Is Hepatovenocaval Syndrome a Different Entity from Budd-Chiari Syndrome in Children?

OBJECTIVE

To differentiate between clinical and demographic spectrum, and outcome in hepatovenocaval syndrome (HVCS) and Budd-Chiari syndrome (BCS).

STUDY DESIGN

Descriptive study.

PLACE AND DURATION OF STUDY

Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital, Lahore, from January 2014 to January 2017.

METHODOLOGY

All children less than 18 years of age, presenting with ascites and visible veins over abdomen, flanks and back were enrolled in the study. Real time Doppler Ultrasonogram was performed in all children for documentation of intra- hepatic part of IVC obstruction along with or without hepatic venous obstruction. Children meeting inclusion criteria underwent liver profile, coagulation profile, diagnostic paracentesis for SAAG gradient, and Gadolinium enhanced multiphasic MR scan. Liver biopsy and venography was performed in selected patients.

RESULTS

A total of 92 children presented with ascites, among them 58 children met our inclusion criteria. Intrahepatic IVC obliteration, i.e. HVCS, found in 67% (n=39) and hepatic venous outflow obstruction, i.e. BCS was found in 33% (n=19) children. Children with BCS were older than HVCS with mean age of 9.5 ±2.58 versus 4.12 ±0.977 years. HVCS group had 14 boys and 24 girls with a ratio of 1:1.8, while BCS had a ratio of 1:0.9 with 10 boys and 9 girls. No etiological factor was found for HVCS, while most of patients with BCS had a procoagulant disorder. Caudate lobe hypertrophy was a consistent feature in BCS, while IVC obstruction was found in HVCS persistently. Orthotopic liver transplant was needed in three cases (7.6%) of HVCS and four (20.96%) of BCS cases. Antibiotic therapy has a good role in HVCS, while anticoagulation and diuretics had good result in BCS.

CONCLUSION

Hepatovenocaval syndrome (HVCS) mostly affected younger children, especially girls. BCS usually affected older age groups with pro-coagulant disorders who responded to anticoagulation and diuretic. Further studies are needed to compare both conditions.