Multidisciplinary approach to the complex treatment for non-cirrhotic portal hypertension – case-report-based discussion

Non-cirrhotic portal vein thrombosis (PVT) in patients with antiphospholipid syndrome (APS) is a rare complication, and the management has to be determined individually based on the extent and severity of the presentation. We report on a 37-year-old male patient with non-cirrhotic chronic PVT related to a severe thrombophilia, comprising APS, antithrombin-, factor V- and factor X-deficiency. Three years after the initial diagnosis of non-cirrhotic PVT, the patient presented with severe hemorrhagic shock related to acute bleeding from esophageal varices, requiring an emergency transjugular intrahepatic portosystemic stent shunt (TIPSS). TIPSS was revised after a recurrent bleeding episode due to insufficient reduction of the portal pressure. Additionally, embolization of the dilated V. coronaria ventriculi led to the regression of esophageal varices but resulted simultaneously in a left-sided portal hypertension (LSPH) with development of stomach wall and perisplenic varices. After a third episode of acute esophageal varices bleeding, a surgical distal splenorenal shunt (Warren shunt) was performed to reduce the LSPH. Despite anticoagulation with low molecular weight heparin and antithrombin substitution, endoluminal thrombosis led to a complete Warren shunt occlusion, aggravating the severe splenomegaly and pancytopenia. Finally, a partial spleen embolization (PSE) was performed. In the postinterventional course, leukocyte and platelet counts increased rapidly and the patient showed no further bleeding episodes. Overall, this complex course demonstrates the need for individual assessment of multimodal treatment options in non-cirrhotic portal hypertension. This young patient required triple modality porto-systemic pressure reduction (TIPSS, Warren shunt, PSE) and involved finely balanced anticoagulation and bleeding control.

Warren shunt combined with partial splenectomy in children with extra-hepatic portal hypertension, massive splenomegaly and severe hypersplenism

Introduction. Extra-hepatic portal vein obstruction (EHPVO) is one of the most often causes of portal hypertension in children. Objective. Establishing the importance of shunt surgery in combination with partial spleen resection in selected pediatric patients with EHPVO, enormous splenomegaly and severe hypersplenism. Methods. Distal splenorenal shunt (DSRS) with partial spleen resection was performed in 22 children age from 2 to 17 years with EHPVO. Indications for surgery were pain and abdominal discomfort caused by spleen enlargement, as well as symptomatic hypersplenism with leucopenia, thrombocytopenia and anemia. The partial spleen resection was performed by ligation of blood vessels to caudal two thirds of the spleen. After ischemic parenchymal demarcation transection with electrocautery LigaSure was performed with preservation of 20-30% of spleen tissue, and then Warren DSRS was created. Platelet and leucocytes counts and liver function tests were obtained before, one month and one year after surgery. Growth was assessed with SD scores (Z scores) for height, weight and body mass index at the time of surgery and one year later. Results. In all patients postoperative period was without significant complications. Platelets and leucocytes counts were normalized. Patency rate of shunts was 100%. Two significant shunts stenosis were observed and successfully treated with percutaneous angioplasty. During the follow-up period (1 to 9 years) all patients were asymptomatic, with improved quality of life and growth. Conclusion. Results of our study indicate that shunt surgery with a partial spleen resection is an effective and safe procedure for patients with enormous splenomegaly and severe hypersplenism caused by EHPVO.

Does the Warren Shunt Correct Hypersplenism?

It has been suggested that patients with bleeding varices and hypersplenism will show significant improvements in leucocyte and platelet counts following distal splenorenal (Warren) shunt surgery. Whilst this may be true in the short term, this report shows that in the long term hypersplenism is not relieved, whereas the lienorenal shunt is associated with a return of normal haematological values.