Abstract

NGAVFs are rare vascular malformations usually presenting in infancy or childhood. We sought to identify clinical and angiographic predictors of clinical outcome for these lesions. Retrospective review of a neurointerventional data base identified 386 pediatric patients with intracranial AVFs and AVMs, from which a cohort of 25 patients with NGAVF were selected for medical record and imaging analysis. NGAVFs constituted 7.3% of pediatric intracranial vascular lesions with a nondural arteriovenous shunt. Seven of 8 patients who presented in the first month of life had CHF and harbored large, complex fistulas with multiple sites of arteriovenous shunting. Single-hole fistulas predominated later in childhood and more frequently presented with seizures, hemorrhage, or focal neurologic deficits. More treatment procedures were performed in subjects presenting at ≤ 2 years of age compared with older children (median = 3 versus 2, P = .041), and in those harboring a multi-hole fistula versus those with a single-hole fistula (median = 3 versus 2, P = .003). Eighteen patients (72%) had complete posttreatment elimination of NGAVF shunting. Compared with patients presenting at >2 years of age, patients presenting in the first 2 years of life were more likely to have a multi-hole fistula (100% versus 25%, P = .0001) and to have a poor clinical outcome (54% versus 0%, P = .0052), defined as a pediatric mRS of ≥ 3. The morbidity of NGAVF appears higher than previously reported despite a somewhat higher rate of angiographic cure. Poor clinical outcome occurred primarily in patients with multi-hole NGAVFs presenting at ≤ 2 years of age.

Highlights

  • MethodsRetrospective review of a neurointerventional data base identified 386 pediatric patients with intracranial AVFs and AVMs, from which a cohort of 25 patients with NGAVF were selected for medical record and imaging analysis

  • AND PURPOSE: NGAVFs are rare vascular malformations usually presenting in infancy or childhood

  • Seven of 8 patients who presented in the first month of life had CHF and harbored large, complex fistulas with multiple sites of arteriovenous shunting

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Summary

Methods

Retrospective review of a neurointerventional data base identified 386 pediatric patients with intracranial AVFs and AVMs, from which a cohort of 25 patients with NGAVF were selected for medical record and imaging analysis. Subjects In this institutional review board–approved study, a neurointerventional radiology data base was used to identify a cohort of 386 patients Յ18 years of age who had undergone evaluation and treatment for intracranial arteriovenous shunt at University of California-San Francisco between 1986 and 2011. Imaging reports and medical records of patients with diagnoses of NGAVF, DAVF, VOGM, and AVM were reviewed. Medical Record Review Baseline variables were recorded for each subject (On-line Table 1). Dural venous sinus dilation was defined as double the expected diameter of a given dural venous sinus, based on angiograms of children of similar ages at our institution without arteriovenous shunting. Treatment and outcome variables are outlined in On-line Table 4

Results
Discussion
Conclusion

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