Introduction

The management of portal hypertension in children is becoming increasingly more focused on correcting the underlying cause of the condition and curing the underlying problem whenever possible. The pediatric surgeon, when confronted with a child who has portal hypertension, must be able to properly advise the family of the child on the appropriate course of therapy. The pediatric surgeon may not infrequently be called in consult to a child with hematemesis or the passage of frank blood or melena in the stool. Alternately, the surgeon may be called upon to see a child with a large spleen, distended abdominal veins or ascites. It is important that the surgeon be familiar with the symptoms and the physical signs of portal hypertension, be able to order appropriate ancillary testing and ultimately participate in a meaningful manner in the management and disposition of the child with portal hypertension.

The most critical aspects of portal hypertension are

• The early recognition of the symptoms of portal hypertension and making the correct diagnosis when confronted with a clinical scenario that is compatible with portal hypertension.
• Be able to advise the consulting services, usually the intensive care unit and the gastroenterology service, on the appropriate course of management in the critical phase when a child first presents to an emergency department or is admitted to a critical care unit.
• Be able to advise the consulting services on the appropriate role of surgery in the management of the cause of the portal hypertension.
• Understand the pathophysiology of portal hypertension and make the appropriate decision in regard to patient care - whether it is to operate on the patient or make a referral.
• Learn the physiological consequences of portal vein thrombosis and the natural history if it goes uncorrected.

The most challenging areas are the early recognition of the signs and symptoms of portal hypertension and to have the ability to discriminate among the causes of portal hypertension that are amenable to surgical correction, those that are best managed medically and those that may require referral to a transplant center for those children whose portal hypertension is a manifestation of end stage liver disease. Most children with portal hypertension should be referred to a center with the multidisciplinary capabilities to adequately deliver the complex care that these children often need.

The operative strategies involved in the correction of portal vein thrombosis are likely to change with regard to the venous grafts that are often necessary for the reconstruction of portal venous blood toward the liver. Work is being done with tissue engineering that may obviate the need for the procurement of autologous vein grafts.

Content in this topic is referenced in SCORE Portal Hypertension overview