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Título

Spontaneous ruptured arteriovenous malformation in a young patient: a case-based update

RESUMO

Case presentation: A 17-year-old female has presented a history of severe frontoparietal headache for 6 days, refractory to analgesics. After admission, the patient evolved with worsening headache associated with drowsiness, nausea, persistent vomiting and inappetence. There are no reports of comorbidities in the family and her parents are not consanguineous. Neurological exam revealed mild paresis in left limbs. A skull Computed Tomography (CT) showed the presence of an extensive frontoparietal hemorrhagic infarction with brain mass effect, indicative of intracranial hypertension. In addition, a brain Digital Subtraction Angiography (DSA) was performed and showed lobar intraparenchymal hemorrhage in the right temporal opercular region, with a estimated volume of 48cm³, presenting circulating vasogenic edema and Spetzler-Martin grade 1. A right temporal arteriovenous malformation was diagnosed.
Discussion: Arteriovenous malformations (AVMs) are the most dangerous of the cerebrovascular malformations with the potential to cause intracranial hemorrhages and even epilepsy. The most common clinical presentations are intracranial hemorrhages, most commonly intraparenchymal, but they can also occur in the intraventricular or subarachnoid space. Bleeding into the subarachnoid space is common in superficial AVMs. In addition, seizures, focal deficits and headache are common. The Spetzler-Martin scale (1 to 5) classifies the risk of surgical removal of the AVM according to its size, location in eloquent areas or not, and whether deep venous drainage is present. High scores correlate with greater risks of surgical morbidity and neurological deficits. Patient age is an important factor in the decision to treat brain AVMs, as patients with a longer life expectancy appear to accumulate a higher lifetime risk of hemorrhage. Thus, therapy is best suited for children and young adults. Brain DSA is essential for treatment planning and postoperative follow-up, as it is possible to better obtain the configuration of the nidus, its relationship with the circulating vessels, and the location of the draining or efferent portion of the cerebral AVM.
Final comments: Spontaneous rupture of arteriovenous malformations in young patients is associated with the possibility of performing immediate surgical interventions, which allow lower risks of acute complications. However, these patients with longer life expectancy appear to have a higher lifetime risk of hemorrhage.

Palavras Chave

arteriovenous malformation, rupture, young patient

Área

Doença Cerebrovascular