TY - JOUR
T1 - Prehepatic portal hypertension with aneurysm of the portal vein
T2 - Unusual but treatable malformative pattern
AU - Bertocchini, Arianna
AU - D'Ambrosio, Giuseppe
AU - Grimaldi, Chiara
AU - Del Prete, Laura
AU - Di Francesco, Fabrizio
AU - Falappa, Piergiorgio
AU - Monti, Lidia
AU - De Ville De Goyet, Jean
PY - 2014
Y1 - 2014
N2 - Introduction Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear. Methods Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography. Results Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time. Conclusions These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension.
AB - Introduction Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear. Methods Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography. Results Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time. Conclusions These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension.
KW - Extrahepatic portal hypertension
KW - Intrahepatic portal vein
KW - Meso Rex bypass
KW - Rex recessus
KW - Vein Aneurysm
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U2 - 10.1016/j.jpedsurg.2013.06.012
DO - 10.1016/j.jpedsurg.2013.06.012
M3 - Article
C2 - 24650473
AN - SCOPUS:84896514391
VL - 49
SP - 436
EP - 440
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 3
ER -