Idiopathic noncirrhotic portal hypertension is the clinical diagnosis of exclusion featuring portal hypertension without hepatic cirrhosis, vascular obstruction, schistosomiasis, or a variety of other chronic liver diseases.
Prior terms for this entity include noncirrhotic portal fibrosis, idiopathic portal hypertension, idiopathic portal fibrosis, and Banti syndrome 5,6. Idiopathic noncirrhotic portal hypertension was proposed in 2011 as the uniform term 5.
As a clinical diagnosis, idiopathic noncirrhotic portal hypertension encompasses a spectrum of histopathologic entities: nodular regenerative hyperplasia, incomplete septal cirrhosis, obliterative portal venopathy, and less commonly partial nodular transformation. The term hepatoportal sclerosis has also been used in a manner overlapping with obliterative portal venopathy. However, these morphologic alterations can be seen without portal hypertension. The Vascular Liver Disease Interest Group proposed in 2019 the term porto-sinusoidal vascular disease to encompass all those patients with histologic features historically associated idiopathic noncirrhotic portal hypertension, regardless of whether they actually have portal hypertension 6.
The condition is rare.
Many systemic disorders have been described in association 5:
- immunological disorders (eg, systemic sclerosis, systemic lupus erythematosus, primary hypogammaglobulinemia)
- chronic infections (eg, HIV, bacterial infections of the gut)
- medications and toxins (eg, azathioprine, 6-thioguanine, arsenic)
- genetic disorders (eg, Turner syndrome, Adams-Oliver syndrome)
The diagnosis of idiopathic noncirrhotic portal hypertension requires at least one clinical sign of portal hypertension 5:
- esophageal varices
- ascites (nonmalignant)
- increased hepatic venous pressure gradient
- portovenous collaterals
The diagnosis of porto-sinusoidal vascular disease requires a noncirrhotic liver biopsy with either of the following 6:
- one clinical sign specific for portal hypertension,
- one histological lesion specific for porto-sinusoidal vascular disease, or
- one clinical sign not specific for portal hypertension and one histologic lesion not specific for porto-sinusoidal vascular disease
The clinical signs of portal hypertension are as follows 6:
- gastric, esophageal, or ectopic varices
- portal hypertensive bleeding
- porto-systemic collaterals at imaging
- not specific
- platelet count <150,000 per μL
- spleen size ≥13 cm in largest axis
In idiopathic noncirrhotic portal hypertension, other chronic liver conditions must be excluded by a combination of liver biopsy, laboratory tests, and imaging 5,6:
- cirrhosis of any etiology
- chronic viral hepatitis (hepatitis B or hepatitis C)
- nonalcoholic steatohepatitis
- alcoholic steatohepatitis
- autoimmune hepatitis
- hereditary hemochromatosis
- Wilson disease
- primary biliary cholangitis
- congenital hepatic fibrosis
- obstruction of the hepatic or portal veins (eg, Budd-Chiari syndrome, portal vein thrombosis)
Similarly, the diagnosis of porto-sinusoidal vascular disease excludes a number of conditions 6:
- bone marrow transplantation (eg, sinusoidal obstruction syndrome)
- Budd-Chiari syndrome or hepatic venous outflow obstruction
- schistosomiasis on liver biopsy (positive serology alone does not exclude the diagnosis)
- cardiac failure or Fontan surgery
- Abernethy syndrome
- hereditary hemorrhagic telangiectasia
- chronic cholestatic diseases
- tumor infiltration of the liver
- congenital hepatic fibrosis
The pathogenesis is unclear but the porto-sinusoidal vasculature is typically implicated 6. The entity is often considered among the presinusoidal causes of portal hypertension due to the prevalence of the specific finding of obliterative portal venopathy (wall thickening, luminal occlusion, and vanishing of the portal veins), which results in increased portal system resistance 5. The other two specific histologic findings are nodular regenerative hyperplasia and incomplete septal fibrosis/cirrhosis 6.
Nonspecific histological lesions in porto-sinusoidal vascular disease include portal tract abnormalities (multiplication, arterial dilation, periportal vascular channels, aberrant vessels), architectural disturbance (irregular distribution of portal tracts and central veins), non-zonal sinusoidal dilation, and mild perisinusoidal fibrosis 6.
