Esophageal varices are dilated submucosal distal esophageal veins connecting the portal and systemic circulations. This happens due to portal hypertension (most commonly a result of cirrhosis), resistance to portal blood flow, and increased portal venous blood inflow. The most common fatal complication of cirrhosis is variceal rupture; the severity of liver disease correlates with the presence of varices and risk of bleeding.
Causes of portal hypertension:
Less frequent causes are schistosomiasis, massive fatty change, diseases affecting portal microcirculation as nodular regenerative hyperplasia and diffuse fibrosing granulomatous disease as sarcoidosis.
Portal hypertension is common in chronic liver disease (CLD) in children.
Portal hypertension causes portocaval anastomosis to develop to decompress portal circulation. This leads to a congested submucosal venous plexus with tortuous dilated veins in the distal esophagus. Variceal rupture results in hemorrhage.
Pathophysiology of portal hypertension:
The first indication of varices is often the presence of a gastrointestinal (GI) bleeding episode: hematemesis, hematochezia, and/or melena. Occult bleeding (anemia) is uncommon.
Initial Tests (lab, imaging)
Hepatic encephalopathy and infection often complicate variceal bleeding.
Variceal band ligation is preferred to sclerotherapy for bleeding varices and for nonbleeding medium-to-large varices to decrease bleeding risk. Ligation has lower rates of rebleeding, fewer complications, more rapid cessation of bleeding and a higher rate of variceal eradication.
Repeat ligation/sclerosant for rebleeding.
If endoscopic treatment fails, consider self-expanding esophageal metal stents or peroral placement of Sengstaken-Blakemore-type tube up to 24 hours to stabilize the patient for TIPS.
As many as two-thirds of patients with variceal bleeding develop an infection, most commonly spontaneous bacterial peritonitis, UTI, or pneumonia. Antibiotic prophylaxis with oral norfloxacin 400 mg or IV ceftriaxone, 1 g q24h for up to a week, is indicated.
Prevent recurrence of acute bleeding:
Refer for endoscopy, liver transplant, and interventional radiology for TIPS.
Pneumococcal and hepatitis A/B (HAV/HBV) vaccine need to be considered.
Endoscopic variceal ligation should be repeated every 1 to 4 weeks until varices are eradicated. If TIPS is done, repeat endoscopy to assess rebleeding. Endoscopic screening should be done in patients with known cirrhosis every two to three years and yearly in patients with decompensated cirrhosis. Patients with liver stiffness less than 20 kPa and with platelets greater than 150,000 can avoid endoscopic screening and may follow up by annual TE and platelet count.
The management of esophageal varices is with an interprofessional team that consists of a gastroenterologist, internist, surgeon, invasive radiologist, and an intensivist. The treatment selected depends on the severity of the disease and patient status. Unless the primary cause of portal hypertension is controlled, recurrence is common with all treatments. The prognosis for patients with esophageal varices is guarded. Multiorgan failure, complications from procedures and infections often lead to premature death.
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