TY - JOUR
T1 - The Role of Liver Transplantation in the Treatment of Hereditary Hemorrhagic Telangiectasia
T2 - A Short Literature Review
AU - Scelzo, C.
AU - Greco, S.
AU - Bonanni, L.
AU - Di Cocco, P.
AU - D'Angelo, M.
AU - Laurenzi, C.
AU - Pisani, F.
AU - Famulari, A.
AU - Orlando, G.
PY - 2007/7
Y1 - 2007/7
N2 - The liver is involved in up to 73% of patients suffering from hereditary hemorrhagic telangiectasia (HHT), but only some of them become symptomatic. Although management is often conservative, sometimes a more aggressive approach is required. The role of surgery is still undefined. Open ligation, banding, or closure of the arteriovenous malformation feeding artery have been proposed but rejected, as they are followed by an unacceptably high incidence of complications, derived from ischemia of the biliary tree. Orthotopic liver transplantation (OLT) has been successfully attempted in 28 patients with cardiac, biliary, or portal hypertension as well as mixed clinical presentations. Twenty-four were alive at time of data collection. Cardiovascular and pulmonary functions have improved after the operation in most cases. Intrahepatic relapse of the hallmark lesion of the disease (telangiectasia and arterovenous malformation) has been recently described in two cases. OLT represents a valuable therapeutic option for hepatic-based HHT, provided early diagnosis and referral to a specialized unit.
AB - The liver is involved in up to 73% of patients suffering from hereditary hemorrhagic telangiectasia (HHT), but only some of them become symptomatic. Although management is often conservative, sometimes a more aggressive approach is required. The role of surgery is still undefined. Open ligation, banding, or closure of the arteriovenous malformation feeding artery have been proposed but rejected, as they are followed by an unacceptably high incidence of complications, derived from ischemia of the biliary tree. Orthotopic liver transplantation (OLT) has been successfully attempted in 28 patients with cardiac, biliary, or portal hypertension as well as mixed clinical presentations. Twenty-four were alive at time of data collection. Cardiovascular and pulmonary functions have improved after the operation in most cases. Intrahepatic relapse of the hallmark lesion of the disease (telangiectasia and arterovenous malformation) has been recently described in two cases. OLT represents a valuable therapeutic option for hepatic-based HHT, provided early diagnosis and referral to a specialized unit.
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U2 - 10.1016/j.transproceed.2007.05.022
DO - 10.1016/j.transproceed.2007.05.022
M3 - Article
C2 - 17692689
AN - SCOPUS:34547609439
VL - 39
SP - 2045
EP - 2047
JO - Transplantation Proceedings
JF - Transplantation Proceedings
SN - 0041-1345
IS - 6
ER -