Non‐cirrhotic portal fibrosis: Current concepts and management

Abstract

Non-cirrhotic portal hypertension (NCPH) comprises diseases having an increase in portal pressure (PP) due to intraheptic or prehepatic lesions, in the absence of cirrhosis. The lesions are generally vascular, either in the portal vein, its branches or in the perisinusoidal area. Because the wedged hepatic venous pressure is near normal, measurement of intravariceal or intrasplenic pressure is needed to assess PP. The majority of diseases included in the category of NCPH are well-characterized disease entities where portal hypertension (PHT) is a late manifestation and, hence, these are not discussed. Two diseases that present only with features of PHT and are common in developing countries are non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO). Non-cirrhotic portal fibrosis is a syndrome of obscure etiology, characterized by 'obliterative portovenopathy' leading to PHT, massive splenomegaly and well-tolerated episodes of variceal bleeding in young adults from low socioeconomic backgrounds, having near normal hepatic functions. In some parts of the world, NCPF is called idiopathic portal hypertension (IPH) or 'hepatoportal sclerosis'. Because 85-95% of patients with NCPF and EHPVO present with variceal bleeding, treatment involves management with endoscopic sclerotherapy (EST) or variceal ligation (EVL). These therapies are effective in approximately 90-95% of patients. Gastric varices are another common cause of upper gastrointestinal bleeding in these patients and these can be managed with cyanoacrylate glue injection or surgery. Other indications for surgery include failure of EST/EVL, and symptomatic hypersplenism. The prognosis of patients with NCPF is good and 5 years survival in patients in whom variceal bleeding can be controlled has been reported to be approximately 95-100%.

Noncirrhotic Portal Hypertension

“…Shunt occlusion, overt chronic portosystemic encephalopathy, and rebleeding after elective shunt surgery are seen in about 10% of patients. 62,63 Endoscopic therapy is preferred in these young patients where lifelong drug compliance is difficult to maintain. Also, therapy with beta-blockers would be difficult to monitor since the HVPG in these patients is normal.…”

“…The long-term outcome of shunt surgery is also favorable and 5-year survival of 88% has been reported. 63 …”

Novel rat model of repetitive portal venous embolization mimicking human non-cirrhotic idiopathic portal hypertension

“…In the absence of cirrhosis, other synonyms are non-cirrhotic portal fibrosis (NCPF), or hepatoportal sclerosis and obliterative venopathy in Eastern countries [1]. Importantly, this condition occurs in the absence of other well characterized causes of portal hypertension such as chronic liver disease and splanchnic venous thrombosis [2][3][4][5]. The prognosis of this disease is considered as benign with a 5 year survival of almost 100%, but long term outcomes are still unknown [4,6,7].…”

“…Since the prevalence of NCIPH in the Western world is low, recently published literature is rare [8]. Various conditions such as chronic infectious diseases, toxin or drug exposure, genetic and immunological disorders have been determined as potential causes of NCIPH [3,6].…”

“…One reason for the absence of experimental models is that the underlying pathomechanisms of NCIPH have been poorly understood so far [3,9,10]. In liver samples of patients with NCIPH, vascular lesions in the portal venules have been observed [3].…”