Portal Hypertension in Childhood Bilateral Wilms' Tumor Survivor: An Excellent Indication for TIPS

Introduction. Increased pressure in portal venous system is relatively a rare complication after chemoradiotherapy for Wilms' tumor (WT). In paediatric population, feasibility and efficacy of transjugular intrahepatic portosystemic shunt (TIPS) in portal hypertension nonresponsive to medical or endoscopic treatment have been recently advocated. We report a case of TIPS positioning in a 15-year-old girl with portal hypertension as a long-term sequel of multimodality therapy in bilateral WT. Case Report. Two-year-old girl was diagnosed for bilateral WT. Right nephrectomy with left heminephrectomy and chemoradiotherapy were performed. At 7 years of age, the first gastrointestinal bleeding appeared, followed by another episode two years later, both were treated successfully with beta-blockers. At 15 years of age, severe unresponsive life-threatening gastroesophageal bleeding without hepatosplenomegaly was managed by TIPS. Reduction of the portosystemic pressure gradient was obtained. Conclusion. TIPS positioning for portal hypertension in long-term tumors' sequel is feasible and could be considered as an additional indication in paediatric patients. 1. Introduction Perinatal events like umbilical catheterization, thrombophilic states, tumors, and abdominal infection are the main causes of hepatic injury leading to portal hypertension (PH) [1]. Children submitted to chemoradiotherapy for Wilms’ tumor (WT) could also end in PH caused by portal vein fibrosis [2]. A wide range of therapeutic modalities, including medical and surgical treatments for PH, has been adopted. The transjugular intrahepatic portosystemic shunt (TIPS) has been introduced as an alternative safe management for complicated PH even in pediatrics [3, 4]. We report a unique case of TIPS positioning for PH observed in a 15-year-old girl subsequent to multimodality therapy for bilateral WT. 2. Case Report A 2-year-old girl underwent right nephrectomy and left heminephrectomy for bilateral WT. Chemotherapy (Vincristine and Actinomycin D) and radiotherapy were done. At 9 years of age, she developed the first episode of melena. Gastroscopy showed oesophageal white F1 varices. No hepatosplenomegaly or dilatation of portal vein was documented on CT scan. Two years later melena and hematemesis reappeared. Gastroscopy evidenced oesophago-gastric varices (F2 blu, red wale markings). Liver biopsy showed periportal fibrosis and portal interlobular venules obliterations, without signs of cirrhosis. Beta-blockers treatment was adopted with resolution of the gastrointestinal bleeding, and oesophageal