Ann Hepatobiliary Pancreat Surg.  2022 Nov;26(4):298-307. 10.14701/ahbps.22-029.

Portal cavernoma cholangiopathy: Update and recommendations on diagnosis and management

• Bhavsar R
• Yadav A
• Nundy S

• KMID: 2536378
• DOI: http://doi.org/10.14701/ahbps.22-029

Abstract

Portal cavernoma cholangiopathy is defined as an obstruction of the biliary system due to distended veins surrounding bile ducts that mainly occur in patients with extrahepatic portal venous obstruction. The periductal venous plexuses encircling the ducts can cause morphological changes which may or may not become symptomatic. Currently, non-invasive techniques such as ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and dynamic contrast enhanced magnetic resonance images are being used to diagnose this disorder. Only a few patients who have symptoms of biliary obstruction require drainage which might be accomplished using endoscopic stenting, decompression of the portal venous system usually via a lienorenal shunt, a difficult direct hepaticojejunostomy, and rarely a liver transplant.

Keyword

Portal cavernoma; Extra hepatic portal venous obstruction; Biliary strictures; Shunts; Hepaticojejunostomy

Figure

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  Fig. 1 Normal anatomy of biliary tract. Cited from the article of Ramesh Babu et al. (J Clin Exp Hepatol 2014;4(Suppl 1):S18-S26) [7]. V, segment V duct; VII, segment VII duct; VIII, segment VIII duct; RASD, right anterior sectoral duct; RPSD, right posterior sectoral duct; RHA, right hepatic artery; LHA, left hepatic artery; CD, cystic duct; CBD, common bile duct; CHA, common hepatic artery; GDA, gastroduodenal artery; SV, splenic vein; SMV, superior mesenteric vein; PD, pancreatic duct; PSPDA, postero-superior pancreatico-duodenal artery.

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  Fig. 2 Normal arterial supply of the biliary tract. Cited from the article of Ramesh Babu et al. (J Clin Exp Hepatol 2014;4(Suppl 1):S18-S26) [7]. RASA, right anterior sectoral artery; RPSA, right posterior sectoral artery; IV-A, segment IV artery; CA, cystic artery; RHA, right hepatic artery; LHA, left hepatic artery; A, artery; PSPDA, postero-superior pancreatico-duodenal artery; GDA, gastro-duodenal artery; CHA, common hepatic artery.

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  Fig. 3 Venous drainage of the biliary tract (anterior & posterior views). Cited from the article of Ramesh Babu et al. (J Clin Exp Hepatol 2014;4(Suppl 1):S18-S26) [7]. V, segment V; IV, segment IV; III, segment III; RASD, right anterior sectoral duct; RPSD, right posterior sectoral duct; LHD, lef t hepatic duct; RHD, right hepatic duct; CHD, common hepatic duct; V., vein; RGV, right gastric vein; CBD, common bile duct; PSPDV, postero-superior pancreatico-duodenal vein; SMV, superior mesenteric vein; D2, 2nd part of duodenum; PV, portal vein; LPV, left portal vein; RPV, right portal vein; LHD, left hepatic duct; RGV, right gastric vein.

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  Fig. 4 Contrast-enhanced computed tomography abdomen of a patient with extra hepatic portal venous obstruction showing col laterals and cavernoma formation resulting in biliary dilatation. (A, C) Showing portal cavernoma formation with compression over the lower common bile duct. (B, D) Showing intra-hepatic biliary radicle dilatation.

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  Fig. 5 Magnetic retrograde cholangiopancreatography. (A, C) Showing portal cavernoma with compression resulting in biliary stricture (arrow) with triple phase computed tomography scan. (B) Showing similiar feature (arrow head).

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  Fig. 6 Intraoperative photos from a primary biliary decompression being done. (A) Common hepatic duct opened just at hepatic bifurcation with stents in situ seen. (B) Large collateral being tied between ligatures. (C) Hepatic duct near bifurcation ready for hepaticojejunostomy (arrow).

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  Fig. 7 Comparative magnetic resonance cholangiopancreatography pictures showing preoperative (extra hepatic portal venous obstruction with portal cavernoma with upstream bi liary di latat ion and hepatolithiasis) (A) and postoperative (shunt surgery followed by biliary bypass showing resolution of biliary dilatation and patent anastomosis) (B).

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