Obliterative Portal Venopathy: A Study of 48 Children

Franchi‐Abella, Stéphanie; Fabrè, Monique; Mselati, Emmanuel; Marsillac, M. E. De; Bayari, Mohamed; Pariente, D.; Jacquemin, Emmanuel; Bernard, Olivier

doi:10.1016/j.jpeds.2014.03.025

Cited by 42 publications

(55 citation statements)

References 9 publications

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“…The age at onset tends to be lower in India (25‐35 years) than in Japan (43‐56 years) . In western countries the median age at diagnosis is about 40 years, although IPH may also appear in children …”

Section: Epidemiologymentioning

confidence: 99%

Idiopathic Portal Hypertension

et al. 2018

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Idiopathic portal hypertension (IPH) is a rare disorder characterized by clinical portal hypertension in the absence of a recognizable cause such as cirrhosis. Laboratory tests often reveal a preserved liver function with anemia, leukopenia, and thrombocytopenia due to splenomegaly. Imaging studies reveal signs of portal hypertension, whereas liver stiffness and portal pressure values are usually normal or slightly elevated. Liver biopsy is considered mandatory in order to rule out other causes of portal hypertension, mainly cirrhosis. Liver histology may only show subtle or mild changes, and the definite diagnosis of IPH often requires an expert pathologist and a high-quality specimen. The most frequent clinical presentation is variceal bleeding. Ascites is rarely observed initially, although it may occasionally appear during follow-up. Typical histological findings associated with IPH have been described in patients without portal hypertension, probably representing early stages of the disease. Although the pathophysiology of this entity remains largely unknown, it is frequently associated with underlying immunological disorders, bacterial infections, trace metal poisoning, medications, liver circulatory disturbances, and thrombotic events. The long-term prognosis of patients with IPH, where ascites and the underlying condition are important prognostic factors, is better than in patients with cirrhosis. Treatments that modify the natural history of the disease remain an unmet need, and management of IPH is frequently restricted to control of portal hypertension-related complications.

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Section: Epidemiologymentioning

confidence: 99%

Idiopathic Portal Hypertension

et al. 2018

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“…1 At times, this periportal hyperechogenicity alternates with hypoechoic stripes resulting in a "layered" appearance of the larger portal tracts (Figure 3). 4 The intrahepatic portal vein radicles show smooth and regular tapering with a sudden cut-off of the segmental and/or subsegmental branches, popularly termed as the "withered-tree" appearance. 1 Apart from substantiating portal hypertension, Doppler ultrasound plays an important role in assessing the patency of the splenoportal axis and hepatic veins that are pivotal to exclude other causes of NCPH, such as extrahepatic portal venous obstruction (EHPVO) or Budd-Chiari syndrome etc.…”

Section: Ultrasonographymentioning

confidence: 99%

“…[1][2][3] Obliterative portal venopathy (OPV) represents an important cause of NCPH that is characterized by sclerosis and obliteration of the medium-sized portal venous branches leading to portal hypertension. [1][2][3][4][5][6][7][8][9][10] Liver biopsy characteristically shows phlebosclerosis and periportal and perisinusoidal fibrosis amid absent cirrhosis ( Figure 1). [1][2][3] Although, the exact aetiology is contentious, infections and prothrombotic states have been implicated in eastern and western patients, respectively.…”

mentioning

confidence: 99%

“…1,2 Additionally, xenobiotic exposure, autoimmune and genetic factors have also been incriminated. [1][2][3][4] Although the disease has a worldwide distribution, it continues to remain poorly understood primarily owing to its relative rarity. [1][2][3][5][6][7][8] Another potential reason is the use of diverse terminologies under which the entity has been described from various parts of the globe, such as non-cirrhotic portal fibrosis in India, idiopathic portal hypertension in Japan and hepatoportal sclerosis in the USA.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Multimodality imaging of obliterative portal venopathy: what every radiologist should know

Arora¹,

Sarin²

2015

BJR

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Obliterative portal venopathy (OPV) is an important cause of non-cirrhotic portal hypertension, which is often erroneously misdiagnosed as cryptogenic cirrhosis. It has a worldwide distribution with majority of cases hailing from the Asian subcontinent. However, recently the disease has gained global attention particularly because of its association with human immunodeficiency virus infection and use of antiretroviral drug therapy (didanosine). As the name suggests, the disorder is characterized by sclerosis and obliteration of the intrahepatic portal vein branches (with attendant periportal fibrosis) leading to portal hypertension amid intriguingly little liver dysfunction. It primarily affects young adults who present with clinically significant portal hypertension in the form of episodes of variceal bleed; however, contrasting liver cirrhosis, the liver function and liver structure remain normal or near normal until late in the disease process. Radiological findings during advanced disease are often indistinguishable from cirrhosis often warranting a liver biopsy. Nevertheless, recent studies have suggested that certain imaging manifestations, if present, can help us to prospectively suggest the possibility of OPV. At imaging, OPV is characterized by a wide range of intrahepatic and/or extrahepatic portal venous abnormalities with attendant changes in liver and splenic volume and stiffness. We shall, through this pictorial review, appraise the literature and illustrate the germane radiological manifestations of OPV that can be seen using different imaging modalities including ultrasonography, CT, MRI, elastography and hepatic haemodynamic studies.

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“…The cause of OPV is unknown: several contributing factors have been suspected, including immunological and haematological mechanisms, chemical or drug toxicity, infection and thrombophilia, but in most cases no cause can be established . Observations of familial cases of OPV and of association of OPV with genetic disorders suggest a potential role for genetics in, at least, a subgroup of patients with OPV . Recently, mutations in several genes have been described in familial cases ( KCNN3 , DGUOK ), or in patients with other inherited ( TERT , TERC and other genes of telomere disorders) or developmental ( NOTCH1 and CTC1 related “syndromic cases”) disorders, and Turner syndrome .…”

Section: Introductionmentioning

confidence: 99%