Portal cavernomatosis in an adult patient. Case report
Keywords:
vascular malformations, protein c deficiency, hypertension, portalAbstract
Introduction: This is a case of portal cavernomatosis in an adult, associated with a rare concomitant deficiency of proteins C and S. This report highlights the importance of investigating thrombophilias in cases of non-cirrhotic portal hypertension and contributes an unusual etiological profile to the regional medical literature.
Clinical Case: A 36-year-old male patient with no significant medical history presented with massive hematemesis and precordial pain. Physical examination revealed splenomegaly and signs of hypovolemia. Laboratory studies showed acute anemia (Hb: 7.0 g/dL), thrombocytopenia (51,000/μL), and liver function tests within normal limits. Upper gastrointestinal endoscopy revealed grade II-III esophageal varices, and the diagnosis of portal cavernomatosis was confirmed by Doppler ultrasound and CT angiography. Thrombophilia testing identified protein C and S deficiencies. Management included hemodynamic stabilization, endoscopic varicose vain ligation, administration of octreotide, beta-blockers, and anticoagulation. The patient's condition improved, with control of bleeding and subsequent hospital discharge.
Conclusions: This case of portal cavernomatosis highlights the importance of considering thrombotic etiologies associated with thrombophilias in patients with portal hypertension without evidence of liver cirrhosis, reinforcing the value of a comprehensive diagnostic approach to similar cases.
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Copyright (c) 2025 Christian Giovanny Ordóñez Vélez, Jhuleydi Cumanda Chuncho Romero
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