Extrahepatic Portal Vein Obstruction

Abstract

Extrahepatic portal vein obstruction (EHPVO) is an important cause of noncirrhotic portal hypertension, especially in Third World countries. The etiology and clinical presentation are different in children and adults. The portal vein is transformed into a cavernoma, resulting in portal hypertension and oesophagogastic varices. In addition, extensive collateral circulation develops, involving paracholecystic, paracholedochal and pancreaticoduodenal veins resulting in formation of ectopic varices, and portal biliopathy. Besides variceal bleeding, which is the commonest presentation, patients may have symptomatic portal biliopathy, hypersplenism, and growth retardation. Although the liver may appear normal, functional compromise develops in the long term. Variceal bleeding in EHPVO can be successfully managed by endoscopic obliteration of varices, which has low morbidity but requires repeated visits, or by portosystemic shunt surgery, which provides good control of bleeding, possibly helps growth retardation, hypersplenism, and protects against future development of portal biliopathy but is associated with surgical mortality and is sometimes not feasible due to nonavailability of a satisfactory vessel.

Collateral Pathways in Portal Hypertension

“…Portal hypertensive biliopathy (PHB) refers to abnormalities of the biliary tract in patients due to these portoportal collaterals. 76,77 These collateral veins are related to 2 preformed venous systems near the extrahepatic bile ducts: the paracholedochal (PACD) veins of Petren, and the epicholedochal (ECD) venous plexus of Saint. The PACD venous plexus of Petren runs parallel to the CBD, and the ECD plexus of Saint veins form a reticular mesh on the surface of the CBD.…”

Surgical Management of Portal Cavernoma Cholangiopathy

“…Ten patients underwent splenorenal shunts and of the remaining 9 patients, 4 of them had nonshuntable veins due to widespread thrombosis, 3 had successful endoscopic biliary drainage before referral, and in 2 patients the diagnosis of PCC was not confirmed before surgery. Hence in the non-shunt group (9), biliary stenting (2), stone extraction (1), and percutaneous biliary drainage were performed for cholangitis (4), and pruritus (2). Four patients underwent biliodigestive anastomoses.…”

“…As stated earlier, PCC is seen in 80-100% of patients with EHPVO; however it is symptomatic in only 5-38%. [1][2][3][4][5][6] Though the numbers are small, there is no dearth of published data on the pathophysiology, diagnosis and management of PCC apart from the guidelines on the algorithmic approach to such patients. There are certain areas in the management which are still controversial and there is no clear cut way forward for example the role of prophylactic shunt in patients with asymptomatic PCC, the role of early shunts in symptomatic PCC as against primary role of endoscopic treatment, and the role of makeshift portosystemic shunt or splenectomy devascularization in patients with nonshuntable veins.…”

“…These changes were reported mainly in patients with extrahepatic portal venous obstruction (EHPVO) where the incidence was as high as 80-100%. [1][2][3][4][5][6] The majority of patients with PCC are asymptomatic, however some present with obstructive jaundice, cholangitis, or even biliary pain due to bile duct stones which form as a result of stasis or repeated infections. Only 5-38% of patients with portal hypertension will have symptomatic PCC.…”

“…Only 5-38% of patients with portal hypertension will have symptomatic PCC. 4 Most patients with EHPVO present with variceal bleeding and hypersplenism and these are the usual indications for surgery. Those who present with PCC may also need decompression of their portal venous system to reverse the biliary obstruction.…”