Portal Hypertension in Children

Etiology and Classification

Portal vein thrombosis is the most common cause of portal hypertension in children, accounting for approximately 80% of cases, with identifiable risk factors present in two-thirds of patients, according to the American Association for the Study of Liver Diseases 1
Intrahepatic causes of portal hypertension include biliary atresia, congenital hepatic fibrosis, and idiopathic non-cirrhotic portal hypertension, as stated by the American Association for the Study of Liver Diseases 1
Posthepatic causes of portal hypertension include Budd-Chiari syndrome and inferior vena cava obstruction, as reported by the American Association for the Study of Liver Diseases 1, 3

Hepatopulmonary syndrome occurs in 4-29% of children with chronic liver disease, characterized by intrapulmonary vascular dilatations causing hypoxia, according to the American Association for the Study of Liver Diseases 1, 4
Portopulmonary hypertension is a rare but devastating complication with mean pulmonary artery pressure >25 mmHg, requiring echocardiographic screening and right heart catheterization for diagnosis, as stated by the American Heart Association 5, 6
Portal biliopathy is a complication of portal hypertension, presenting with jaundice or cholestasis, as reported by the American Association for the Study of Liver Diseases 2

Doppler ultrasound is the first-line investigation to assess portal vein patency, direction of flow, presence of collaterals, and splenomegaly, according to the American Institute of Ultrasound in Medicine 2
Pulse oximetry is recommended for regular screening of hepatopulmonary syndrome in all children with portosystemic shunting, as stated by the American Thoracic Society 1, 4
Transthoracic echocardiography is the best non-invasive screening tool for portopulmonary hypertension, with pulmonary artery systolic pressure >40 mmHg warranting further evaluation, according to the American Heart Association 5, 4

The meso-Rex bypass is the optimal surgical treatment for children with extrahepatic portal vein obstruction and patent intrahepatic left portal vein, as stated by the American Pediatric Surgical Association 1, 3, 2
Endoscopic variceal band ligation is the optimal therapy for preventing rebleeding and should be combined with non-selective beta-blockers, according to the American Society for Gastrointestinal Endoscopy 2
Liver transplantation should be considered for children with cardiopulmonary complications of portal hypertension or progressive liver failure, even in non-cirrhotic portal hypertension, as stated by the American Association for the Study of Liver Diseases 1, 3, 7

Children with non-cirrhotic portal hypertension require regular screening for portal vein thrombosis and cardiopulmonary complications, according to the American Association for the Study of Liver Diseases 2
Pulse oximetry screening is recommended at each clinic visit to detect early hepatopulmonary syndrome, as stated by the American Thoracic Society 1, 4
Echocardiography is recommended for periodic screening for portopulmonary hypertension, particularly in patients with progressive symptoms, according to the American Heart Association 5, 6