Paediatric stroke and cerebral sinovenous thrombosis: Case

This girl has a cerebral sinovenous thrombosis until proven otherwise. The progressive headache and vomiting, the papilloedema, the new seizure, and the recent otitis media together form the classic presentation of a dural sinus thrombosis that has spread from the middle ear to the adjacent transverse and sigmoid sinuses. The right hemiparesis suggests a venous infarct in the left hemisphere, which may be haemorrhagic given the venous origin. The priority is to confirm the thrombosis with venous imaging and to initiate anticoagulation. [9][1]

Clinical findings and assessment

The key findings are the progressive headache with papilloedema, the seizure, the fever, and the mild right hemiparesis. The papilloedema is the sign that separates a venous sinus thrombosis from a routine postictal headache, because it reflects the raised intracranial pressure from the obstructed venous outflow. The recent otitis media, despite antibiotics, raises the possibility of a complicated ear infection with spread to the mastoid and the adjacent dural sinus, which is the classic local cause of cerebral sinovenous thrombosis in children. [9]

The bedside assessment confirms that the airway is patent, the glucose is normal, and the child is cardiovascularly stable, which allows the team to proceed to imaging without delay. The differential of headache, papilloedema, and seizures in a febrile child includes a cerebral sinovenous thrombosis, a brain abscess from the ear infection, and a bacterial meningitis, and the imaging and the lumbar puncture, once safe, will separate them. The working diagnosis is cerebral sinovenous thrombosis, which directs the imaging to include the MRV and the treatment to include anticoagulation. [3]

Immediate management

The immediate management is to secure the airway, treat the seizure with a standard anticonvulsant, and obtain urgent imaging. The imaging request is for an MRI brain with diffusion-weighted imaging, MRA, and MRV, because the MRV is the sequence that will reveal the venous sinus occlusion that a plain MRI or a CT would miss. If the child is too unstable for an MRI, a contrast-enhanced CT with a CT venogram is the alternative. The scan in this girl would show an absent flow in the left transverse and sigmoid sinuses on the MRV, with a venous infarct in the adjacent temporal and parietal region, which may be haemorrhagic. [9][3]

Once the diagnosis is confirmed, the definitive treatment is anticoagulation. I would initiate low-molecular-weight heparin with enoxaparin at 1 mg per kg subcutaneously every twelve hours, because she is over two months old and the predictable pharmacokinetics and the lower monitoring burden favour it over unfractionated heparin. I would continue the anticoagulation even if the venous infarct is haemorrhagic, because the haemorrhage is a consequence of the venous obstruction and the anticoagulation addresses the root cause. The American Heart Association and Mandel-Shorer and colleagues both affirm this approach, and withholding anticoagulation out of fear of the bleed is the classic pitfall. [2][9]

I would treat the underlying ear infection in parallel. I would send blood cultures, start broad-spectrum intravenous antibiotics to cover the common otogenic organisms, and involve the ear, nose, and throat team to assess for a mastoidectomy if the imaging shows a mastoid abscess. The fever and the infection are the precipitants, and their treatment is part of the management of the thrombosis, because an untreated infection drives the thrombosis to extend. [9]

Cause search and intensive care

The cause search is tailored to the cerebral sinovenous thrombosis. I would send iron studies, because iron deficiency anaemia is a treatable trigger in young children. I would send a thrombophilia screen, including protein C, protein S, antithrombin, factor V Leiden, and antiphospholipid antibodies, while acknowledging that the acute-phase derangement complicates the interpretation and that some results need repeating at three months. I would review the hydration status, because dehydration is the commonest systemic precipitant, and I would ensure adequate fluid resuscitation. [1][9]

The girl would be admitted to the paediatric intensive care unit for the acute phase, because the raised intracranial pressure, the seizure risk, and the need for close neurological monitoring warrant a high-dependency setting. The team would manage the airway, the seizure prophylaxis, the anticoagulation, and the intracranial pressure, and the paediatric neurology and infectious disease teams would be involved early to guide the cause search and the longer-term plan. [8]

Outcome and follow-up

The prognosis of cerebral sinovenous thrombosis is favourable, with about eighty percent of children surviving without deficit or with only a mild deficit. Mineyko and colleagues showed that seizures in the acute phase are the main predictor of a worse neurological outcome at one year, and this girl has had a seizure, which places her in the group that needs close follow-up. The recurrence rate is low when the underlying cause is treated, and the anticoagulation is typically continued for three to six months, guided by the resolution of the thrombosis on a follow-up MRV. [8][9]

I would counsel the family honestly at every stage. I would explain that their daughter has a clot in one of the veins draining the brain, that the clot came from the ear infection, and that the headache and the seizure came from the pressure building up behind the clot. I would explain that the medicine to thin the blood will stop the clot growing and allow the vein to reopen, that the antibiotics will treat the ear infection that caused it, and that the team will watch her closely in intensive care. I would state that most children recover well, that the rehabilitation will address any weakness that remains, and that the team will arrange a follow-up scan and a clinic visit to confirm the clot has resolved. Before discharge, the family would be connected to the paediatric neurology service, and a clear plan would be provided for what to do if the symptoms recur. [3][8]