Cerebral vascular malformations

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Target exams

Red flags

• A sudden severe headache or new focal deficit in a child is an intracranial bleed until imaging proves otherwise, and a vascular malformation is a leading cause
• A neonate with high-output cardiac failure, a loud cranial bruit, and cerebral venous congestion has a vein of Galen malformation until proven otherwise
• A cavernous malformation that has bled into the brainstem or caused drug-resistant epilepsy is a surgical lesion
• A developmental venous anomaly must never be resected, because it drains normal brain and removing it causes a venous infarct
• A paediatric arteriovenous malformation recurs more often than an adult lesion, so a post-treatment angiogram and years of follow-up are mandatory
• A vein of Galen malformation with a low Bicetre neonatal score is not embolised in the neonatal period, because the procedural mortality outweighs the benefit
• A familial cavernous malformation with multiple lesions points to a CCM gene mutation and demands family screening

Life stages

Care settings

Clinical exam formats

Board mappings

• Classifies cerebral vascular malformations into arteriovenous malformation, vein of Galen aneurysmal malformation, cavernous malformation, and developmental venous anomaly, and states that the high-flow lesions present with haemorrhage
• Recognises that a sudden intracerebral haemorrhage in a child and neonatal high-output cardiac failure are the two emergency presentations of a vascular malformation
• States that a developmental venous anomaly must never be resected because it drains normal brain
• Applies the Spetzler-Martin grade to an arteriovenous malformation and the Bicetre neonatal evaluation score to a vein of Galen malformation to guide management
• Coordinates the multidisciplinary treatment of an arteriovenous malformation with microsurgery, embolisation, and stereotactic radiosurgery
• Explains why a paediatric arteriovenous malformation recurs and why a post-treatment angiogram and years of follow-up are mandatory
• Classification of cerebral vascular malformations by flow and structure, with haemorrhage as the dominant paediatric presentation
• Urgent MRI brain with MRA and MRV as the imaging standard, with catheter angiography reserved for confirmation and treatment planning
• The Bicetre neonatal evaluation score for vein of Galen and the Spetzler-Martin grade for arteriovenous malformation
• Runs the acute assessment of a child with a sudden intracerebral haemorrhage and stabilises the airway, breathing, and circulation before imaging
• Recognises neonatal high-output cardiac failure as the presentation of a vein of Galen malformation and escalates to a paediatric interventional neuroradiology centre
• Counsels the family on the diagnosis, the treatment options, the recurrence risk, and the long-term follow-up
• Level 1: Recognition that a vascular malformation is a leading cause of nontraumatic intracerebral haemorrhage in a child
• Level 2: Application of the urgent imaging pathway and the multidisciplinary treatment plan
• Level 3: Coordination of the long-term angiographic follow-up and the family screening for the familial cavernous malformation
• Classification and epidemiology of cerebral vascular malformations, with arteriovenous malformation the commonest lesion to present in childhood
• The Bicetre neonatal evaluation score and its role in timing the embolisation of a vein of Galen malformation
• The familial cavernous malformation and the CCM1, CCM2, and CCM3 genes
• Structured bedside assessment and stabilisation of a neonate with high-output cardiac failure from a vein of Galen malformation
• Recognition of an intracerebral haemorrhage from an arteriovenous malformation and the multidisciplinary treatment plan
• Communication of the diagnosis, the treatment options, and the long-term follow-up to the family
• Arteriovenous malformation as the commonest cerebral vascular malformation to present with haemorrhage in a child
• Vein of Galen malformation with neonatal high-output cardiac failure and transarterial embolisation
• Cavernous malformation with seizures or haemorrhage and surgical resection for the symptomatic lesion
• Recognition of the emergency presentations of a cerebral vascular malformation and escalation to a paediatric neurosciences centre
• Supportive care and the multidisciplinary treatment pathway for arteriovenous malformation and vein of Galen malformation
• Arrangement of the long-term angiographic follow-up and the family screening
• Canadian approach to cerebral vascular malformations within a regional paediatric neurosciences network
• Spetzler-Martin grading of an arteriovenous malformation and the Bicetre neonatal evaluation score for vein of Galen
• Transarterial embolisation of vein of Galen malformation and surgical resection of the symptomatic cavernoma