Treatment

The most common, clinically significant complication of portal hypertension is gastrointestinal bleeding from esophageal varices. In the acute setting, hemorrhage is managed with intensive care monitoring. Volume resuscitation should be rapidly instituted with crystalloid and red blood cells as necessary. Fresh frozen plasma, platelets and vitamin K may be indicated in the presence of coagulopathy. A nasogastric (or orogastric) tube should be placed for lavage and monitoring. Intubation and sedation may be required for airway protection and to minimize the agitation that may increase variceal pressure.

Figure 4.1 The Sengstaken-Blakemore tube

In patients with ongoing bleeding, pharmacologic therapies are warranted. Medical treatment of portal hypertension is aimed at relieving the pressure in the portal system. β-blockade, if tolerated, reduces cardiac output and therefore portal venous pressure. Vasopressin decreases portal blood flow by increasing splanchnic vascular tone. Vasopressin is initially bolused at 0,33 U/kg over 20 minutes and is then infused continuously at the same dose on an hourly basis or as an infusion of 0,2 U/1,73 m²/min. Vasopressin has a half-life of approximately 30 minutes. Side effects of vasopressin use are secondary to cardiac or visceral vasoconstriction and may be ameliorated by transdermal nitroglycerin administration. Infusion of octreotide, a long-acting somatostatin analog, has also been shown to decrease splanchnic blood flow. Endoscopic variceal banding or sclerotherapy is used in cases where hemorrhage does not resolve with supportive care. Once the patient has stabilized, endoscopy with sclerotherapy or banding is employed to prevent repeat episodes of hemorrhage. In bleeding that is refractory to both pharmacologic and endoscopic interventions, placement of a Sengstaken-Blakemore tube may be warranted (Fig. 4.1). Clinicians should be cognizant of the significant rates of complications with its use, including recurrent bleeding, pulmonary aspiration and gastroesophageal perforation.

Since there is a normally functioning liver, gastrointestinal hemorrhage in children with extrahepatic portal hypertension tends to be less severe than bleeding that occurs in children with IHPH. In addition to coagulation abnormalities, many patients with IHPH also have significant malnutrition that contributes to the greater morbidity and mortality of gastrointestinal bleeds in this group.

Surgical treatment of portal hypertension can be either direct, which involves ligation of the varices themselves (esophagogastric devascularization with or without splenic artery ligation) or indirect, in which the portal venous system is decompressed via a portosystemic shunt.

Surgical options for shunt procedures are based on the primary etiology of portal hypertension (Table 4.7).

Table 4.7

Portal hypertension surgical options (by Peter Mattei, 2011)

Nonselective portosystemic shunts divert the majority of portal blood to the caval system, which may result in a higher incidence of hepatic encephalopathy. Examples include portocaval, mesocaval and central splenorenal shunts. Selective portosystemic shunts divert a portion of portal blood into the systemic circulation, with the distal splenorenal (Warren) shunt being the most common. Recently, selected children with EHPH due to portal vein thrombosis have been successfully treated by surgical creation of a mesenterico-left portal venous bypass (Rex shunt). Recurrent bleeding, shunt thrombosis and hepatic encephalopathy are the most common complications of surgical shunting.

In many centers, transjugular intrahepatic portosystemic shunt (TIPS) therapy has become first line treatment for refractory bleeding or hypersplenism. Complications of TIPS treatment include hepatic encephalopathy and shunt thrombosis or stenosis. Care must be taken that the shunt length does not extend into the main portal branches which may be used in a future liver transplant.

Treatment of intrahepatic portal hypertension focuses on the primary liver disease. In biliary atresia, surgery to decompress the biliary tract is ideally performed within the first 3 months of life. With the Kasai procedure, the atretic segments of the extrahepatic bile ducts are excised and a Roux loop is anastomosed to the porta hepatis. If performed early, before significant liver injury and cirrhosis, the Kasai procedure can delay liver failure and the need for transplantation in as many as two-thirds of patients. For advanced cirrhosis and other intrahepatic sources of portal hypertension, liver transplantation represents definitive treatment. Ideally, surgical interventions prior to transplant should neither incur significant risk to the patient, nor should they interfere with the ability to perform a liver transplant in the future.