Budd-Chiari Syndrome-A Single Center Experience From the United Kingdom
Abstract
Pediatric Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver disease in Europe and North America. In order to understand the long-term effect of radiological intervention on BCS we performed a single center retrospective review. Fourteen cases were identified; 6 of 14 (43%) had a congenital thrombophilia with many having multiple prothrombotic mutations. Two were managed with medical anticoagulation alone and two required super-urgent transplant for acute liver failure. The remaining 10 of 14 (71%) underwent radiological intervention: 1 of 14 thrombolysis, 5 of 14 angioplasty, and 4 of 14 transjugular intrahepatic portosystemic shunt (TIPS). Six of 14 (43%) patients required repeat radiological intervention (1 angioplasty, 5 TIPS) but none required surgical shunts or liver transplantation for chronic liver disease. The time between diagnosis and treatment did not predict the need for repeat radiological intervention. These data show that radiological intervention can be highly effective, and reduces the need for surgery, though it requires specialist multidisciplinary teams for monitoring.
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Date
2023-06-14
Type
Article
Subject
Gastroenterology
Collections
Citation
Mann JP, Ikram F, Modin L, Kelgeri C, Sharif K, Olliff S, McGuirk S, Gupte GL. Budd-Chiari Syndrome-A Single Center Experience From the United Kingdom. J Pediatr Gastroenterol Nutr. 2023 Oct 1;77(4):455-459. doi: 10.1097/MPG.0000000000003860. Epub 2023 Jun 14. PMID: 37314703.
Journal / Source Title
Journal of Pediatric Gastroenterology and Nutrition
DOI
10.1097/MPG.0000000000003860
PMID
37314703
Publisher
Lippincott, Williams & Wilkins
Publisher’s URL
https://journals.lww.com/jpgn/pages/default.aspx