Intrahepatic Portal Venopathy and Related Disorders of the Liver

Abstract

Intrahepatic portal venopathy leads to various entities that are important causes of portal hypertension. Noncirrhotic portal fibrosis (NCPF) occurs in the Indian subcontinent, whereas idiopathic portal hypertension (IPH) occurs in Japan although the pathogenesis and presentation of both are similar. NCPF presents mainly with upper gastrointestinal bleeding; IPH presents with massive splenomegaly. The liver functions are preserved. Wedged hepatic venous pressure is normal, but portal venous pressure is high indicating a presinusoidal block. Patients are best managed with endoscopic therapy or surgery, with better results than in patients with cirrhosis. Nodular regenerative hyperplasia is a histological diagnosis characterized by development of nodules in the liver due to uneven perfusion of the portal venous blood. These patients may develop portal hypertension and if they bleed would require treatment as in NCPF/IPH. Schistosomiasis produces portal hypertension by the development of fibrous tissue around the portal veins as a response to schistosome eggs. Gratifying results have been reported with praziquantel therapy. Rarely sarcoidosis and chronic biliary obstruction may also produce portal venopathy.

Idiopathic portal hypertension: Natural history and long-term outcome

“…Finally, a total of 69 well-characterized patients with IPH were included. Mean duration of follow-up from diagnosis was 6.7 6 4.6 years (median, 6; range, [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18].…”

“…In those with symptoms, confirming previous observations, VB was the most common initial manifestation. 1,5,13 The lack of specific symptoms or laboratory abnormalities may defer the diagnosis of IPH. Indeed, 7% of our patients were previously misdiagnosed with cryptogenic cirrhosis and the diagnosis was delayed for more than 1 year in 25%, confirming that IPH diagnosis is demanding and requires a high index of suspicion.…”

“…HVPG was normal (5 mmHg) in 8 patients and mildly increased (6-10 mmHg) in 22. Twenty patients (40%) had HVPG greater than 11 mmHg (range, [11][12][13][14][15][16][17][18][19][20].…”

Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)—Newer Insights into Pathogenesis and Emerging Newer Treatment Options

“…14,15 EPIDEMIOLOGY NCIPH has been reported from all parts of the world, though more so from developing countries where it is most commonly encountered in young males in their third to fourth decade. [16][17][18] In contrast, in Japan and the West there is a female preponderance and a tendency to present around the fifth decade. 19 The relative rarity of the disease in the West and an apparent decline in its incidence elsewhere as living standards and hygienic conditions improve support the notion that enteropathy plays a role in disease pathogenesis.…”

Portal hypertension in nodular regenerative hyperplasia: A mixed bag!

“…1 NRH can also be considered a component of intrahepatic portal venopathy, an entity which also includes diseases like noncirrhotic portal fibrosis (NCPF) in the Indian subcontinent and idiopathic portal hypertension (IPH) in Japan. 2 Overall, NRH is an uncommon condition with only a few hundred cases described in the world literature. Autopsy studies have shown NRH in 2.6% of autopsy livers with a higher prevalence (5.6%) in patients aged more than 80 years, and those having systemic arteritis, polymyalgia rheumatica and massive tumor infiltration of liver.…”