Summer school: pediatric hepatology
Portal hypertension in children

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Summary

The main causes of intrahepatic portal hypertension in children are cirrhosis and congenital hepatic fibrosis. Non cirrhotic portal hypertension in children is mostly due to extrahepatic portal vein obstruction. In half of cases, no underlying disorder is found. The meso-Rex bypass is the preferred treatment, when it is possible. The closest to the portal vein the obstruction, the highest the risk of esophagogastric varices.

Section snippets

Keypoints

  • The main causes of intrahepatic portal hypertension are cirrhosis and congenital hepatic fibrosis.

  • Non cirrhotic portal hypertension in children is mostly due to extrahepatic portal vein obstruction. In half of cases, no cause is found.

  • The meso-Rex bypass is the preferred treatment for extrahepatic portal vein obstruction, when it is possible.

  • The closest to the portal vein the obstruction, the highest the risk of esophagogastric varices.

Portal hypertension is defined as a pathological increase

Clinical symptoms

Typical presentation symptoms are episodes of gastrointestinal haemorrhage, incidental diagnosis of splenomegaly or thrombopenia (hypersplenism), while ascites, jaundice, and hepatic encephalopathy are uncommon at first diagnosis. When the liver function is well preserved, PH also may have odd presentations, such as glomerulonephritis or hypoxemia due to hepatopulmonary syndrome.

The main cause of non cirrhotic PH in children is extrahepatic portal vein obstruction (EHPVO) [2]. The thrombosis

Diagnosis

Doppler ultrasonography (US) is the most useful diagnostic tool, with endoscopy as the second important investigation for staging the disease, and treating the complications. Doppler US helps for the differential diagnosis: the aspect of the hepatic parenchyma, the regular, or not, aspect of the liver capsule, the patency of portal vein or its replacement by a cavernoma, the flow pattern of hepatic veins and hepatic artery, the presence of splenomegaly or liver atrophy, are important elements

Medical and endoscopic treatment

The closer the obstruction is from the portal vein, the higher the risk for esophagogastric varices. The endoscopy shows varices at diagnosis in almost 85–95% of patients with EHPVO. Gastric varices are more common in EHPVO than in cirrhosis. The gastropathy developing after variceal obliteration is usually transitory and non-progressive in EHPVO. The role of prophylactic or pre-emptive variceal banding, as performed in adults, is still controversial in children, due to the absence of

Surgical treatment

The only definitive treatment of portal hypertension is the treatment of its cause, that is liver transplantation for cirrhosis, or meso-Rex bypass for EHPVO. In this latter case, the conventional procedure for decreasing portal pressure, a porto-systemic shunt, puts the patient at risk of long-term complications, such as hyperammoniemia and hepato-pulmonary syndrome. The meso-Rex bypass on the contrary restores the liver physiology and function [3].

Management of PH due to EHPVO depends on the

Disclosure of interest

The authors declare that they have no conflicts of interest concerning this article.

References (5)

  • B. Shneider et al.

    Portal hypertension in children: Expert pediatric opinion on the report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension

    Pediatr Transplantation

    (2006)
  • B. Weiss et al.

    Etiology and long-term outcome of extrahepatic portal vein obstruction in children

    World J Gastroenterol

    (2010)
There are more references available in the full text version of this article.

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