Portal hypertension in children.

Portal hypertension is an uncommon but serious complication in children. The etiology may be intrahepatic or from an extrahepatic vascular occlusion. The pathophysiology is unknown, but the development of increased vascular resistance and portal flow are believed to be the primary changes. Increased portal flow is created by a decrease in systemic vascular resistance regionalized to the splanchnic vascular bed. Abnormally reduced response to vasoactive substances may be responsible. Advances in ultrasonography and endoscopy may improve our ability to evaluate portal hypertensive vascular changes. However, pharmacologic management is still limited to controlling the hemodynamic disturbances after they have occurred. Nonsurgical shunt management is increasingly being used in children, limiting the need for surgical shunt placement. Liver transplantation is now a viable option for managing end-stage disease. Future management depends on understanding the role of vasoactive substances controlling portal flow velocity, subsequent development of targeted pharmacologic therapy, and application of nonsurgical shunt technology in children. Transjugular intrahepatic portosystemic shunt placement is the next technologic advance moving from the adult into the pediatric realm.