Guideline sources The following summarized guidelines for the evaluation and management of idiopathic noncirrhotic portal hypertension (INCPH) are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2016). 1 Guidelines 1. Screening and diagnosis Diagnostic criteria: consider the diagnosis of INCPH in any patient with portal hypertension, particularly when there is no other cause for liver disease. B 2. Diagnostic investigations Initial evaluation: exclude cirrhosis and other causes of non-cirrhotic portal hypertension prior to diagnosing INCPH. B 3. Diagnostic procedures https://web.pathway.md/diseases/recMfBRZlb2Oa7Pgw 1/2 6/24/23, 2:28 PM Idiopathic noncirrhotic portal hypertension Pathway Liver biopsy: obtain a liver biopsy to diagnose INCPH. A 4. Medical management Management of portal hypertension: manage portal hypertension according to guidelines elaborated for patients with cirrhosis. B 5. Surgical interventions Liver transplantation: consider liver transplantation in patients with INCPH who develop liver failure or unmanageable portal hypertension-related complications. B 6. Follow-up and surveillance Monitoring for portal vein thrombosis: screen patients with INCPH at least every 6 months for the occurrence of portal vein thrombosis. B References 1. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: Vascular diseases of the liver. J Hepatol. 2016 Jan;64 1 179 202. Open 2. Hwajeong Lee, Aseeb Ur Rehman, M Isabel Fiel. Idiopathic Noncirrhotic Portal Hypertension: An Appraisal. J Pathol Transl Med. 2016 Jan;50 1 17 25. Open 3. J N L Schouten, F Nevens, B Hansen et al. Idiopathic noncirrhotic portal hypertension is associated with poor survival: results of a long-term cohort study. Aliment Pharmacol Ther. 2012 Jun;35 12 1424 33. Open 4. Oliviero Riggio, Stefania Gioia, Ilaria Pentassuglio et al. Idiopathic noncirrhotic portal hypertension: current perspectives. Hepat Med. 2016 Jul 27;8 81 8. Open 5. Italian Association for the Study of the Liver AISF , Italian Association for the Study of the Liver AISF. AISF position paper on liver disease and pregnancy. Dig Liver Dis. 2016 Feb;48 2 120 37. Open https://web.pathway.md/diseases/recMfBRZlb2Oa7Pgw 2/2