From the Guidelines
Esophageal varices without liver cirrhosis are often caused by non-cirrhotic portal hypertension (NCPH), which can be due to various underlying conditions such as portal vein thrombosis, schistosomiasis, or splenic vein thrombosis, as well as primary sclerosing cholangitis (PSC) with pre-sinusoidal block at the level of the portal tract.
Causes of Esophageal Varices without Liver Cirrhosis
- Portal vein thrombosis
- Schistosomiasis
- Splenic vein thrombosis
- Primary sclerosing cholangitis (PSC) with pre-sinusoidal block at the level of the portal tract, where ductular proliferation and pronounced portal fibrosis may have a profound impact on hepatic vascular resistance 1
- Nodular regenerative hyperplasia and obliterative portal venopathy, which can occur in the absence of histological cirrhosis 1
Management of Esophageal Varices without Liver Cirrhosis
- Investigation for underlying causes
- Treating the primary cause
- Preventing variceal bleeding
- For primary prophylaxis in patients with medium to large varices, non-selective beta-blockers like propranolol or nadolol are recommended 1
- Endoscopic variceal ligation (EVL) is an alternative for those who cannot tolerate beta-blockers
- Acute bleeding requires immediate resuscitation, vasoactive drugs like octreotide, and urgent endoscopic therapy
- Secondary prophylaxis combines beta-blockers with EVL every 2-4 weeks until varices are eradicated
- In refractory cases, transjugular intrahepatic portosystemic shunt (TIPS) or surgical shunts may be considered
- Regular endoscopic surveillance is essential, typically every 6-12 months
Important Considerations
- Patients with PSC may develop varices even in the absence of established cirrhosis, and the possibility of non-cirrhotic portal hypertension should be considered in all PBC patients with a GI bleed 1
- The management of portal hypertension-related complications in PSC should follow AASLD and Baveno/EASL guidelines, which are largely based on evidence derived from studies including mostly patients with alcohol-related and viral aetiologies 1
From the Research
Causes of Esophageal Varices without Liver Cirrhosis
- Portal hypertension (PH) is a common cause of esophageal varices, which can occur due to various conditions, including non-cirrhotic portal hypertension 2
- Non-cirrhotic portal hypertension can be caused by porto-sinusoidal vascular disease (PSD) or other conditions, leading to the development of esophageal varices 2
- Esophageal varices can also occur in patients with stomal variceal bleeding due to PH, which can be caused by cirrhotic or non-cirrhotic portal hypertension 2
- The exact causes of esophageal varices without liver cirrhosis are not fully understood and may involve various factors, including genetic and environmental factors
Risk Factors for Esophageal Varices without Liver Cirrhosis
- Presence of portal hypertension (PH) is a significant risk factor for the development of esophageal varices, regardless of the underlying cause 3, 4
- Patients with non-cirrhotic portal hypertension, such as those with PSD, are at risk of developing esophageal varices 2
- Patients with a history of variceal bleeding or other complications of portal hypertension are also at increased risk of developing esophageal varices 5, 6
Diagnosis and Treatment of Esophageal Varices without Liver Cirrhosis
- Diagnosis of esophageal varices typically involves endoscopy and imaging studies to assess the presence and severity of varices 3, 4
- Treatment of esophageal varices without liver cirrhosis may involve a combination of medical and endoscopic therapies, including beta-blockers, endoscopic variceal banding, and sclerotherapy 5, 6
- Transjugular intrahepatic portosystemic shunt (TIPS) implantation may also be considered in certain cases, particularly in patients with refractory variceal bleeding 4, 2