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Steven W. Hetts, Parham Moftakhar, Neil Maluste, Heather J. Fullerton, Daniel L. Cooke, Matthew R. Amans, Christopher F. Dowd, Randall T. Higashida and Van V. Halbach

OBJECTIVE

Intracranial dural arteriovenous fistulas (DAVFs) are rare in children. This study sought to better characterize DAVF presentation, angioarchitecture, and treatment outcomes.

METHODS

Children with intracranial DAVFs between 1986 and 2013 were retrospectively identified from the neurointerventional database at the authors' institution. Demographics, clinical presentation, lesion angioarchitecture, treatment approaches, angiographic outcomes, and clinical outcomes were assessed.

RESULTS

DAVFs constituted 5.7% (22/423) of pediatric intracranial arteriovenous shunting lesions. Twelve boys and 10 girls presented between 1 day and 18 years of age; boys presented at a median of 1.3 years and girls presented at a median of 4.9 years. Four of 8 patients ≤ 1 year of age presented with congestive heart failure compared with 0/14 patients > 1 year of age (p = 0.01). Five of 8 patients ≤ 1 year old presented with respiratory distress compared with 0/14 patients > 1 year old (p = 0.0021). Ten of 14 patients > 1 year old presented with focal neurological deficits compared with 0/8 patients ≤ 1 year old (p = 0.0017). At initial angiography, 16 patients harbored a single intracranial DAVF and 6 patients had 2–6 DAVFs. Eight patients (38%) experienced DAVF obliteration by the end of treatment. Good clinical outcome (modified Rankin Scale score 0–2) was documented in 77% of patients > 1 year old at presentation compared with 57% of patients ≤ 1 year old at presentation. Six patients (27%) died.

CONCLUSIONS

Young children with DAVFs presented predominantly with cardiopulmonary symptoms, while older children presented with focal neurological deficits. Compared with other pediatric vascular shunts, DAVFs had lower rates of angiographic obliteration and poorer clinical outcomes.

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Fabio Settecase, Andrew D. Nicholson, Matthew R. Amans, Randall T. Higashida, Van V. Halbach, Daniel L. Cooke, Christopher F. Dowd and Steven W. Hetts

A 13-year-old boy with meningiomatosis, McCune-Albright syndrome, and gray platelet syndrome presented with an enlarging “lump” on his right forehead. A head CT scan revealed a polyostotic fibrous dysplasia involving the entire skull. A 3.4-cm right frontal osseous cavity and an overlying right forehead subcutaneous soft-tissue mass were seen, measuring 5.2 cm in diameter and 1.6 cm thick. Ultrasound of the cavity and overlying mass showed swirling of blood and an arterialized waveform. MRI revealed an en plaque meningioma underlying the cavity. An intraosseous pseudoaneurysm fed by 3 distal anterior division branches of the right middle meningeal artery (MMA) with contrast extravasation was found on angiography. Two MMA feeders were embolized with Onyx, with anterograde filling of the intraosseous cavity with Onyx. A small pocket of residual intracavity contrast filling postembolization from a smaller third MMA feeder eventually thrombosed and the forehead lump regressed.

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Jared Narvid, Matthew R. Amans, Daniel L. Cooke, Steven W. Hetts, William P. Dillon, Randall T. Higashida, Christopher F. Dowd and Van V. Halbach

OBJECT

Retroclival hematomas are rare, appearing mostly as posttraumatic phenomena in children. Spontaneous retroclival hematoma (SRH) in the absence of trauma also has few descriptions in the literature. None of the reported clinical cases features the combination of an SRH and intraventricular hemorrhage (IVH). Nevertheless, despite extensive cases of idiopathic or angiographically negative subarachnoid hemorrhage (SAH) of the posterior fossa, only a single case report of a patient with a unique spontaneous retroclival hematoma has been identified. In this study, the authors reviewed the presentation, management, and clinical outcome of this rare entity.

METHODS

The authors performed a retrospective analysis of all patients with diagnosed SRH at their institution over a 3-year period. Collected data included clinical history, laboratory results, treatment, and review of all imaging studies performed.

RESULTS

Four patients had SRH. All were appropriately evaluated for coagulopathic and/or traumatic etiologies of hemorrhage, though no etiology could be found. Moreover, all of the patients demonstrated SRH that both clearly crossed the basioccipital synchondrosis and was contained within a nondependent configuration along the retroclival dura mater.

CONCLUSIONS

Spontaneous retroclival hematoma, often associated with IVH, is a rare subtype of intracranial hemorrhage frequently recognized only when MRI demonstrates compartmentalization of the posterior fossa hemorrhage. When angiography fails to reveal an underlying lesion, SRH patients, like patients with traditional angiographically negative SAH, enjoy a remarkably good prognosis.

