Paediatric intracranial dural arteriovenous shunts: types, clinical presentation and therapeutic management

Abstract

Paediatric intracranial dural arterio-venous shunts have clinical presentations and evolutions, with angiographic characteristics that differ from those described in adults. We report our experience concerning their therapeutic management, emphasizing the relevance of early diagnosis and appropriate treatment for satisfactory neurocognitive development. Using a prospective database, we reviewed the clinical and radiological data of all children with dural arteriovenous shunts managed between 2002 and 2020. Dural shunts were categorized into three types: dural sinus malformations with arteriovenous shunts; infantile dural arteriovenous shunts; adult-type dural arteriovenous shunts. Therapeutic strategies and outcomes were analyzed depending on lesional subtypes. Modified Rankin Scale for the paediatric population was assessed pre-treatment and at last follow-up. Twenty-eight patients (16 girls [57.1%]; 12 boys [42.9%]) were included: 17 dural sinus malformation (10 boys [58.8%]; seven girls [41.2%]), three infantile shunts (three girls [100%]), eight adult-type shunts (four girls [50%]; four boys [50%]), with a mean age of 19.2±36.6 months at presentation. Twelve (42.9%) had a modified Rankin Scale score of 0-2, four (14.3%) a score of 3, three (10.7%) a score of 4 and eight (28.6%) a score of 5. Embolization was performed in 22 children (78.6%; 12 girls [54.5%]; 10 boys [45.5%]). Fifteen patients could be cured (68.2%): 11 dural sinus malformations (73.3%), four adult-type lesions (100%), but no infantile shunt. Mean post-treatment follow-up was 39.5 months (max. 139 months): 14 patients (63.6%) presented a modified Rankin Scale score 0-2 and eight (36.4%) a score ≥3. In the dural sinus malformation group, the modified Rankin Scale score was improved in 11 patients (73.3%) and unchanged in three (20%). Only one patient with infantile subtype (33.3%) improved clinically. In the adult-subtype group, all children (100%) improved. Of six untreated patients (four girls [66.7%]; two boys [33.3%]), four with adult-subtype shunts showed uneventful evolutions, one with dural sinus malformation died, and therapeutic abortion was conducted in an antenatally diagnosed dural sinus malformation. Pediatric dural fistulas comprise different subtypes with variable clinical courses. Proper diagnosis is mandatory for optimal therapeutic strategies within appropriate therapeutic windows.

Anticoagulation helps shrink giant venous lakes and arteriovenous fistulas in dural sinus malformation

“…Finally, as we saw a decrease in the size of the giant venous lakes and remaining AV shunts, anticoagulation makes the management of embolization easier, in that it was not necessary to target small AV shunts in the dura or pial AV shunts. Compared with other case series in the literature, our technique required fewer embolization sessions,1–7 and we targeted only the largest dural AV shunts that are usually easy to catheterize without compromising the venous network. As stated above, we did not perform embolization of the pial network that could be involved in feeding the malformation but would be at higher risk of intraoperative cerebral complication, either hemorrhagic or ischemic.…”

“…Nevertheless, partial embolization as well as sinovenous thrombosis can lead to an uncontrolled neoangiogenic reaction, which can worsen the already difficult to manage situation. However, recent publications7 have shown some improvement in the outcome of these patients compared with earlier published results, especially when combining embolization with anticoagulation 8…”

Remodeling of venous drainage after the treatment of dural sinus malformation with arteriovenous shunts in a neonate: a case report and literature review

“…DSM is a rare subtype of the pediatric dural arteriovenous stula accounting for 1.9% of all intracranial arteriovenous malformations and 10% of pediatric arteriovenous stulas [2][3][4]. DSM involving the torcular Herophili accounted for 46.6% of all DSM cases [2].…”

“…The transarterial embolization is often preferred because venous pouch often functions as normal venous drain [8], thus, AV shunts must be reduced while preserving the patency of venous drainage [2]. If the venous pouch is not involved in normal venous drainage, coil embolization in the venous pouch can effectively [3,8]. The ow reduction of DSM could lead to thrombosis, venous pouch shrinking, and dural sinus remodeling.…”

Multimodal MRI diagnosis and transvenous embolization of a basicranial emissary vein dural arteriovenous fistula: A case report

“… 1 , 2 DAVFs may manifest as either benign or aggressive, based on venous drainage patterns, while cortical venous reflux can potentially result in intracranial hemorrhage, neurological damage, or even death. 3 , 4 The association between venous reflux patterns and aggressive intracranial lesions has been well established in cranial DAVFs. 5 …”