Diagnosis and Management of Arteriovenous Malformations in Children

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Incidence and natural history

Matson12 evaluated 34 pediatric patients and declared AVM “the most frequent abnormality of intracranial circulation in childhood.” Excluding hemorrhages of prematurity and early infancy, AVM is the most common cause of spontaneous intraparenchymal hemorrhage in children. Hence, a spontaneous intraparenchymal hemorrhage in a child should be considered an AVM until proven otherwise. Because most children diagnosed with AVMs undergo initial treatment emergently, the natural history of AVMs in the

Pathology

AVMs may form anywhere in the embryonic brain but most originate above the tentorium, where their roots extend over the hemispheric surfaces and dig deep into the cortex. There is a structural defect in the formation of the arteriolar capillary network that is normally present between arteries and veins within the substance of the brain. The exact mechanism by which these malformations form is unknown; however, it is hypothesized that most malformations occur during the third week of

Diagnosis

Most AVMs in pediatric patients do not come to clinical attention unless they hemorrhage. The high mortality rate of hemorrhagic events associated with an AVM underscores the importance of accurate diagnosis. Advances in imaging modalities have greatly contributed to the ease in diagnosis of intracerebral hemorrhages caused by AVMs. Computed tomography (CT) is often the initial study performed, and it shows the presence of an intracranial hemorrhage and any calcification. Contrast enhancement

General Considerations

Treatment of AVMs focuses on the complete obliteration or resection of the vascular lesion to prevent future recurrence of hemorrhage and to preserve and restore neurologic function. Success of the treatment depends on the location and size of the AVM, its hemodynamic properties, the clinical condition of the patient, and the treatment modality selected. The armamentarium available for AVM management has grown with technological advances and now includes microsurgical resection, endovascular

Follow-up

There is an increasingly recognized phenomenon of late AVM recurrence, even after angiographic cure.3, 40, 46, 74, 75, 76 Klimo and colleagues3 recently reviewed the literature and identified 29 published cases of recurrent AVMs, of which 20 (69%) were that of children. They also found that diffuse-type AVMs were more likely to develop a recurrence. The longest reported interval between total surgical removal and recurrence is 19 years.76 Maher and Scott4 recently reported the Children's

Summary

The optimal management for pediatric AVMs remains controversial. Children with intracranial AVMs represent a special challenge in that they harbor unacceptable lifelong risks of hemorrhage and potential neurologic deficits. Treatment of these lesions has evolved during the last century with advances in the medical, surgical, and technological fronts. Treatment of pediatric AVMs should be undertaken in a multidisciplinary fashion, and patients should be evaluated on a case-by-case basis to

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