Advances in the management of childhood portal hypertension

Abstract

Portal hypertension is one of the most serious complications of childhood liver disease, and variceal bleeding is the most feared complication. Most portal hypertension results from cirrhosis but extra hepatic portal vein obstruction is the single commonest cause. Upper gastrointestinal endoscopy endoscopy remains necessary to diagnose gastro-esophageal varices. Families of children with portal hypertension should be provided with written instructions in case of gastrointestinal bleeding. Children with large varices should be considered for primary prophylaxis on a case-by-case basis. The preferred method is variceal band ligation. Children with acute bleeding should be admitted to hospital and treated with antibiotics and pharmacotherapy before urgent therapeutic endoscopy. All children who have bled should then receive secondary prophylaxis. The preferred method is variceal band ligation and as yet there is little evidence to support the use of β-blockers. Children with extrahepatic portal vein obstruction should be assessed for suitability of mesoportal bypass.

Keywords:

• β blockers
• children
• endoscopic variceal ligation
• esophageal varices
• hematemesis
• meso-portal
• portal hypertension
• portosystemic shunts
• TIPS

Financial & competing interests disclosure

P McKiernan has acted as a consultant for Sobi AB. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Normal portal pressure ranges from 5 to 10 mmHg. Complications such as esophageal varices and ascites develop when portal pressure rises more than 12 mmHg.

• Hepatic Venous Pressure Gradient is the gradient between pressures in the portal vein and the intra-abdominal part of inferior vena cava and is an indirect measure of portal pressure unless there is presinusoidal disease.

• Splenomegaly and hypersplenism may be the first presentation in children with Portal Hypertension (PH). Children with cirrhosis and splenomegaly have a higher risk of developing varices.

• Esophago-gastroduodenoscopy is the gold standard for the diagnosis and treatment of esophageal and gastric varices in children.

• Endoscopic band ligation is safe and effective in the primary and secondary prophylaxis of children with esophageal varices.

• Non-selective β-blockers are the preferred method for primary prophylaxis in adults with PH. Non-selective β-blockers reduce portal pressure by decreasing cardiac output and inducing splanchnic vasoconstriction. There are few studies on the use of non-selective β-blockers in children. Its use in children should be titrated by hepatic venous pressure gradient response.

• Acute variceal bleeding is the most serious complication of PH. Initial management is stabilizing the child and judicious resuscitation. The use of a nasogastric tube should be considered in all cases. Pharmacotherapy using Octreotide or Terlipressin and antibiotics should be started on admission and continued for 2–5 days. Therapeutic endoscopy should be carried out as soon as possible, and certainly within 24 h, once the patient is hemodynamically stable.

• Transjugular intrahepatic portosystemic shunting is an effective second-line treatment for acute variceal bleeding and for secondary prophylaxis where endoscopic treatment has failed or is not tolerated.

• Mesoportal bypass is a physiological shunt used in the treatment of PH secondary to extrahepatic portal venous obstruction. It is created by a natural vascular graft placed between the superior mesenteric vein and the umbilical branch of the portal vein, hence restoring physiological portal blood flow.

• Hepatopulmonary syndrome is defined as the triad of an arterial oxygenation defect, intrapulmonary vasodilation and liver disease. The diagnosis is best confirmed by microbubble echocardiography. There is no established medical treatment and most affected children will require liver transplantation.