A four-year-old boy was admitted to the hospital due to recurrent oral aphthae and erythema nodosum. He had epididymitis in two occasions. The patient has nonconsanguineous healthy parents. Oral mucosa ulcerations and anal fissure were noticed in physical examination. The patient did not have uveitis. Pathergy test was positive. Laboratory test results are presented in Table 1. The patient was diagnosed with BD and treated with colchicine and prednisolone (1mg/kg/day).

The patient had normal blood pressure; however, bilateral papilledema was detected. Magnetic resonance imaging (MRI) showed hyperintense thrombosis in the superior sagittal sinus and in both proximal segments of transverse sinuses (Fig. 1a). Subcutaneous low-molecular-weight heparin (LMWH) was started. The patient was investigated for a prothrombotic state and a methylenetetrahydrofolate reductase (MTHFR) heterozygous mutation was found. Azathioprine was added. One month later bilateral papilledema was detected again. The brain MRI was repeated and a new thrombosis distal to the superior sagittal sinus was detected. After permission from the ministry of health, infliximab was used at a 5mg/kg/day dose and 3 doses were applied on the 0, 2 and 6 weeks. Before the infliximab, azathioprine was stopped; prednisolone was tapered and stopped at the 7th day of infliximab. Ten days after first dose of infliximab, C-reactive protein was decreased to normal range, and both frequency and severity of the oral aphthous lesions were reduced. Resolution of the thrombosis was seen after the second dose of infliximab in the brain MRI (Fig. 1b). Ongoing therapies consist of colchicine and LMWH. Infliximab was well tolerated by this patient; no drug-induced side effects were recorded.

Fig. 1.

(a) Magnetic resonance imaging of brain before the infliximab. Hyperintense thrombosis filling the superior sagittal sinus and proximal segments of both transverse sinuses was showed and (b) after second dose of infliximab, significant dissolution of the thrombosis was observed in the brain MRI.

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The mean age of onset in pediatric BD epidemiologic studies has been 7 years.2,3 The earliest age reported for thrombotic BD is 11-years.4 To the best of our knowledge, this is the youngest Behçet's patient who had cerebral thrombosis.

Vascular lesions in BD can involve both arterial and venous vessels, and cause refractory complications with poor prognosis. In our patient, cerebral thrombosis was refractory to treatment which corticosteroids, immunosuppressants, and anti-coagulants. The administration of infliximab resulted in the improvement of clinical and laboratory findings. In addition, cerebral sinus thrombosis in MRI was reduced.

The vascular involvement of BD develops in the active stage. Mononuclear cells infiltrate the vessel walls and damages them, and this may cause thrombosis.5 Infliximab may inhibit the activation of infiltrated mononuclear cells by blockade of TNF and TNF-producing cells, resulting in the prevention of thrombosis. Although long-term efficacy evaluations are still needed in this case, infliximab therapy may be a good option in childhood BD with refractory sinus thrombosis.

Since the International Study Group criteria are accepted for adult BD but not for pediatric BD, the delay in diagnosis of pediatric BD is unavoidable. Especially in the Silk Road countries, even in the absence of the cardinal findings of BD, pediatric patients with recurrent oral aphthous ulcers should be followed up for early detection and treatment of BD complications.

None.