Abstract
The management of complications of portal hypertension in children remains a challenge due to limited evidenced-based approaches and the marked heterogeneity of the relevant pediatric disorders. It is not necessarily appropriate to apply treatment paradigms developed for adults to children given the marked differences in the pathophysiology of the operant diseases. This review summarizes progress made in the diagnosis and management of pediatric portal hypertension since Baveno V, with a focus on issues related to varices in two disorders, extrahepatic portal venous obstruction (EHPVO) and biliary atresia (BA). Portal hypertension in both diseases presents in the context of relatively preserved hepatocellular function, which distinguishes this from experiences with adults. Approaches to secondary prophylaxis of variceal hemorrhage seem more secure than either screening for or primary prophylaxis of varices. Response to the Kasai hepatoportoenterostomy for BA, in particular the establishment of bile flow, has a potentially marked impact on progression of not only complications of portal hypertension but overall short-term prognosis and therefore the approach to the management of portal hypertension. The ability of the mesoRex bypass to “cure” EHPVO in some children has profound implications for optimal management of this disease. In pediatrics, generalized guidelines are difficult to establish, and a complete understanding of the natural history of a particular pediatric disorder along with knowledge of the pros and cons of potential interventions is critical for relatively informed clinical decision-making.
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Shneider, B.L. (2016). Portal Hypertension in Pediatrics: Controversies and Challenges 2015 Report. In: de Franchis, R. (eds) Portal Hypertension VI. Springer, Cham. https://doi.org/10.1007/978-3-319-23018-4_29
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DOI: https://doi.org/10.1007/978-3-319-23018-4_29
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