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OBJECT Pediatric cerebral arteriovenous fistulas (AVFs) are rare but potentially lethal vascular lesions. Management strategies for these lesions have undergone considerable evolution in the last decade with the advent of new endovascular, surgical, and radiosurgical technologies. This study sought to review current treatment strategies and long-term clinical outcomes at a high-volume cerebrovascular institute. METHODS A retrospective chart review was performed on patients with a diagnosis of cerebral AVF from 1999 to 2012. Patients with carotid-cavernous fistulas, vein of Galen malformations, and age > 18 years were excluded from final analysis. Medical history, surgical and nonsurgical treatment, and clinical outcomes were documented. Pre- and postoperative angiograms were analyzed to assess for obliteration of the fistula. RESULTS Seventeen patients with pial AVFs (29.4%), dural AVFs (64.7%), or mixed pial/dural AVFs (5.9%) were identified. The majority of lesions were paramedian (70.6%) and supratentorial (76.5%). The study population had a mean age of 6.4 years, with a slight male predominance (52.9%), and the most common presenting symptoms were seizures (23.5%), headaches (17.6%), congestive heart failure (11.7%), and enlarging head circumference (11.7%). Among patients who underwent intervention (n = 16), 56.3% were treated with endovascular therapy alone, 6.3% were treated with open surgery alone, and 37.5% required a multimodal approach. Overall, 93.8% of the treated patients received endovascular treatment, 43.8% received open surgery, and 12.5% received radiosurgery. Endovascular embolysates included Onyx (n = 5), N-butyl cyanoacrylate (NBCA; n = 4), or coil embolization (n = 7) with or without balloon assistance (n = 2). Complete angiographic obliteration was achieved in 87.5% at the last follow-up evaluation (mean follow-up 3.1 years). One infant with incomplete AVF obliteration died of congestive heart failure, and 1 patient with complete obliteration died of acute sinus thrombosis, with an overall complication rate of 18.8%. CONCLUSIONS Pediatric cerebral AVFs are challenging neurosurgical lesions. Although advancements in endovascular therapy in the last decade have greatly changed the natural course of this disease, a multidisciplinary approach remains necessary for a large subset of patients. Surgeon experience with a thorough analysis of preoperative imaging is paramount to achieving acceptable clinical outcomes.

Pipeline Embolization Devices (PEDs) have been shown to be effective for intracranial internal carotid artery (ICA) aneurysms, and are now approved by the FDA specifically for this use. Potential pitfalls, however, have not yet been described in the pediatric neurosurgical literature. The authors report on a 10-year-old boy who presented to the Barrow Neurological Institute after progressive visual decline. He had undergone placement of a total of 7 telescoping PEDs at another facility for a large ICA aneurysm. Residual filling of the aneurysm and significant expansion of intraaneurysmal thrombus with chiasmal compression on admission images were causes for concern. The patient underwent a surgical bailout with a superficial temporal artery-middle cerebral artery bypass, with parent artery occlusion. Postoperative vascular imaging was notable for successful occlusion of the parent vessel, with no evidence of filling of the aneurysm. Reports on the pitfalls of PEDs in the neurosurgical literature are scarce. To the authors' knowledge this represents the first paper describing a successful open surgical bailout for residual aneurysmal filling and expansion of thrombus after placement of a PED.

Object: Aneurysms are relatively rare in the pediatric population and tend to include a greater proportion of large and giant lesions. A subset of these large and giant aneurysms are not amenable to direct surgical clipping and require complex treatment strategies and revascularization techniques. There are limited data available on the management of these lesions in the pediatric population. This study was undertaken to evaluate the outcome of treatment of large and giant aneurysms that required microsurgical revascularization and vessel sacrifice in this population., Methods: The authors retrospectively identified all cases in which pediatric patients (age < 18 years) with aneurysms were treated using cerebral revascularization in combination with other treatment modalities at their institution between 1989 and 2013., Results: The authors identified 27 consecutive patients (19 male and 8 female) with 29 aneurysms. The mean age of the patients at the time of treatment was 11.5 years (median 13 years, range 1-17 years). Five patients presented with subarachnoid hemorrhage, 11 with symptoms related to mass effect, 2 with stroke, and 3 with seizures; in 6 cases, the aneurysms were incidental findings. Aneurysms were located along the internal carotid artery (n = 7), posterior cerebral artery (PCA) (n = 2), anterior cerebral artery (n = 2), middle cerebral artery (MCA) (n = 14), basilar artery (n = 2), vertebral artery (n = 1), and at the vertebrobasilar junction (n = 1). Thirteen were giant aneurysms (45%). The majority of the aneurysms were fusiform (n = 19, 66%), followed by saccular (n = 10, 34%). Three cases were previously treated using microsurgery (n = 2) or an endovascular procedure (n = 1). A total of 28 revascularization procedures were performed, including superficial temporal artery (STA) to MCA (n = 6), STA to PCA (n = 1), occipital artery to PCA (n = 1), extracranial-intracranial (EC-IC) bypass using radial artery graft (n = 3), EC-IC using a saphenous vein graft (n = 7), STA onlay (n = 3), end-to-end anastomosis (n = 1), and in situ bypasses (n = 6). Perioperative stroke occurred in 4 patients, but only one remained dependent (Glasgow Outcome Scale [GOS] score 3). At a mean clinical follow-up of 46 months (median 14 months, range 1-232 months), 26 patients had a good outcome (GOS score 4 or 5). There were no deaths. Five patients had documented occlusion of the bypass graft. The majority of aneurysms (n = 24) were obliterated at last follow-up. There was a single case of a residual aneurysm and one case of recurrence. Angiographic follow-up was unavailable in 3 cases., Conclusions: Cerebral revascularization remains an essential tool in the treatment of complex cerebral aneurysms in children.

