Portal hypertension after combined liver and intestinal transplantation, a diagnostic and therapeutic challenge?

Abstract

3 Abstract A widely accepted technique to transplant the liver-bowel bloc is first to perform a piggyback anastomosis of the donor suprahepatic vena cava to the recipient vena cava; second to restore the arterial blood supply through an aortic interposition graft; and third to insure venous drainage of the native foregut. The venous drainage of the native foregut can be restored through an end-to-end portocaval anastomosis between the donor infrahepatic vena cava and the recipient portal vein.Stenosis of this anastomosis can lead to portal hypertension presenting with upper gastrointestinal congestion, bleeding and hypersplenism. We report the successful treatment of this complication using an expanded poly-tetra-fluoro-ethylene-covered stent inserted following balloon angioplasty.4

Late Bleeding Episodes Following Intestinal Transplantation: It Is Not Always Rejection or Infection

“…Gondolesi et al ( 4 ) suggested a modification of the conventional technique to an end-to-end anastomosis of native portal vein and donor IVC to mitigate the risks of development of varices. However, despite the adaptation of the technique, there have been case reports of development of thrombosis within the portacaval shunt ( 5 ). The portacaval shunt post liver intestinal transplant may have higher propensity for thrombosis due to the following reasons: (1) the hyperdynamic circulation present pretransplant in children with intestinal failure liver disease and portal hypertension is changed to a “low flow” system as it only drains part of the stomach, jejunum, and spleen; (2) correction of coagulation with a new functioning liver; and (3) presence of a procoagulant environment in immediate post-transplant period ( 7 ).…”

“…There is scant literature on how to treat ectopic variceal bleeding in those who have undergone Itx. The 3 existing reports on portal hypertensive bleeding in the liver-Itx cohort utilized a balloon dilation of stenosed shunt ( 5 ), side-to-side distal splenorenal shunt after unsuccessful balloon dilation of the portacaval anastomosis ( 3 ), and an end-to-end anastomosis for portosystemic shunts rather than end to side, potentially creating higher flow ( 4 ). Anastomotic technique in the portacaval shunt of case 1 was end to side.…”

“…Ectopic varices may present in the duodenum (17%), jejunum or ileum (18%), colon (14%), rectum (8%), and peritoneal region (9%) (1,2). Reports of ectopic variceal bleeding from portal hypertension as a consequence of portosystemic shunt blockage post pediatric liver-intestinal transplant (Itx) are extremely rare (3)(4)(5). The purpose of this case description is to highlight this etiology and other rare causes, such as development of chronic liver disease post isolated Itx in the Itx cohort.…”