Liver Stiffness Measurements in Patients with Noncirrhotic Portal Hypertension—The Devil Is in the Details

Abstract

Noncirrhotic portal hypertension (NCPH) is often a diagnostic challenge due to signs and symptoms of portal hypertension that overlap with cirrhosis. The etiology of NCPH is broadly classified as prehepatic, hepatic (presinusoidal and sinusoidal) and posthepatic. Some common etiologies of NCPH encountered in clinical practice include portal vein thrombosis (prehepatic) and nodular regenerative hyperplasia (hepatic). Liver histology, although considered gold standard to exclude cirrhosis in individuals with suspected NCPH, is often limited by subtle histologic features or inadequate sampling. Liver stiffness measurements (LSMs) by vibration-controlled transient elastography may provide clinically important information to distinguish NCPH from cirrhosis by revealing normal LSM in prehepatic and presinusoidal NCPH.

Letter to the Editor: Liver Stiffness in Noncirrhotic Portal Hypertension: The Devil Is in the Diagnosis

“…Previous studies have reported that hepatic causes, such as nodular regenerative hyperplasia (NRH), can have variable liver stiffness measurement (LSM) based on severity and concomitant fibrosis. (1)(2)(3) In addition to observing similar variability in LSM in patients with NRH, we also found a relationship between hepatic venous pressure gradient and LSM. However, our assumption that LSM would be normal in patients with portal vein thrombosis (PVT), a prehepatic cause of NCPH, was not observed in all.…”

“…We have read with great interest the article by Vuppalanchi et al, (1) who studied the diagnostic role of liver stiffness measurements (LSMs) in patients with noncirrhotic portal hypertension (NCPH) due to hepatic (nodular regenerative hyperplasia [NRH]) or prehepatic origin (portal vein thrombosis [PVT]). The role of LSMs in identifying a cirrhosis as the cause of the portal hypertension is well established, (2) and the evidence of PVT in any imaging technique is sufficient to attribute the cause of portal hypertension to PVT and does not require further histological or elastographical confirmation.…”

Transient Elastography (Fibroscan) in Patients with Non-cirrhotic Portal Fibrosis

“…(3) Finally, although transient elastography (TE) can be used to differentiate NCPH from cirrhosis, Vuppalanchi et al demonstrated that there was significant overlap in liver stiffness measurements (LSM) between the two diseases. (4,5) Mean LSM values of their NCPH compared with a matched cohort with cirrhosis was 13.2 ± 11.5 versus 26.6 ± 18.4 kPa. Furthermore, LSM has not been shown to have any correlation to any meaningful clinical outcomes in NCPH.…”

Portal Pressure in Noncirrhotic Portal Hypertension: To Measure or Not to Measure

“…This is likely due to other pathology such as bone marrow suppression from infections and medications resulting in thrombocytopenia in these patients. Liver stiffness measurements by vibration‐controlled transient elastography was also recently shown to be inadequate in this disease . Further research needs to be done to discover noninvasive markers of portal hypertension in NCPH.…”