A liver biopsy is required to rule out cirrhosis and some causes of noncirrhotic portal hypertension, as well as identify the histologic changes associated with porto-sinusoidal vascular disease. Liver biopsy is usually accomplished with image-guided percutaneous or transjugular approaches. A core at least 20 mm long is required to exclude cirrhosis 6.
Several findings support the presence of portal hypertension:
- esophageal and/or gastric varices or other portosystemic collateral vessels (most specific)
- splenomegaly 5
- ascites 5
- elevated hepatic venous pressure gradient (>5 mm Hg, measured on fluoroscopy-guided hepatic vein catheterization)
The appearance of the liver in idiopathic noncirrhotic portal hypertension / porto-sinusoidal vascular disease is nonspecific. The liver surface is typically smooth but it is sometimes nodular due to nodular regenerative hyperplasia, mimicking cirrhosis 5,6. The combination of segment 4 atrophy and segment 1 hypertrophy seen in cirrhosis is uncommon in noncirrhotic portal hypertension 6. Altered parenchymal perfusion (increased arterial perfusion, diminished subcapsular portal perfusion) is occasionally seen 7,8.
Patent large hepatic and portal veins have been cited as a requirement for diagnosis 5, but intrahepatic and extrahepatic portal vein abnormalities are common in obliterative portal venopathy, including thrombosis, diminished caliber, lack of visibility, and wall thickening 7,8. The hepatic artery may be enlarged 6.
Treatment and prognosis
Thought to have a more favorable outcome than cirrhotic portal hypertension, but treatment strategies currently attempt to control the varices rather than treat the portal hypertension.
- 1. Nakanuma Y, Tsuneyama K, Ohbu M et-al. Pathology and pathogenesis of idiopathic portal hypertension with an emphasis on the liver. Pathol. Res. Pract. 2001;197 (2): 65-76. Pubmed citation
- 2. Khanna R, Sarin SK. Non-cirrhotic portal hypertension - diagnosis and management. J. Hepatol. 2014;60 (2): 421-41. doi:10.1016/j.jhep.2013.08.013 - Pubmed citation
- 3. Cordeau MP, Prosmanne O, Robillard P. Case of the day. Noncirrhotic idiopathic portal hypertension (Banti syndrome). Radiographics. 1990;10 (1): 114-6. doi:10.1148/radiographics.10.1.2296683 - Pubmed citation
- 4. Jha P, Poder L, Wang ZJ et-al. Radiologic mimics of cirrhosis. AJR Am J Roentgenol. 2010;194 (4): 993-9. doi:10.2214/AJR.09.3409 - Pubmed citation
- 5. Schouten JN, Garcia-Pagan JC, Valla DC, Janssen HL. Idiopathic noncirrhotic portal hypertension. (2011) Hepatology (Baltimore, Md.). 54 (3): 1071-81. doi:10.1002/hep.24422 - Pubmed
- 6. De Gottardi A, Rautou PE, Schouten J, Rubbia-Brandt L, Leebeek F, Trebicka J, Murad SD, Vilgrain V, Hernandez-Gea V, Nery F, Plessier A, Berzigotti A, Bioulac-Sage P, Primignani M, Semela D, Elkrief L, Bedossa P, Valla D, Garcia-Pagan JC. Porto-sinusoidal vascular disease: proposal and description of a novel entity. (2019) The lancet. Gastroenterology & hepatology. 4 (5): 399-411. doi:10.1016/S2468-1253(19)30047-0 - Pubmed
- 7. Glatard AS, Hillaire S, d'Assignies G, Cazals-Hatem D, Plessier A, Valla DC, Vilgrain V. Obliterative portal venopathy: findings at CT imaging. (2012) Radiology. 263 (3): 741-50. doi:10.1148/radiol.12111785 - Pubmed
- 8. Arora A, Sarin SK. Multimodality imaging of obliterative portal venopathy: what every radiologist should know. (2015) The British journal of radiology. 88 (1046): 20140653. doi:10.1259/bjr.20140653 - Pubmed