TY - JOUR
T1 - Management of portal hypertension and ascites in polycystic liver disease
AU - Bernts, Lucas H. P.
AU - Drenth, Joost P. H.
AU - Tjwa, Eric T. T. L.
PY - 2019/11/1
Y1 - 2019/11/1
N2 - Patients suffering from polycystic liver disease may develop Hepatic Venous Outflow Obstruction, Portal Vein Obstruction and/or Inferior Caval Vein Syndrome because of cystic mass effect. This can cause portal hypertension, leading to ascites, variceal haemorrhage or splenomegaly. For this review, we evaluate the evidence to provide clinical guidance for physicians faced with this complication. Diagnosis is made with imaging such as ultrasound, computed tomography or magnetic resonance imaging. Therapy includes conventional therapy with diuretics and paracentesis, and medical therapy using somatostatin analogues. Based on disease phenotype various (non-)surgical liver-volume reducing therapies, hepatic or portal venous stenting, transjugular intrahepatic portosystemic shunts and liver transplantation may be considered. Because of complicated anatomy, use of high-risk interventions and lack of empirical evidence, patients should be treated in expert centres.
AB - Patients suffering from polycystic liver disease may develop Hepatic Venous Outflow Obstruction, Portal Vein Obstruction and/or Inferior Caval Vein Syndrome because of cystic mass effect. This can cause portal hypertension, leading to ascites, variceal haemorrhage or splenomegaly. For this review, we evaluate the evidence to provide clinical guidance for physicians faced with this complication. Diagnosis is made with imaging such as ultrasound, computed tomography or magnetic resonance imaging. Therapy includes conventional therapy with diuretics and paracentesis, and medical therapy using somatostatin analogues. Based on disease phenotype various (non-)surgical liver-volume reducing therapies, hepatic or portal venous stenting, transjugular intrahepatic portosystemic shunts and liver transplantation may be considered. Because of complicated anatomy, use of high-risk interventions and lack of empirical evidence, patients should be treated in expert centres.
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85074045401&origin=inward
UR - https://www.ncbi.nlm.nih.gov/pubmed/31505092
U2 - https://doi.org/10.1111/liv.14245
DO - https://doi.org/10.1111/liv.14245
M3 - Review article
C2 - 31505092
SN - 1478-3223
VL - 39
SP - 2024
EP - 2033
JO - Liver international
JF - Liver international
IS - 11
ER -