpose of the Study:
- To describe the clinical characteristics, diagnostic methods, and outcomes of Budd-Chiari syndrome in a pediatric cohort.
- To investigate potential associated conditions and etiologies in children with Budd-Chiari syndrome.
- To evaluate the efficacy of portasystemic shunts in managing pediatric Budd-Chiari syndrome.
Main Methods:
- Retrospective case series analysis of seven pediatric patients diagnosed with Budd-Chiari syndrome.
- Diagnostic modalities included ultrasonography, cavography, hepatic veins angiography, and liver biopsy.
- Etiologic investigations explored potential underlying causes, including infections and autoimmune conditions.
Main Results:
- Hepatomegaly was a consistent finding across all seven cases.
- Onset varied from fulminant liver failure to insidious presentation with isolated hepatomegaly.
- Associated conditions included total villous atrophy, psoriasis, hepatitis B, hepatitis A, and giardiasis.
- Portasystemic shunts were performed in three patients, with two achieving good long-term health outcomes.
Conclusions:
- Budd-Chiari syndrome in children presents with diverse clinical features and variable liver function abnormalities.
- While definitive etiologies remain elusive in many cases, associated conditions may offer diagnostic clues.
- Portasystemic shunting appears to be a viable therapeutic option, offering significant long-term benefits for select pediatric patients.
Abstract:
Seven cases of Budd-Chiari syndrome are reported in children. The mode of onset was fulminant in one case with rapidly lethal liver failure, acute in 5 cases with rapid appearance of hepatomegaly and ascites and insidious in one case, with isolated hepatomegaly. Hepatomegaly, which is a constant sign, was present in the 7 patients. Ascites and collateral venous circulation were present in 6, splenomegaly in 2 and moderate jaundice in one only. Liver function tests, deeply abnormal in the patient with fulminant liver failure, was only slightly abnormal in the 6 others. Diagnosis was corroborated by ultrasonography, cavography, hepatic veins angiography and liver biopsy in 6 patients and by post mortem examination in the 7th. Etiologic investigations did not allow finding the cause of Budd-Chiari syndrome. However, this series can be distinguished by associated total villous atrophy in 3 cases, psoriasis in one, hepatitis B in one, hepatitis A and intestinal giardiasis in one. Portasystemic shunts were performed in 3 patients. One died in the immediate postoperative period, the 2 others are presently in good health with a 5 and 6 1/2 year-follow-up. One patient died rapidly from fulminant liver failure. Another, untreated, died 16 years after the onset of the disease, from an unknown cause. Two patients are lost to follow-up.