Cerebral venous thrombosis (CVT) is a serious multifactorial disease that has been reported to result from a combination of prothrombotic risk factors and/or underlying clinical conditions. The aetiology is related to thrombophilia, head and neck infections, and systemic illnesses. The incidence is estimated at .67 per 100 000 children and is still poorly understood. In addition, the clinical presentation can be easily confused with idiopathic intracranial hypertension.
We report the case of a previously healthy 11-year-old male referred to an ophthalmologist with a 10-day history of headache and several days of blurry vision. After normal ocular exam, motility examination demonstrated no stereoacuity with a right esotropia at distance and near. Posterior pole examination exposed optic nerve swelling bilaterally, and subsequent lumbar puncture showed a markedly elevated opening pressure of 50 mmHg with normal cerebrospinal fluid composition.
The patient was admitted to the hospital for further management where acetazolamide 250 mg three times a day was started. Since the initial magnetic resonance imaging was normal, management was based on a working diagnosis of pseudotumour cerebri. Further magnetic resonance arteriography/venography of the head revealed a left internal jugular thrombosis with decreased flow in the left transverse sinus shown in Figure 1. The patient was started on 70-mg enoxaparin subcutaneously every 12 h. On day eight of his hospitalisation, the results of the dilute rapid viper venom test and IgG cardiolipin antibody returned as positive, suggesting antiphospholipid antibody syndrome. On day 10, the patient's symptoms were improving, and he was discharged with continued long-term oral anticoagulation.
Two weeks after discharge, the child's ophthalmic examination was improving; there was only limited abduction deficit in the right eye. Upon his six-week follow-up, his headaches, swelling and sixth nerve palsy were completely resolved.
In this case, there were a number of unusual factors that would have further delayed the correct diagnosis if not for doing a magnetic resonance venography. The atypical presentation for CVT and antiphospholipid antibody syndrome included his age, gender and lack of inciting infection. The diagnosis in children is susceptible to being misdiagnosed as pseudotumour which can delay proper treatment and lead to increased morbidity. The unusual presentation in this case highlights the importance of early MRV for children presenting signs of pseudotumour, whether they have other neurologic symptoms or not.