Spinal juvenile (Type III) extradural-intradural arteriovenous malformations

Abstract

Object Owing to their rarity, demographics, natural history, and treatment, results for spinal juvenile (Type III) extradural-intradural arteriovenous malformations (AVMs) are frequently only provided in case report format. Methods A pooled analysis was performed utilizing the PubMed database through April 2013. Individualized patient data were extracted to elucidate demographics, hemorrhage risk, and treatment result information. Results Twenty-nine studies describing 51 patients were included. The mean age at presentation was 15.0 ± 10.5 years with a slight male predilection (63%, 1.7:1 sex ratio). Presentation modality included progressive deficits in 35%, hemorrhage in 31%, acute deficits not attributed to hemorrhage in 22%, and asymptomatic/incidental in 12% of patients. The annual hemorrhage rate was 2.1%; statistically significant risk factors for hemorrhage included presentation age (HR 0.39 [95% CI 0.18–0.87]) and associated aneurysms (HR 8.74 [95% CI 1.76–43.31]). Seventy-seven percent of patients underwent treatment; after a mean follow-up of 2.6 ± 3.2 years, 73% were improved, 10% were the same, and 17% were worse neurologically. Of 25 cases with described angiographic results, 8 lesions were obliterated (32%). Of these 25 patients, 8 had AVMs with associated aneurysms, and the aneurysm was obliterated in all 8 patients. Over the course of 57.9 patient-years of follow-up, including 55.3 patient-years for partially treated AVMs, no hemorrhages were described, reflecting a trend toward protection from hemorrhage after treatment (p = 0.12, likelihood ratio test). Conclusions Spinal juvenile (Type III) extradural-intradural AVMs commonly present symptomatically. Associated arterial aneurysms increase their hemorrhage risk, and protection from hemorrhage may be achieved from partial obliteration of these lesions, particularly if targeted toward associated aneurysms.

Spinal Arteriovenous Malformation: Case Report and Review of the Literature

“…Early suspicion, workup, diagnosis, and management could have resulted in the improved neurological outcome in this case. As with other compressive spinal cord pathology, such as a tumor, progressive deficits can become permanent [11]. Any further delay in the treatment of this patient may have led to further ascending deficits and possibly death from the expanding AVM and possible extensive rupture [10].…”

“…Intradural-extradural spinal AVMs, also referred to as Juvenile vascular malformations, is a rare, complex, and locally aggressive lesion which can occupy bone, muscle, dura, spinal cord, and nerve roots [10]. This lesion commonly presents within younger patients, leading to an average age of 15.0 years (SD of 10.5) in one study of 51 patients [11]. While type III AVMs can present similarly to type II, with acute hemorrhages and myelopathy, they can be differentiated with the presence of signs of local tissue involvement.…”

“…One example is the presence of spinal instability due to vertebral body involvement and degeneration after AVM hemorrhage [12]. The likelihood of type III AVMs having multiple feeding vessels makes jeopardizes the patient's surgical candidacy; therefore, the main method of treatment is embolization for symptomatic relief and hemorrhage protection [11].…”

Classifications of spinal arteriovenous malformations. Review

“…Частота кровоизлияний, связанных со СпАВМ III типа, составляет 2,1 % в год. У пациентов с данным типом поражения после лечения наступает улучшение в 73 % случаев, динамика отсутствует -в 10 %, ухудшение неврологических симптомов происходит в 17 % [16]. Частота кровоизлияний у пациентов со СпАВМ IV типа составляет 2,5 % в год.…”

Spinal cord arteriovenous malformations: a practical guide

“…These exceptionally rare and complex lesions, also called juvenile, metameric or type III malformations, present highly variable arterial and venous support and, even though they are usually intramedullary, they may have extramedullary and paraspinal extension, involving vertebrae, spinal nerve roots, soft tissues and skin. 12 Among all the SCAVMs, they present at the youngest age, have the highest rate of associated aneurysms (almost 50% of patients), which represent an additional risk factor for hemorrhage onset, 13 and are the most difficult to treat, given their complex nature. Endovascular embolization and surgery can be used and, although complete obliterations have been reported, the primary aim of intervention is usually palliative.…”