The authors report the case of a 7-year-old boy with headaches, in whom CT angiography showed multiple intracranial aneurysms from the terminus region of the right internal carotid artery through the proximal right middle cerebral artery (MCA). Initially, the patient underwent clip reconstruction of the M(1) segment. Multiple microsurgical and endovascular treatments were required because the largest of these aneurysms recurred several times over the next 1.5 years. The first recurrence was treated with stent coiling and the second by microsurgical occlusion of the MCA combined with the use of a radial artery graft in a common carotid artery-to-MCA bypass. The aneurysm again recurred and was treated by proximal coil occlusion 15 months after the first treatment session. At a 7.5-year follow-up examination, the aneurysms remained occluded. This case highlights the benefit of combined endovascular and microsurgical techniques in the treatment of a complex and unusual case of proximal MCA aneurysmal disease. The patient, now 15 years old, is neurologically intact and able to participate in all activities.

Object: Craniofacial approaches provide excellent exposure to lesions in the anterior and middle cranial fossae. The authors review their experience with craniofacial approaches for resection of large juvenile nasopharyngeal angiofibromas., Methods: Between 1992 and 2009, 22 patients (all male, mean age 15 years, range 9-27 years) underwent 30 procedures. These cases were reviewed retrospectively., Results: Gross-total resection of 17 (77%) of the 22 lesions was achieved. The average duration of hospitalization was 8.2 days (range 3-20 days). The rate of recurrence and/or progression was 4 (18%) of 22, with recurrences occurring a mean of 21 months after the first resection. All patients underwent preoperative embolization. Nine patients (41%) developed complications, the most common of which was CSF leakage (23%). The average follow-up was 27.7 months (range 2-144 months). The surgery-related mortality rate was 0%. Based on their mean preoperative (90) and postoperative (90) Karnofsky Performance Scale scores, 100% of patients improved or remained the same., Conclusions: The authors' experience shows that craniofacial approaches provide an excellent avenue for the resection of large juvenile nasopharyngeal angiofibromas, with acceptable rates of morbidity and no deaths.

Object: Pial arteriovenous fistulas (AVFs) of the brain are rare vascular malformations associated with significant risks of hemorrhage and neurological deficit. Depending on their location and high-flow dynamics, these lesions can present treatment challenges for both endovascular and open cerebrovascular surgeons. The authors describe a novel endovascular treatment strategy that was used successfully to treat 2 pediatric patients with a pial AVF, and they discuss the technical nuances specific to their treatment strategy., Methods: A single-channel high-flow pial AVF was diagnosed in 2 male patients (6 and 17 years of age). Both patients were treated with endovascular flow arrest using a highly conformable balloon followed by Onyx infusion for definitive closure of the fistula., Results: Neither patient suffered a complication as a result of the procedure. At the 6-month follow-up in both cases, the simple discontinuation of blood flow had resulted in durable obliteration of the fistula and stable or improved neurological function., Conclusions: Onyx can be delivered successfully into high-flow lesions after flow arrest to allow a minimally invasive and durable treatment for pial AVFs.

Cavernous malformations (CMs) or hemangiomas arising from within the dural sinuses are rare entities that differ from their parenchymal counterparts in that they are highly vascular lesions. While parenchymal CMs are typically angiographically occult, intrasinus malformations may have large, dural-based arterial feeding vessels that are amenable to preoperative embolization. The novel liquid embolic Onyx (ev3, Inc.) is an ideal agent for the embolization of these lesions. The authors present the first known case of a giant intrasinus CM embolized with Onyx before gross-total resection. The authors report the case of a 9-year-old boy with brief apneic episodes in whom MR imaging revealed a giant CM arising from within the right transverse and sigmoid sinuses and infiltrating the right tentorium cerebelli. At another institution, the patient had undergone 1 prior embolization and 2 unsuccessful attempts at resection. Both surgeries had been complicated by massive blood loss and were aborted. Under the authors' care, the patient underwent preoperative transarterial embolization with Onyx during which a substantial volume of the mass lesion was filled with embolisate. Subsequently, complete circumferential excision of the mass from the tentorium was accomplished with minimal intraoperative blood loss.