An institutional series and literature review of pial arteriovenous fistulas in the pediatric population

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Pial arteriovenous fistulas (PAVFs) are a rare form of cerebrovascular disease that tend to be overrepresented in the pediatric population. There have been limited studies of the clinical features and outcomes in this group of patients. Here, the authors attempt to better delineate this clinical entity with institutional cases and a review of the literature.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>A retrospective review of cases at our institution was performed to identify all pediatric patients treated for a PAVF between 2000 and 2012.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>Five patients treated for a PAVF were identified. Patients had a mean age of 1.9 years at diagnosis, and the most common presenting symptoms were seizure and macrocephaly. Patients were treated primarily with embolization, and 3 patients required both <jats:italic>N</jats:italic>-butyl cyanoacrylate (NBCA) glue and coiling. Four of the patients had complete obliteration of the PAVF and had a pediatric overall performance category score of either 1 (n = 3) or 2 (n = 1) at follow-up. There was 1 death due to heart failure. Analysis of the literature review suggested that a younger age or presence of intracerebral hemorrhage (ICH) or congestive heart failure (CHF) at presentation likely predicts a worse prognosis. Older patients presented more often with ICH, whereas younger patients presented significantly more often in CHF. The majority of pediatric patients reported on in the literature were treated with endovascular embolization, most commonly with NBCA glue alone. Most patients (65.4%) in the literature had an excellent outcome without neurological deficit.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Pial AVFs represent a serious yet rare form of cerebrovascular disease. Pediatric patients with ICH or CHF at presentation or those who are very young are likely to have a worse prognosis. Endovascular management of these patients has greatly changed the natural history of this disease, but the complication and mortality rates suggest the need for continued insights and advances in treatment.</jats:p></jats:sec>

Treatment of a cerebral pial arteriovenous fistula in a patient with sickle cell disease-related moyamoya syndrome: case report

“…The vast majority of prior AVMs in patients with moyamoya have been treated via an endovascular approach for this reason. 22 Very few cases of pAVFs embolized with Onyx have been reported in literature. 37 Some authors have advised against using nonadherent embolic agents, such as Onyx, to treat fistulous lesions because Onyx utilization requires more fluoroscopic time in children and the potentially large doses required for embolization of these fistulas increase the risk for dimethyl sulfoxide-related pulmonary toxicity associated with Onyx, which may exacerbate the preexisting cardiovascular and pulmonary comorbidities common in this group of children.…”

“…37 Some authors have advised against using nonadherent embolic agents, such as Onyx, to treat fistulous lesions because Onyx utilization requires more fluoroscopic time in children and the potentially large doses required for embolization of these fistulas increase the risk for dimethyl sulfoxide-related pulmonary toxicity associated with Onyx, which may exacerbate the preexisting cardiovascular and pulmonary comorbidities common in this group of children. 22 Newman et al, published the first successful balloon-assisted Onyx embolization of a giant, high-flow pAVF, noticing the increased ability to control the delivery of Onyx, as compared with NBCA glue, allowing more precise targeting and delivery of embolic material to the fistulous point. 27 In our case, we were able to specifically target the fistula with no reflux into the MCA, which was only a few millimeters away.…”

“…9,10,28 The majority of pediatric patients with pAVFs described in the literature have been treated with endovascular embolization 22,37 Furthermore, in the setting of moyamoya arteriopathy and SCD, where cerebral vascularization is dependent on collaterals arising from external carotid scalp and dural arteries, a craniotomy would carry a high risk of cerebral ischemia. The vast majority of prior AVMs in patients with moyamoya have been treated via an endovascular approach for this reason.…”

Effectiveness of intraoperative indocyanine green videoangiography in direct surgical treatment of pediatric intracranial pial arteriovenous fistula

“…3,8,18 The goal of treatment is to obliterate the arteriovenous shunt, with preservation of the normal arterial supply and venous drainage of the normal brain tissue. However, the identification of the fistula point is occasionally obscured on digital subtraction angiography.…”

Multimodal treatment strategies for complex pediatric cerebral arteriovenous fistulas: contemporary case series at Barrow Neurological Institute

“…6,10 A review of modern treatment options and clinical outcomes for pediatric cerebral AVFs is lacking, with recent articles often consolidating a multidecade experience 8,20 or showing heavy bias toward endovascular therapy alone. 12,18,19 We sought to review the treatment methodology and clinical outcomes of our institution's multidisciplinary approach to treating these complex cerebrovascular lesions in the modern era.…”