KEGG   DISEASE: Extrahepatic portal vein obstruction
Entry
H01687                      Disease                                
Name
Extrahepatic portal vein obstruction;
Extrahepatic portal venous obstruction
Description
Extrahepatic portal vein obstruction (EHPVO) is a vascular disorder of liver characterized by obstruction and cavernomatous transformation of portal vein with or without the involvement of intrahepatic portal vein, splenic vein, or superior mesenteric vein. The most characteristic imaging manifestation is the formation of porto-portal collaterals that allow hepatopetal flow. Gradually there is organization and temporal evolution of thrombus with formation of portal collaterals termed "cavernoma" aiming to bypass the obstructed portal vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension (PHT) in developing countries.
Category
Digestive system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 13 Diseases of the digestive system
  Diseases of liver
   DB98  Vascular disorders of the liver
    H01687  Extrahepatic portal vein obstruction
Comment
See also H01686 Idiopathic portal hypertension
Other DBs
ICD-11: DB98.3
Reference
  Authors
Pargewar SS, Desai SN, Rajesh S, Singh VP, Arora A, Mukund A
  Title
Imaging and radiological interventions in extra-hepatic portal vein obstruction.
  Journal
World J Radiol 8:556-70 (2016)
DOI:10.4329/wjr.v8.i6.556
Reference
  Authors
Arora A, Sarin SK
  Title
Multimodality imaging of primary extrahepatic portal vein obstruction (EHPVO): what every radiologist should know.
  Journal
Br J Radiol 88:20150008 (2015)
DOI:10.1259/bjr.20150008
Reference
  Authors
Wani ZA, Bhat RA, Bhadoria AS, Maiwall R
  Title
Extrahepatic portal vein obstruction and portal vein thrombosis in special situations: Need for a new classification.
  Journal
Saudi J Gastroenterol 21:129-38 (2015)
DOI:10.4103/1319-3767.157550
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