TIPS and splenorenal shunt for complications of portal hypertension in chronic hepatosplenic schistosomiasis-A case series and review of the literature

Author summaryHepatosplenic schistosomiasis is a severe form of chronic infection with various trematodes of the genus Schistosoma, characterized by portal venous fibrosis, splenomegaly with hypersplenism, and portal hypertension with subsequent life-threatening bleeding events. While effective anthelminthic treatment is available, portal fibrosis is only partially reversible. Portal hypertension with subsequent bleeding events as a complication is hitherto insufficiently addressed. Surgical techniques are currently the best established treatment option for HSS, despite their inherent complication risk and irreversibility. Interventional procedures like TIPS have rarely been assessed in HSS with mixed results. In a series of 13 cases with follow-up periods up to 99 months and a cumulative follow-up of 30.9 years, we demonstrate excellent bleeding prophylaxis and a low adverse event rate of TIPS and-if TIPS proves infeasible-splenorenal shunt surgery. Main complications of TIPS comprise transient hepatic encephalopathy and increase of liver enzymes, especially in patients with hepatic comorbidities. Due to necessary infrastructure and skills, TIPS implantation is currently limited to specialized centers. However, we think that our study can support the establishment and development of new treatment options for schistosomiasis and, in the medium term, also improve the prognosis of this neglected tropical disease in endemic regions. BackgroundTransjugular intrahepatic portosystemic shunt (TIPS) and shunt surgery are established treatment options for portal hypertension, but have not been systematically evaluated in patients with portal hypertension due to hepatosplenic schistosomiasis (HSS), one of the neglected tropical diseases with major impact on morbidity and mortality in endemic areas. MethodsIn this retrospective case study, patients with chronic portal hypertension due to schistosomiasis treated with those therapeutic approaches in four tertiary referral hospitals in Germany and Italy between 2012 and 2020 were included. We have summarized pre-interventional clinical data, indication, technical aspects of the interventions and clinical outcome. FindingsOverall, 13 patients with confirmed HSS were included. 11 patients received TIPS for primary or secondary prophylaxis of variceal bleeding due to advanced portal hypertension and failure of conservative management. In two patients with contraindications for TIPS or technically unsuccessful TIPS procedure, proximal splenorenal shunt surgery in combination with splenectomy was conducted. During follow-up (mean follow-up 23 months, cumulative follow-up time 31 patient years) no bleeding events were documented. In five patients, moderate and transient episodes of overt hepatic encephalopathy were observed. In one patient each, liver failure, portal vein thrombosis and catheter associated sepsis occurred after TIPS insertion. All complications were well manageable and had favorable outcomes. ConclusionsTIPS implantation and shunt surgery are safe and effective treatment options for patients with advanced HSS and sequelae of portal hypertension in experienced centers, but require careful patient selection