From the Guidelines
Esophageal varices in patients without liver cirrhosis are typically caused by non-cirrhotic portal hypertension, which can occur due to various etiologies, including primary sclerosing cholangitis (PSC) and Fontan-associated liver disease (FALD). The management of esophageal varices in these patients involves both acute treatment and long-term prevention strategies.
Causes of Esophageal Varices
- Non-cirrhotic portal fibrosis
- Extrahepatic portal vein obstruction
- Primary sclerosing cholangitis (PSC)
- Fontan-associated liver disease (FALD) According to the study by 1, progressive hepatic fibrosis and cirrhosis may result in portal hypertension in PSC, and clinically significant portal hypertension (CSPH) is defined by either endoscopic finding of gastro-oesophageal varices (GEVs), invasive measurement of hepatic venous pressure gradient (HVPG) >10 mmHg, or pathognomonic imaging findings including portosystemic collaterals and ascites.
Management of Esophageal Varices
For acute variceal bleeding, immediate resuscitation with two large-bore IV lines, fluid resuscitation, blood transfusion to maintain hemoglobin around 7-8 g/dL, and correction of coagulopathy are essential, as recommended by 1. Pharmacotherapy includes vasoactive drugs like octreotide (50-100 mcg IV bolus followed by 25-50 mcg/hour infusion) or terlipressin (2 mg IV every 4 hours), which should be continued for 3-5 days. Endoscopic therapy, preferably band ligation, should be performed within 12 hours, as suggested by 1. Antibiotic prophylaxis with ceftriaxone 1g IV daily for 5-7 days is recommended even without cirrhosis. For secondary prevention, combination therapy with non-selective beta-blockers (propranolol starting at 20 mg twice daily or nadolol 20-40 mg daily, titrated to heart rate of 55-60 bpm) and endoscopic band ligation every 2-4 weeks until varices are eradicated is recommended, as stated in 1. However, the study by 1 suggests that the impact of non-selective beta-blockers (NSBBs) on portal pressure has yet to be addressed in Fontan patients, and NSBBs could be less effective or ineffective in the FALD model of portal hypertension.
Investigation and Treatment
The underlying cause of portal hypertension should be investigated with imaging studies (Doppler ultrasound, CT, or MRI) and potential thrombophilia workup, as recommended by 1. Unlike cirrhotic patients, those with non-cirrhotic portal hypertension often have preserved liver function but may still develop severe bleeding complications requiring similar management approaches. In summary, the management of esophageal varices in patients without liver cirrhosis involves a multidisciplinary approach, including acute treatment, long-term prevention strategies, and investigation of the underlying cause of portal hypertension, as recommended by 1 and 1.
From the Research
Causes of Esophageal Varices
- Esophageal varices are typically associated with liver cirrhosis and portal hypertension 2, 3, 4, 5, 6
- However, the provided studies do not directly address the causes of esophageal varices in patients without liver cirrhosis
- Portal hypertension is a key factor in the development of esophageal varices, but the studies focus on patients with cirrhosis and do not explore other potential causes of portal hypertension in non-cirrhotic patients 2, 3, 4, 5, 6
Portal Hypertension
- Portal hypertension can lead to the formation of esophageal varices, regardless of the underlying cause 2, 3, 4, 5, 6
- The studies discuss the treatment and management of esophageal varices in patients with cirrhosis, but do not provide information on the causes of esophageal varices in patients without cirrhosis 2, 3, 4, 5, 6