A Systematic Review of Non-Galenic Pial Arteriovenous Fistulas

Lim, Jaims; Kuo, Cathleen C.; Waqas, M.; Cappuzzo, Justin M; Monteiro, André; Baig, Ammad A; Snyder, Kenneth V.; Davies, Jason M.; Levy, Elad I.; Siddiqui, Adnan H.

doi:10.1016/j.wneu.2022.09.007

Cited by 5 publications

(2 citation statements)

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“…Common data elements reduce bias associated with missing results. This shortcoming is exaggerated in similar reviews of rare pathologies in the neurosurgical literature that by nature involve small sample size [ 6 ]. Another limitation is publication bias intrinsic to systematic reviews.…”

Section: Discussionmentioning

confidence: 99%

“…The majority of diagnoses occur either shortly after birth due to heart failure or cerebral venous congestion/neurologic decline or following spontaneous intracranial hemorrhage later in life [ 5 ]. The molecular pathophysiology underlying pAVF formation in utero is not well characterized, but thought to involve perturbations in angiogenic growth factors and embryonic vascular morphogenesis [ 6 – 8 ]. The most frequently associated genetic mutations involve the hereditary hemorrhagic telangiectasia (HHT) genes (ENG, ACVRL and SMAD4) and RASA1 -- which are also implicated in brain AVM and VOGM, respectively [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

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Pediatric non-galenic pial arteriovenous fistula’s characteristics and outcomes: a systematic review

Thrash,

Hale,

Feldman

et al. 2024

Childs Nerv Syst

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Introduction Pediatric non-galenic pial arteriovenous fistulas (pAVFs) are rare vascular malformations that are characterized by a pial arterial-venous connection without an intervening capillary bed. Outcomes and treatment strategies for pAVFs are highly individualized, owing to the rarity of the disease and lack of large-scale data guiding optimal treatment approaches. Methods We performed a systematic review of pediatric patients (< 18 years at diagnosis) diagnosed with a pAVF by digital subtraction angiogram (DSA). The demographics, treatment modalities, and outcomes were documented for each patient and clinical outcome data was collected. Descriptive information stratified by outcome scores were classified as follows: 1 = excellent (no deficit and full premorbid activity), 2 = good (mild deficit and full premorbid activity), 3 = fair (moderate deficit and impaired activity), 4 = poor (severe deficit and dependent on others), 5 = death. Results A total of 87 studies involving 231 patients were identified. Median age at diagnosis was 3 years (neonates to 18 years). There was slight male preponderance (55.4%), and 150 subjects (81.1%*) experienced excellent outcomes after treatment. Of the 189 patients treated using endovascular approaches, 80.3% experienced excellent outcomes and of the 15 patients surgically treated subjects 75% had an excellent outcome. The highest rate of excellent outcomes was achieved in patients treated with Onyx (95.2%) and other forms of EvOH (100%). High output heart failure and comorbid vascular lesions tended to result in worse outcomes, with only 54.2% and 68% of subjects experiencing an excellent outcome, respectively. *Outcomes were reported in only 185 patients. Conclusion pAVFs are rare lesions, necessitating aggregation of patient data to inform natural history and optimal treatment strategies. This review summarizes the current literature on pAVF in children, where children presenting with heart failure as a result of high flow through the lesion were less likely to experience an excellent outcome. Prospective, large-scale studies would further characterize pediatric pAVFs and enable quantitative analysis of outcomes to inform best treatment practices.

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Section: Discussionmentioning

confidence: 99%