[size=20px]A five-year old boy with a history of biliary atresia and liver failure after an initially successful Kasai procedure is transferred to the pediatric intensive care unit with hematemesis. He receives 20 mL/kg of lactated Ringers and one unit of pRBCs. The hemoglobin in 9 gm/dL. Flexible upper gastrointestinal endoscopy reveals bleeding from esophageal varices. Octreotide is administered.

 

The next best step in management in this child with bleeding esophageal varices is

 

A transjugular intrahepatic portosystemic shunt (TIPS).

 

B placement of a Sengstaken Blakemore tube (balloon tamponade).

 

C emergency mesocaval shunt procedure.

 

D variceal banding.

 

E variceal sclerotherapy.[/size]

E

E variceal sclerotherapy

Think again my friend

Think again my friend

A

A transjugular intrahepatic portosystemic shunt (TIPS)

Think again my friend

Think again my friend

D

D

correct

 

Variceal bleeding is categorized as:

• presinusoidal (intrahepatic or extrahepatic)
  
• sinusoidal - cirrhosis (currently the most frequent cause of portal hypertension)
  
• postsinusoidal (Budd-Chiari syndrome, veno-occlusive disease, cardiac disease)
  

While the liver is usually normal in the first category, hepatic dysfunction or insufficiency is common in the latter two.

 

Acute variceal bleeding can occur with portal hypertension. Bleeding may be massive. Gastric varices have an increased risk of bleeding compared to esophageal. The definition of portal hypertension is greater than 5 mm Hg pressure in the portal vein or a portal vein to hepatic vein gradient of greater than 10 mm Hg. In patients who do have a portal to hepatic vein gradient of greater than 12 mm Hg, one third will have gastrointestinal bleeding. The risk of bleeding is related to the cause of the portal hypertension: very low in Budd-Chiari syndrome but as high as 80% in extrahepatic portal vein obstruction and approximately 33% with cirrhosis.

 

The initial interventions include placement of large bore intravenous access, transfer to the intensive care unit, blood and blood product availability, transfusion of pRBCs and gastric lavage with saline (iced saline is not necessary). Vasoactive drugs (terlipressin, octreotide, somatostatin, vasopressin) are usually indicated. The primary diagnostic procedure is endoscopy.

 

The preferred treatment is banding of the varices. Sclerotherapy is also an option but banding is safer, more effective and leads to resolution of the varices in 90% of patients. Banding can be significantly more difficult in small children, particularly those under the age of three years, and sclerotherapy is often needed in this population.

 

If endoscopic manipulations fail, tamponade with a Linton tube for small children, or a Sengstaken-Blakemore tube for children larger than 40 kg is usually the next option. Balloon tamponade can only be left in place for 12 to 24 hours and is associated with the risks of aspiration, rupture, ulceration, airway obstruction and necrosis of the esophagus. Tamponade is successful in about 90%. Emergency shunting with TIPS is usually the next step.

 

None of these measures treats the underlying problem of portal hypertension. Rebleeding occurs in as many as 50% of patients over the ensuing two to six week interval with a mortality of 20 to 70%. The risk of death from bleeding correlates with the severity of cirrhosis (bilirubin levels are a marker).