Background
Fibromuscular dysplasia (FMD) is a small to medium-vessel arterial disease most commonly affecting cerebrovascular and renal systems. Anti-platelet therapy is typically initiated to prevent thrombotic risks such as ischaemic stroke. Haemorrhagic sequalae are infrequent initial presentations, and spontaneous spinal subdural haematomas (SSDH) are exceedingly rare with few cases attributed to vasculopathies. The challenge arises in balancing these competing risks in multi-focal multi-vessel FMD.
Case
A 57-year-old female presented with bilateral lower limb paraesthesia, mild weakness and gait ataxia. She then developed headache, back pain and fever. Serum inflammatory markers were raised, and MRI brain showed posterior fossa meningeal hyperintensities. Evolution of her lower limb signs expedited MRI spine revealing an extensive (cervical-sacral) SSDH. She was conservatively managed with plateaued mild deficit. Catheter angiography confirmed FMD of spinal arteries (including a T7 radiculomedullary pseudoaneurysm) and profound involvement of intra- and extracranial neck, bronchial, visceral and lower extremity arteries. Full neurological recovery with radiologic SSDH resolution occurred after 1 month. Anti-platelets have not yet been instituted with an evolving consideration guided by multidisciplinary surveillance.
Discussion
This is the first reported case of an acute SSDH secondary to spinal angiographic-proven FMD. The acute management was complicated by infective concerns, considerable longitudinal extent, and widespread multi-vessel involvement; a readiness for neurosurgical decompression was in place. There are limited evidence-based principles to direct post-acute medical therapy in FMD, especially after haemorrhagic presentations. This case illustrates the importance of individualised decision-making for competing complexities, and reliance on long-term multidisciplinary input in multi-vessel FMD.