Abstract
Among the important etiologies of pediatric portal hypertension, Budd–Chiari syndrome (BCS) is a potentially treatable cause. Pediatric BCS has distinctive differences as compared to adults in terms of etiology, natural history, and management. Predominant clustering in Asia and absence of a true underlying thrombophilia are unique issues in children. During the management of ascites and portal hypertension, an early and optimal therapeutic window is sought for radiological intervention which is the cornerstone of outcome. Endovascular management has challenges in children due to varying age, weight, and size of pediatric liver. Choice of procedure and intervention hardware (guidewires, balloons, and appropriate sized stents) needs to be customized accordingly. The overall vascular patency rates after radiological intervention are 87%, 82%, and 62% at 1, 5, and 10 years of follow-up in chronic BCS. Procedural complications are seen in 1–3%. Prior to endovascular intervention, pediatric end-stage liver disease (PELD) score <4 predicts good response to intervention. Zeitoun index >4.3 in unintervened chronic BCS children require an urgent radiological procedure. Intervention is also recommended in an asymptomatic BCS. Concerns in children are issues related to stents, number of procedures, dangers of lifelong anticoagulation, and searching for the underlying etiology.
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Yachha, S.K., Sen Sarma, M., Srivastava, A. (2020). Budd–Chiari Syndrome in Children. In: Qi, X. (eds) Budd-Chiari Syndrome. Springer, Singapore. https://doi.org/10.1007/978-981-32-9232-1_16
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DOI: https://doi.org/10.1007/978-981-32-9232-1_16
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