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  • Title: [SOLVING A MEDICAL MYSTERY THROUGH THE EYES OF A YOUNG PATIENT WITH BUDD-CHIARI SYNDROME AND A NEW VISION LOSS].
    Author: Rabinovitch T, Habot-Wilner Z.
    Journal: Harefuah; 2022 Oct; 161(10):637-641. PubMed ID: 36315212.
    Abstract:
    INTRODUCTION: Budd-Chiari syndrome is a heterogeneous group of disorders characterized by venous drainage obstruction of the liver and is extremely rare. The clinical manifestations are usually ascites, varicose veins and in severe cases - hepatic insufficiency. Behcet's disease is a chronic, idiopathic, inflammatory disease that manifests as obstructive vasculitis and affects a variety of organ systems. Ocular involvement occurs in approximately 70% of the patients, and is a major clinical criterion in the diagnosis. Rarely, Behcet's disease can be a cause of Budd-Chiari syndrome. In these cases, the diagnosis has a crucial impact on the treatment and prognosis of the patients since patients with Budd-Chiari syndrome secondary to Behcet's disease, will usually improve under systemic medications with no need for surgery. In addition, in these patients there is a higher chance for developing hepatocellular carcinoma so they need to have a tight and a long follow-up. DISCUSSION: In this article we discuss a case of a young patient with Budd-Chiari syndrome, who was examined due to acute vision loss in his left eye. Left eye examination revealed panuveitis presenting with anterior uveitis, intermediate uveitis and an occlusive retinal vasculitis. The ocular examination raised suspicion that the diagnosis was Behcet's disease. Actually, Budd-Chiari syndrome was part of the presentation of Behcet's disease, which was not diagnosed until he was examined by us. The patient was treated with corticosteroid therapy and biological treatment with adalimumab, an anti-TNF drug. During follow-up, a complete resolution of the intraocular inflammation was achieved, as well as stabilization of its general condition, with the disappearance of the clinical signs indicative of liver failure.
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