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Christoph J. Griessenauer, Christopher S. Ogilvy, Paul M. Foreman, Michelle H. Chua, Mark R. Harrigan, Christopher J. Stapleton, Aman B. Patel, Lucy He, Matthew R. Fusco, J Mocco, Peter A. Winkler, Apar S. Patel and Ajith J. Thomas

OBJECTIVE

Contemporary treatment for paraophthalmic artery aneurysms includes flow diversion utilizing the Pipeline Embolization Device (PED). Little is known, however, about the potential implications of the anatomical relationship of the ophthalmic artery (OA) origin and aneurysm, especially in smaller aneurysms.

METHODS

Four major academic institutions in the United States provided data on small paraophthalmic aneurysms (≤ 7 mm) that were treated with PED between 2009 and 2015. The anatomical relationship of OA origin and aneurysm, radiographic outcomes of aneurysm occlusion, and patency of the OA were assessed using digital subtraction angiography. OA origin was classified as follows: Type 1, OA separate from the aneurysm; Type 2, OA from the aneurysm neck; and Type 3, OA from the aneurysm dome. Clinical outcome was assessed using the modified Rankin Scale, and visual deficits were categorized as transient or permanent.

RESULTS

The cumulative number of small paraophthalmic aneurysms treated with PED between 2009 and 2015 at the 4 participating institutions was 69 in 52 patients (54.1 ± 13.7 years of age) with a male-to-female ratio of 1:12. The distribution of OA origin was 72.5% for Type 1, 17.4% for Type 2, and 10.1% for Type 3. Radiographic outcome at the last follow-up (median 11.5 months) was available for 54 aneurysms (78.3%) with complete, near-complete, and incomplete occlusion rates of 81.5%, 5.6%, and 12.9%, respectively. Two aneurysms (3%) resulted in transient visual deficits, and no patient experienced a permanent visual deficit. At the last follow-up, the OA was patent in 96.8% of treated aneurysms. Type 3 OA origin was associated with a lower rate of complete aneurysm occlusion (p = 0.0297), demonstrating a trend toward visual deficits (p = 0.0797) and a lower rate of OA patency (p = 0.0783).

CONCLUSIONS

Pipeline embolization treatment of small paraophthalmic aneurysms is safe and effective. An aneurysm where the OA arises from the aneurysm dome may be associated with lower rates of aneurysm occlusion, OA patency, and higher rates of transient visual deficits.

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Parham Moftakhar, Daniel L. Cooke, Heather J. Fullerton, Nerissa U. Ko, Matthew R. Amans, Jared A. Narvid, Christopher F. Dowd, Randall T. Higashida, Van V. Halbach and Steven W. Hetts

OBJECT

Although the development and prevalence of cerebral vasospasm (CV) has been extensively investigated in adults, little data exist on the development of CV in children. The authors hypothesized that even though children have highly vasoreactive arteries, because of a robust cerebral collateral blood flow, they rarely develop symptomatic CV.

METHODS

The authors retrospectively reviewed their university hospital's neurointerventional database for children (that is, patients ≤ 18 years) who were examined or treated for aneurysmal or traumatic subarachnoid hemorrhage (SAH) during the period 1990–2013. Images from digital subtraction angiography (DSA) were analyzed for the extent of CV and collateralization of the cerebral circulation. Results from transcranial Doppler (TCD) ultrasonography were correlated with those from DSA. Cerebral vasospasm on TCD ultrasonography was defined according to criteria developed for adults. Clinical outcomes of CV were assessed with the pediatric modified Rankin Scale (mRS).

RESULTS

Among 37 children (21 boys and 16 girls ranging in age from 8 months to 18 years) showing symptoms of an aneurysmal SAH (comprising 32 aneurysms and 5 traumatic pseudoaneurysms), 17 (46%) had CV confirmed by DSA; CV was mild in 21% of these children, moderate in 50%, and severe in 29%. Only 3 children exhibited symptomatic CV, all of whom had poor collateralization of cerebral vessels. Among the 14 asymptomatic children, 10 (71%) showed some degree of vessel collateralization. Among 16 children for whom TCD data were available that could be correlated with the DSA findings, 13 (81%) had CV according to TCD criteria. The sensitivity and specificity of TCD ultrasonography for diagnosing CV were 95% and 59%, respectively. The time to CV onset detected by TCD ultrasonography was 5 ± 3 days (range 2–10 days). Twenty-five (68%) of the children had good long-term outcomes (that is, had mRS scores of 0–2).

CONCLUSIONS

Children have a relatively high incidence of angiographically detectable, moderate-to-severe CV. Children rarely develop symptomatic CV and have good long-term outcomes, perhaps due to robust cerebral collateral blood flow. Criteria developed for detecting CV with TCD ultrasonography in adults overestimate the prevalence of CV in children. Larger studies are needed to define TCD ultrasonography–based CV criteria for children.

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Oral Presentations

2010 AANS Annual Meeting Philadelphia, Pennsylvania May 1–5, 2010