Atlanto-axial rotatory fixation caused by spontaneous intracerebral haemorrhage in a child

Atlanto-axial rotatory fixation is rare in children and usually associated with upper respiratory tract infection or trauma. We present a patient who developed the condition acutely as a consequence of rapidly evolving hemiplegia secondary to intracerebral haemorrhage.

A previously well 12-year-old boy suddenly developed left hemiparesis within 30 min. Soon after, he developed painful torticollis. On examination, a dense left hemiplegia was associated with left facial and hypoglossal weakness. On computerised tomography (CT) scan there was an intracerebral haemorrhage in the deep aspect of the right frontal lobe in the corona radiata, with extension in the ventricular system. The plain radiographs of the cervical spine, and CT scan later, confirmed the presence of C1–C2 rotatory subluxation. The haematoma was aspirated stereotactically through a burr hole, and at the same session, the subluxation was reduced by manipulation and traction of the cervical spine. The patient was treated with a hard collar. The torticollis did not recur. The hemiparesis resolved within 6 months. A subsequent magnetic resonance imaging and magnetic resonance angiography scan indicated possible arterio-venous malformation (AVM) at the site of the haemorrhage, which was confirmed with digital subtraction angiography. The AVM was surgically excised 1 year from the original haemorrhage.

In the absence of any other predisposing factor, it is postulated that the acute atlanto-axial rotatory subluxation was secondary to the acute loss of muscular tone due to the acute hemiparesis. This explains that the subluxation has not recurred a year after, while the hemiparesis has completely resolved.