Medical and surgical management of portal hypertension in children

Abstract

The treatment of portal hypertension in children has undergone considerable evolution in the past decade. The treatment offered depends on the cause of the hypertension and the underlying health of the liver. The diagnosis of portal hypertension often can be made by the history and physical examination. Upper gastrointestinal bleeding in the presence of splenic enlargement is pathognomonic for portal hypertension. Bleeding and hypersplenism are the principal symptoms. Treatment of bleeding starts with confirming the diagnosis with esophageal and gastric endoscopy. The patient is admitted to an intensive care unit and started on intravenous octreotide. Banding or sclerosis of esophageal varices will result in cessation of the bleeding but not a permanent cure. A careful investigation for the cause of the portal hypertension should be done. This includes imaging studies of intra-abdominal arteries and veins, a liver biopsy, and liver function tests, including coagulation studies. For patients with extrahepatic portal vein thrombosis, early consideration should be given to surgical treatment with a meso-Rex bypass. Patients with liver disease should be treated for the underlying disorder and undergo regular endoscopic monitoring for recurrence of varices. Patients with well-compensated cirrhosis should be considered for selective surgical shunting, and those with advanced disease for liver transplantation. The benefit of long-term beta blockers in children has not been proven by clinical trials.

Growth impairment in children with extrahepatic portal vein obstruction is improved by mesenterico-left portal vein bypass

“…As a result, the presence and severity of the complications of portal hypertension such as variceal bleeding and hypersplenism have determined if affected children required treatment. Surgical shunting of blood from the portal circulation has been the mainstay of such treatment, with the distal splenorenal shunt being the preferred approach until recently [3][4][5]. This approach has proven effective in reducing the morbidity associated with portal hypertension in children with EHPVO.…”

“…Application of the mesenterico-left portal vein bypass (MLPVB) has fundamentally changed the management of EHPVO [4,6,7]. The procedure was initially used to correct EHPVO after liver transplantation but later adapted to patients with idiopathic EHPVO [8][9][10][11].…”

Surgery for Portal Hypertension in Children

“…These shunts may be either selective or nonselective [3]. Selective shunts preserve the majority of portal and mesenteric blood flow to the liver.…”

Progression of Noncirrhotic Portal Hypertension in a Pediatric Population

“…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][2][3][4][5][6][7][8] Noncirrhotic etiologies of portal hypertension are classified as prehepatic, hepatic, and posthepatic.…”

“…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.…”