Asymptomatic Portal Cavernoma Revealing Activated Protein C Resistance

Salem Bouomrani*, Nesrine Regaïeg, Mayada BenHamad, Souad Yahyaoui and Nesrine Belgacem

Received: September 12, 2018;   Published: September 17, 2018

DOI: 10.32474/CTGH.2018.01.000116

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Abstract

Introduction: Unlike the child, the portal cavernoma (PC) remains an exceptional and unusual complication of portal thrombosis in adults and the elderly. We report an original observation of asymptomatic PC secondary to chronic portal thrombosis revealing Factor V Leiden mutation in a young adult.

Observation: A 27-year-old female with no significant pathological history was explored for thrombocytopenia found on a routine check-up. Radiological investigation concluded to the diagnosis of post-thrombotic PC with extra-hepatic portal hypertension. Systematic screening for acquired and inherited thrombophilia objectified activated protein C resistance, and genetic test confirmed the specific mutation of the coagulation factor V gene (Factor V Leiden). Under effective anticoagulation, the evolution was favorable.

Conclusion: As rare as it is in adult, PC deserves to be known and his diagnosis imposes a systemic screening for inherited thrombophilic disorders even in the absence of family and/or personal history of thromboembolic disease.

Keywords: Portal Cavernoma; Portal Vein Thrombosis; Factor V Leiden; Activated Protein C Resistance; Inherited Thrombophili

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