P ortal hypertension is classically caused by cirrhotic liver disease. However, there are several etiologies of portal hypertension that do not coincide with liver failure. Noncirrhotic portal hypertension may manifest clinically by upper gastrointestinal (GI) bleeding from esophageal varices, leukopenia, and thrombocytopenia from splenomegaly. 1,2 While studies have shown that certain liver functions are affected as a result of reduced portal venous flow, ascites and clinical encephalopathy are not common given the lack of intrinsic liver dysfunction.1-8 Noncirrhotic etiologies of portal hypertension are classified as prehepatic, hepatic, and posthepatic. Prehepatic causes are associated with thrombosis within the portal venous system. Hepatic causes include noncirrhotic portal fibrosis and schistosomiasis. Finally, Budd-Chiari syndrome, inferior vena cava (IVC) thrombosis/occlusion, and constrictive pericarditis are posthepatic etiologies of portal hypertension.In pediatric patients, portal vein thrombosis (PVT), or extrahepatic portal vein obstruction (EHPVO), is the most common cause of noncirrhotic portal hypertension. The pathophysiology of PVT is poorly understood, although studies have identified several pre-disposing factors, including neonatal umbilical vein catheterization and hypercoagulable states.9,10 Indications for surgical intervention on these complex children are controversial, but include refractory variceal hemorrhage, severe thrombocytopenia, severe leukopenia, encephalopathy, and porto-pulmonary hypertension.2 There are various surgical interventions available that attempt to improve the portal flow, including the meso-Rex bypass, 11 distal splenorenal shunt, 12,13 splenectomy followed by proximal splenorenal shunt, and the mesocaval shunt. 14 The aforementioned procedures are all significant operations and have a broad range of potential complications, including shunt thrombosis. Consideration of these risks is important, given that many patients have been reported to do well long-term without any surgical intervention.
1,15While surgery is often recommended following a GI bleed requiring blood transfusion, specific indications for surgical intervention are unclear. Further, with the development of advanced endovascular techniques, clinical decision-making has become even more complex. We have recently established a pediatric portal hypertension program at our institution, which includes hepatologists, interventional radiologists, radiologists, and surgeons. As we develop our own protocols for the management of children with noncirrhotic portal hypertension, we have reviewed Background/Objectives: The optimal management of children with noncirrhotic portal hypertension is controversial. Some groups suggest early and aggressive surgical intervention, while others report long-term success with conservative management. Methods: We conducted a retrospective study of 26 patients with noncirrhotic portal hypertension treated at our institution. We compared platelet counts, white blood cell (WBC...