Transjugular Intrahepatic Portosystemic Shunt For Portal Vein Thrombosis With Portal Hypertensive Complication

?Background?Portal vein thrombosis(PVT) includes non-cirrhotic PVT and cirrhotic PVT. The chronic non-cirrhotic PVT is also named extra-hepatic portal venous obstruction(EHPVO). EHPVO, leading to portal hypertension, is the most common cause of upper gastrointestinal bleeding in children in developing countries. Growth retardation, splenomegaly and hypersplenism are the other common presenting features, which greatly affect patient quality of life. PVT is a critical but fairly common event in patients with cirrhosis, with a prevalence ranging from 2% to 23%. PVT may deteriorate the prognosis of cirrhotic patients because it worsen liver function through decreasing liver perfusion and increase risk of complications via further increasing portal hypertension. Moreover, complete or extensive PVT complicates the liver transplant operation and increase post-transplantation morbidity and mortality.Transjugular intrahepatic portosystemic shunt(TIPS) is the mainstay treatment option for the complications of portal hypertension in liver cirrhosis. However, in the setting of PVT, the technical difficulty and risk of TIPS is remarkably increased. Experience of TIPS in children with EHPVO is extremely limited, with only a few cases reported. The feasibility, safety, and efficacy of the TIPS for the treatment of EHPVO in children with symptomatic portal hypertension is still unknown. Several small case series have explored the feasibility and safety of TIPS in patients with PVT. However, it is still unknown whither there are differences in post-TIPS outcomes comparing patients with PVT to those without PVT.?Aims?1. To evaluate the feasibility and efficacy of transjugular intrahepatic portosystemic shunt(TIPS) for extra-hepatic portal venous obstruction(EHPVO) with recurrent variceal bleeding(RVB) in children.2. To determine whether there are differences in post-TIPS outcomes comparing patients with PVT to those without PVT.?Methods?1. From November 2005 to December 2013, 28 consecutive paediatric patients with EHPVO treated with TIPS for RVB refractory to medical/endoscopic therapy and/or surgical treatment in a tertiary-care centre were followed until last clinical evaluation or death.2. From March 2001 to December 2014, 1171 consecutive patients with cirrhosis treated with TIPS for acute variceal bleeding(n=175), recurrent variceal bleeding(n=867) or refractory ascites(n=129) in a tertiary-care centre were followed until last clinical evaluation or death. Cox proportional hazards model was used to compare post-TIPS outcomes between patients with PVT(n =212; 18%) and without PVT(n =959; 82%) after adjusting for baseline characteristics.?Results?1. Seventeen boys and eleven girls aged 7.1 to 17.9 years(median 12.3 years) weighing 19.0-62.0 kg(median 33.5 kg) were treated. The median follow-up time was 36.0 months(range 4.0-106.0 months). TIPS was successfully placed in 17 of 28(60.7%) of patients via a transjugular approach alone(n=4), a combined transjugular/transhepatic approach(n=9), or a combined transjugular/transsplenic approach(n=4). Shunt dysfunction occurred in 6 of 17(35.3%) patients. The cumulative 1- and 3- year free- from- variceal-rebleeding rates in TIPS success group were higher than those in TIPS failure group(75.0% and 67.5% versus 45.5% and 18.2%, respectively, p=0.0075). Compared with the TIPS failure group, the improvements in the height-for-age z-scores were greater in the TIPS success group(p=0.017). Procedure-related complication occurred in one patient(3.6%), and no episode of post-TIPS hepatic encephalopathy occurred in any patient. Except one patient in the TIPS success group died at 115 post-operative days, all patients were alive.2. During a median follow-up period of 28.2(range, 0-177.2) months, 217(19%) patients developed shunt dysfunction, 349(30%) experienced symptom recurrence, 558(48%) developed hepatic encephalopathy(HE), 507(43%) died and 28(2%) underwent transplantation. Compared with patients without PVT, patients with PVT had a similar risk of shunt dysfunction(adjusted hazard ratio [AHR], 1.190; 95% confidence interval [CI], 0.766-1.598), symptom recurrence(AHR, 1.208, 95% CI, 0.880-1.657), or HE(AHR, 0.871; 95% CI, 0.680-1.116), and a lower risk of the combined outcome of death or transplantation(AHR, 0.742; 95% CI, 0.565-0.975). After stratifying according to degree of PVT, a reduced mortality was only associated with partial PVT but not complete PVT.?Conclusions?1. TIPS is feasible and effective in children with EHPVO and RVB. TIPS could represent a less invasive alternative to traditional surgical portosystemic shunting or a valuable treatment option if surgery and endoscopic treatment failed.2. Among cirrhotic patients undergoing TIPS, patients with PVT has similar rate of shunt dysfunction, symptom recurrence, HE and lower mortality than comparable patients without PVT. Prospective randomized studies should investigate whether TIPS is the best therapeutic option in cirrhotic patients with PVT.