Naanous RJ, Murrieta PE, Ramírez LJ

Language: Spanish
References: 15
Page: 317-322
PDF size: 490.58 Kb.

ABSTRACT

Budd-chiari syndrome is a rare disease in which signs of portal hypertension develop secondary to an obstructive process, most frequently thrombotic, of the hepatic flow. The symptoms vary according to the time of evolution, so a high clinical suspicion is required to achieve the diagnosis. Due to this, the incidence of this disease is unknown, most of the literature is based on case reports. In patients with signs of portal hypertension without a clear origin, we must rule out this disease, and in turn seek its etiological diagnosis. We present the case of a young woman, 29 years old, who comes to evaluation for presenting clinical data compatible with a portal hypertension syndrome characterized by the presence of jaundice, ascites, hepatomegaly, digestive tract bleeding secondary to the presence of esophageal and rectal varicose veins. Abdominal tomography with contrast revealed multiple hepatic lesions, reported as probable secondary deposits of unknown primary. Magnetic Resonance Imaging was performed where a decrease in the caliber of the suprahepatic and portal vessels was identified, as well as that of the inferior vena cava in the intrahepatic segment with the presence of a thrombus inside, for which the diagnosis of syndrome of Budd-Chiari. As a therapeutic measure, a transjugular intrahepatic portosystemic shunt was performed with significant clinical improvement. After an extensive approach for non-cirrhotic causes of portal hypertension of posthepatic location, the JAK2 V617F mutation was detected in relation to a myelodysplastic syndrome associated with Budd-Chiari syndrome.

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