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Is Hepatovenocaval Syndrome a Different Entity from Budd-Chiari Syndrome in Children?
Nadia Waheed1, Huma Arshad Cheema1, Hassan Suleman1
1Department of Pediatric Gastroenterology and Hepatology, The Children Hospital, Lahore.
Insights
Hepatovenocaval syndrome (HVCS) predominantly affects younger girls, while Budd-Chiari syndrome (BCS) impacts older children with clotting disorders. Treatment varies, with antibiotics for HVCS and anticoagulation/diuretics for BCS.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Vascular Medicine
Background:
- Hepatovenocaval syndrome (HVCS) and Budd-Chiari syndrome (BCS) are distinct venous outflow obstructions affecting the liver.
- Differentiating these conditions is crucial for appropriate management and understanding their unique clinical presentations.
Purpose of the Study:
- To compare the clinical, demographic, and outcome differences between HVCS and BCS in pediatric patients.
- To identify specific etiological factors and treatment responses for each syndrome.
Main Methods:
- A descriptive study was conducted on 58 pediatric patients (<18 years) with ascites and abdominal vein distension.
- Diagnosis involved Doppler Ultrasonogram, liver profile, coagulation profile, MR scan, and in select cases, liver biopsy and venography.
- Patients were categorized into HVCS (intrahepatic IVC obstruction) and BCS (hepatic venous obstruction).
Main Results:
- HVCS was diagnosed in 67% of patients, predominantly younger children (mean age 4.1 years) and girls (1:1.8 ratio).
- BCS was found in 33% of patients, typically older children (mean age 9.5 years) with a near 1:1 sex ratio and associated procoagulant disorders.
- Caudate lobe hypertrophy was characteristic of BCS, while IVC obstruction was persistent in HVCS. Liver transplant was required in 7.6% of HVCS and 20.96% of BCS cases.
Conclusions:
- HVCS primarily affects younger children, particularly girls, and responds well to antibiotic therapy.
- BCS is more common in older children with procoagulant disorders, showing good response to anticoagulation and diuretics.
- Further research is recommended to delineate the distinct characteristics and management strategies for HVCS and BCS.
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.
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