Superior vena cava syndrome revealing a Behçet’s disease

Abstract

IntroductionBehçet’s disease (BD) is a rare vasculitis in sub-Saharan Africa. Vascular thrombosis, especially venous, is common in this condition and also constitutes a basic diagnostic criterion. Its affection of the superior vena cava is rather rare with only a few cases described in the literature.Case reportA 42-year-old male patient was seen at consultation presenting with a pulsatile, warm and slightly painful right latero-cervical swelling extending to the supraclavicular fossa with the presence of collateral venous circulation for three weeks prior to presentation associated with a mild headache. There were oral and genital ulcerations and erythematous skin lesions associated with a history of inflammatory recurrent arthralgia. Chest computed tomo-angiography showed cruoric internal jugular vein thrombosis extending to the superior vena cava with significant venous collateral circulation. The patient was treated with prednisolone (1 mg/kg/day) and colchicine (2 mg/day), as well as anticoagulation with heparin and vitamin K antagonist (Acenocoumarol) with regular INR monitoring. Clinical evolution was favorable during hospitalization, with residual discrete right supraclavicular swelling. There was no bleeding associated with anticoagulants use.ConclusionThe case stresses the importance of maintaining a high degree of suspicion for Behçet’s disease in all cases of venous thrombosis.

Anticoagulation of Behcet’s Disease with Thrombosis: A Series of 28 Cases in a Tertiary Care Centre in Saudi Arabia

“…The vascular manifestations were reported in 10-40% of cases of BD and venous involvement was more common 80% to 90% of cases [10,11]. 57% of our patients had unprovoked thrombosis.…”

Retropharyngeal edema secondary to superior vena cava syndrome revealing Behcet's disease

“…1 It is characterized by recurrent oral ulcers, genital ulcers, uveitis, suggestive skin lesions (pseudofolliculitis, erythema nodosum, etc), and vascular thrombosis. 2 Its diagnosis is based on minor and major criteria: oral and genital ulcerations, skin lesions, uveitis, arthritis, and neurological and digestive signs according to the International Study Group for Behçet's Disease. 1 Behcet's disease is more common in the Mediterranean region and the Middle East.…”

“…1 Behcet's disease is more common in the Mediterranean region and the Middle East. 2 Superior vena cava syndrome is a group of clinical signs resulting from obstruction of blood flow through the superior vena cava. The most common symptoms are cervico-facial swelling, arm swelling, dyspnea, distended neck veins, and collateral venous circulation.…”

“…Behcet's disease is an inflammatory disease with vascular tropism; its clinical presentation is polymorphic, and its diagnosis is based on a set of criteria 1 . It is characterized by recurrent oral ulcers, genital ulcers, uveitis, suggestive skin lesions (pseudofolliculitis, erythema nodosum, etc), and vascular thrombosis 2 . Its diagnosis is based on minor and major criteria: oral and genital ulcerations, skin lesions, uveitis, arthritis, and neurological and digestive signs according to the International Study Group for Behçet's Disease 1 .…”

Evaluation of hearing results in Behçet's disease

“…Gastrointestinal, renal, pulmonary, cardiovascular, and musculoskeletal system involvement may be seen in addition to the triad described in patients with Behçet's disease [4]. In rare cases, vena cava superior syndrome [5], optic perineuritis [6], oculopalatal tremor [7], and audiovestibular symptoms have also been reported [8].…”