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Classification of noncirrhotic portal hypertension. Partial list of common causes of NCPH as separated by the location of disease. a Hepatic causes can otherwise be separated into presinusoidal, sinusoidal and postsinusoidal.

Classification of noncirrhotic portal hypertension. Partial list of common causes of NCPH as separated by the location of disease. a Hepatic causes can otherwise be separated into presinusoidal, sinusoidal and postsinusoidal.

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Purpose of review: Noncirrhotic portal hypertension represents a heterogeneous group of liver disorders that is characterized by portal hypertension in the absence of cirrhosis. The purpose of this review is to serve as a guide on how to approach a patient with noncirrhotic portal hypertension with a focus on recent developments. Recent findings:...

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... Abdominal imaging to characterize the liver, spleen and associated vasculature [ultrasound with Doppler assessment, computed tomography (CT), MRI] can also provide clues relating to portal hypertension [24 && ]. Also, once NPCH is ultimately diagnosed, imaging can be used to understand if the cause is prehepatic, hepatic or posthepatic in nature (Fig. ...

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Citations

Article
Cavernous transformation of the portal vein is a condition characterized by the formation of a network of dilated collateral veins along a previously thrombosed portal vein. It is considered a low-frequency entity and occurs more commonly in the pediatric population. We present the case of an adult woman with a diagnosis of cavernous transformation of the portal vein, originated as a consequence of occult essential thrombocythemia. As a therapeutic measure for portal hypertension, endoscopic ligation of gastroesophageal varices was performed on multiple occasions without positive results. Portosystemic bypass was not performed due to the presence of abundant collaterals. Finally, a splenectomy was performed, after which essential thrombocythemia was evidenced. The patient evolved with multiple medical-surgical complications, which led to multisystem failure and subsequent death. There are no regional prevalence data, nor have there been reports of cavernous transformation of the portal vein associated with essential thrombocythemia, for which reason we consider of great importance to make this case known, in order to help establish the diagnosis and treatment of this rare entity with greater precision and speed.
Article
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 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 is frequently restricted to control of portal hypertension related complications. This article is protected by copyright. All rights reserved.