Cerebral vascular malformations: SAQ

This boy has an acute intracerebral haemorrhage from a ruptured arteriovenous malformation in the left parietal lobe. The sudden severe headache, the vomiting, and the right-sided weakness in a previously well child point to a bleed, and the tangle of vessels on the MRA confirms the underlying malformation. The priority is to stabilise the airway, breathing, and circulation, to manage the raised intracranial pressure, and to involve the multidisciplinary neurovascular team early, because the definitive treatment is lesion-specific. ^[9][2]

Question 1 (10 marks)

Outline the classification of cerebral vascular malformations, the acute management of this boy from the moment of arrival, and the investigations you would request. ^[1]

Cerebral vascular malformations divide into four lesions along two axes. The arteriovenous malformation and the vein of Galen malformation are high-flow lesions with a direct artery-to-vein connection, and the cavernous malformation is a low-flow lesion with no arterial input. The developmental venous anomaly is a normal drainage pattern and not a true malformation. The arteriovenous malformation is a tangle called a nidus, the vein of Galen malformation is a dilated midline venous sac, and the cavernous malformation is a cluster of thin-walled blood cavities. ^[1][2]

The acute management begins with the airway, breathing, and circulation. This boy is drowsy, so I would assess his airway and secure it if his Glasgow coma score falls or if he cannot protect it. I would maintain a normal oxygen saturation and a normal carbon dioxide, because both hypoventilation and hyperventilation shift the cerebral blood flow. I would support the circulation with isotonic fluids and avoid an excessive blood pressure, because a raised pressure can worsen the bleed. ^[9]

The raised intracranial pressure is managed with the head elevation to thirty degrees, the normothermia, and the normoglycaemia, and I would involve the neurosurgical team early because a large or a posterior-fossa haematoma may need an urgent decompression. The bedside glucose is confirmed normal, and the seizures are treated with a standard anticonvulsant if they occur. I would keep the child nil by mouth until the swallow is assessed, and I would arrange the retrieval to the paediatric neurosciences centre with a team that can manage the airway and the haemodynamics. ^[9][1]

The investigations begin with the cross-sectional imaging. The urgent CT has shown the haemorrhage, and the MRI with MRA has confirmed the arteriovenous malformation. I would request a catheter angiogram as the gold standard to resolve the nidus and the draining veins and to plan the treatment. I would send the routine bloods including a full blood count, a coagulation screen, and an electrolyte profile, and I would request an electroencephalogram if a seizure is suspected. ^[1][2]