Pediatric dural arteriovenous malformations

Abstract

<jats:p>Pediatric dural arteriovenous malformations (dAVMs) are rare lesions that have a high mortality rate and require complex management. The authors report 3 cases of pediatric dAVMs that presented with macrocrania and extracranial venous distension. Dural sinus thrombosis developed in 2 of the cases prior to any intervention, which is an unusual occurrence for this particular disease. All 3 cases were treated using staged endovascular embolization with a favorable outcome in 1 case and a poor outcome in the other 2 cases. Complications developed in all cases and included dural sinus thrombosis, parenchymal hemorrhage, intracranial venous hypertension, and seizures. The strategies and challenges used in managing these patients will be presented and discussed, along with a review of the literature. While outcomes remain poor, the authors conclude that prompt treatment with endovascular embolization provides the best results for children with these lesions. A well-established venous collateral circulation draining directly to the internal jugular veins may further improve the rate of favorable outcome after embolization.</jats:p>

Therapeutic progress in pediatric intracranial dural arteriovenous shunts: A review

“…Anticoagulation therapy for dural sinus thrombosis improves outcomes, even in the presence of intracranial hemorrhages; however, the guidelines for the management of these lesions in the context of coexisting infantile dAVSs are unclear. 24…”

“…Pediatric intracranial dAVS exhibits a more aggressive clinical course than adult dAVS, and the reported mortality rate is > 25%; 23 however, in general, pediatric dAVS is often stable for a period of time and they are associated with well-tolerated congestive heart failure, until the dAVS regrows or the venous outlet is occluded by thrombosis. 24 At the moment the hemodynamic equilibrium is broken, new symptoms or signs appear. 14 In contrast to acquired dAVS in adults, these congenital pediatric dAVS have a very small chance of causing spontaneous occlusion; and very few cases of ADAVS have been reported in the cavernous sinus .…”

“…Furthermore, some dAVSs occur far from the cavernous sinus; however, when the draining vein or sinus is occluded by stenosis or thrombosis, the cavernous sinus may be captured, and symptoms such as facial venous distension and pulsatile proptosis may appear. 24,40 Other symptoms…”

“…Many complications, such as intracerebral hemorrhages, can be encountered during the treatment of IDAVSs. For example: In 2014, Appaduray et al 24 reported on three pediatric IDAVS treatments. Following the embolization of the fistulae via the transarterial approach, intracerebral hemorrhages occurred in two of those patients.…”

“…Anticoagulation therapy for dural sinus thrombosis improves outcomes, even in the presence of intracranial hemorrhages; however, the guidelines for the management of these lesions in the context of coexisting infantile dAVSs are unclear. 24 In IDAVS, the sinus sump effect creates a pial AVS. These shunts are asymptomatic and may regress, following the occlusion of the primary dAVSs.…”