CASE PRESENTATION AND MANAGEMENT OF PORTAL CAVERNOMA CHOLANGIOPATHY

Document Type

Conference Proceeding

Publication Date

7-17-2025

Publication Title

J Gen Intern Med

Keywords

alcohol, alkaline phosphatase, anticoagulant agent, antithrombin III, beta adrenergic receptor blocking agent, cardiolipin antibody, hemoglobin, low molecular weight heparin, protein C, abdominal pain, anticoagulation, antiphospholipid syndrome, arterial embolization, ascites, biliary cirrhosis, case report, clinical article, coil embolization, collateral vein, common bile duct, common hepatic duct, complication, conference abstract, drug therapy, female, first-line treatment, gastrointestinal hemorrhage, hematemesis, hepatic portal vein, human, intrahepatic bile duct, liver failure, liver fibrosis, melena, menorrhagia, oligomenorrhea, portal biliopathy, portal hypertension, portal vein thrombosis, right upper abdominal quadrant, smoking, spleen artery aneurysm, splenic artery, splenomegaly, surgery, thrombophilia, variceal bleeding, variceal ligation, varicosis

Abstract

CASE: A 32-year-old female presented with acute, projectile hematemesis, melena, and sharp right upper quadrant abdominal pain (9/10). She had no prior medical history aside from menorrhagia and oligomenorrhea, and denied smoking, alcohol, or drug use. Lab results showed AST 29, ALT 21, alkaline phosphatase 60, hemoglobin 9.4, PT 23.7, and INR 2.13. An EGD revealed multiple grade 3-4 varices which were banded. CT imaging showed severe dilation of intrahepatic biliary ducts, massive splenomegaly, extensive portal vein (PV) thrombosis, and an enhancing mass-like structure circumferentially surrounding the common hepatic duct. Findings were suspicious for portal cavernoma cholangiopathy (PCC). Laparoscopy confirmed splenomegaly, 1L ascites, and a cystic mass around the common bile duct. Splenic angiography demonstrated a splenic artery aneurysm, which was treated with coil embolization. Hypercoagulable workup indicated a JAK2 mutation, decreased protein C and S, elevated cardiolipin antibodies, and decreased antithrombin III activity, indicating a complex hypercoagulable state and potential antiphospholipid syndrome. IMPACT/DISCUSSION: Portal Cavernoma Cholangiopathy (PCC) results from chronic portal vein thrombosis (PVT), leading to the formation of collateral veins, which compress surrounding structures, causing biliary or hepatic duct compression. PCC may lead to complications like liver fibrosis or secondary biliary cirrhosis, causing liver failure if left untreated. The patient presented with gastrointestinal bleeding due to portal hypertension, highlighting the latent nature of PVT. The patient's hypercoagulable state was due to a combination of genetic (JAK2 mutation), acquired, and autoimmune factors, leading to an increased thrombosis risk. First-line treatment for PVT is anticoagulation with unfractionated or low molecular weight heparin to improve vessel patency and reduce disease progression. Despite the initial bleeding risk, long-term anticoagulation outweighs the short-term risks, and treatment may be transitioned to oral anticoagulants. Variceal bleeding may be managed with non-selective beta blockers or variceal ligation. Splenic artery embolization (SAE) was performed to reduce portal hypertension, splenomegaly, and variceal bleeding risk. Early identification and management of the hypercoagulable state could have prevented PVT and its complications. CONCLUSION: Early Recognition of Hypercoagulable States: Unexplained thrombosis in atypical sites should prompt evaluation for underlying hypercoagulable conditions to prevent severe complications like PCC. Balance of Anticoagulation: In patients with both thrombosis and bleeding, anticoagulation decisions must weigh risks and benefits. Long-term anticoagulation is crucial despite initial bleeding risks. Multidisciplinary Approach: Effective management of portalhypertension requires a combination of interventions. Collaboration across specialties is key to optimizing outcomes in complex conditions like PCC.

Volume

40

First Page

S172

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