Therapeutic progress in pediatric intracranial dural arteriovenous shunts: A review

Abstract

Pediatric dural arteriovenous shunts (dAVSs) are a rare form of vascular disease: Fewer than 100 cases are reported in PubMed and the understanding of pediatric dAVS is limited. For this study, we searched in PubMed, reviewed and summarized the literature related to pediatric dAVSs. Our review revealed that pediatric dAVSs have an unfavorable natural history: If left untreated, the majority of pediatric dAVSs deteriorate. In a widely accepted classification scheme developed by Lasjaunias et al., pediatric dAVSs are divided into three types: Dural sinus malformation (DMS) with dAVS, infantile dAVS (IDAVS) and adult-type dAVS (ADAVS). In general, the clinical manifestations of dAVS can be summarized as having symptoms due to high-flow arteriovenous shunts, symptoms from retrograde venous drainage, symptoms from cavernous sinus involvement and hydrocephalus, among other signs and symptoms. The pediatric dAVSs may be identified with several imaging techniques; however, the gold standard is digital subtraction angiography (DSA), which indicates unique anatomical details and hemodynamic features. Effectively treating pediatric dAVS is difficult and the prognosis is often unsatisfactory. Transarterial embolization with liquid embolic agents and coils is the treatment of choice for the safe stabilization and/or improvement of the symptoms of pediatric dAVS. In some cases, transumbilical arterial and transvenous approaches have been effective, and surgical resection is also an effective alternative in some cases. Nevertheless, pediatric dAVS can have an unsatisfactory prognosis, even when timely and appropriate treatment is administered; however, with the development of embolization materials and techniques, the potential for improved treatments and prognoses is increasing.

Keywords: Angiography; brain circulation; cerebral venous drainage; dural arteriovenous shunt; dural sinus malformation; imaging; pediatrics; procedures; review; sinus thrombosis; therapy.

Figure 1.

Representative images of pediatric dAVS. (a) DSM with dAVS that involved the posterior sinus with giant dural lakes; (b) DSM with dAVS that involved the jugular bulb, other sinuses and normal sinuses that appear as a sigmoid sinus-jugular bulb ‘diaphragm’; (c) IDAVS without venous lakes or sinus malformations; (d) ADAVS in the cavernous sinus. ADAVS: adult dural arteriovenous shunt; dAVS: dural arteriovenous shunt; DSM: dural sinus malformation; IDAVS: infant dural arteriovenous shunt.

Figure 2.

Treatment protocol of pediatric dAVSs. ADAVS: adult-type dural arteriovenous shunt; CT: computed tomography; dAVS: dural arteriovenous shunt; DSM: dural sinus malformation; IDAVS: infantile dural arteriovenous shunt.