In the long-term after liver transplantation (LT), some children develop prehepatic portal hypertension (PPH) and raise problems not very well known yet; many of the lessons learned with the management of these patients may be useful outside the LT.
Aim: 1. To analyze the incidence and risk factors of PPH after LT. 2. To evaluate the results with the different treatments used.
Methods: Retrospective study over 164 children surviving more than 1 year after LT. Univariant analysis of possible risk factors associated and multivariant (logistic regression), for those that had significance in the univariant analysis. Other factors associated are analyzed as well as the indications and results of two types of treatment: percutaneous pneumatic dilatation and surgical shunt (splenorenal and Rex shunt).
Results: 9 children developed symptomatic PPH (hemorrhage in 8, ascites in 1), associated to lymphoproliferative post-LT disease in 2, and to anastomotic biliary stricture in 1. The age at first LT (children under 1 year old), weight (below 10 kg), and need of retransplantation (reLT) were in the univariant analysis the associated variables with increased risk of PPH. The diagnosis (biliary atresia) and the emergency status of the LT were almost significative. In the multivariant analysis, the need of reLT is the only independent variable that increases the risk (relative risk: 4.5, confidence interval 95%: 1.29-18.87). At diagnosis 3 cases showed portal estenosis, and 5 showed absence of permeability with cavernomatous transformation. The PPH was caused in one case because of the esplenic vein disconnection (treatment not required at the moment); the three cases of portal estenosis were dilated percutaneously with success, and 2 of the 5 cases with portal thrombosis have been surgically shunted: one by an splenorenal shunt and the other by a Rex shunt (first case done in Spain); the other 3 cases are stable waiting for a surgical solution. The hepatic function is normal in the 9 cases.
Conclusions: The PPH can complicate the prognostic of the pediatric LT in the long term. The treatment depends on the permeability of the portal trunk. Whenever possible, percutaneous dilatation should be attempted; should surgery be required, the Rex shunt is the best option.