Progression of Noncirrhotic Portal Hypertension in a Pediatric Population

Abstract

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...

Natural Course of Pediatric Portal Hypertension

“…(17) This finding differed from a study that analyzed 26 patients with noncirrhotic pHTN and found that the majority did well in the long term without surgical intervention. (3) In our study, the majority of patients who had an intervention underwent a transplantation and only a portion of these patients had a pre-operative gastrointestinal bleed. While variceal bleeding may be associated with confirmed gastrointestinal bleeding on endoscopy in patients with PRE who undergo a shunt, (10) it was a poor predictor for undergoing surgical intervention for pHTN in this study.…”

“…While alcoholic, nonalcoholic steatohepatitis, and viral-induced cirrhosis are the most common causes of pHTN in adults in Western countries, intrahepatic (biliary atresia) and prehepatic (portal vein thrombosis) etiologies predominate in the pediatric population. (1)(2)(3)(4) A paucity of data in the literature exists regarding the outcomes and evidence-based management of pHTN in children, highlighting the need for further understanding of these variations.…”

Pediatric portal hypertension

“…The current scoring system has been adopted from adult practice; however, it has not been validated in the literature 1,2,11. One retrospective study conducted in a single academic hospital setting found that the majority of patients with noncirrhotic portal hypertension did well long term without surgical shunt placements, and treatment focused mainly on surveillance and treatment of varices 12. However, this practice has not been well studied and reviewed in the literature 12…”