Management of PHACES syndrome: Risk of stroke and its prevention from a neurosurgical perspective

BACKGROUND AND OBJECTIVES A multidisciplinary approach for PHACES is essential. A meticulous diagnostic and treatment protocol for PHACES patients with cerebrovascular anomalies within the intermediate and high risk strata for ischemic stroke is presented. We also differentiate the vasculopathy associated with PHACES syndrome from moyamoya angiopathy. METHODS Medical records and radiological imaging were reviewed. After initial magnetic resonance imaging/angiography (MRI/MRA), H$_{2}$$^{15}$O-PET scan (baseline and Acetazolamide challenge) was performed in three patients and 6-vessel cerebral angiography was performed in two patients. Two patients with significant intracranial cerebrovascular anomalies underwent cerebral revascularization. RESULTS Each patient presented with a facial hemangioma at birth and additional cerebrovascular anomalies ranging from hypoplasia to steno-occlusive changes of intracranial cerebral arteries. Additional involvement of the cardiovascular system was observed in two patients. Additional to MRI/MRA, a H$_{2}$$^{15}$O-PET helped stratify the three patients into intermediate (n=1) and high risk groups (n=2). The high-risk group patients underwent individualized cerebral revascularization for future stroke prevention. The patient in intermediate risk group will be followed. Cerebrovascular angiopathy seen in all patients was typical for PHACES without moyamoya and was not progressive at follow-up. CONCLUSIONS Patients within the intermediate and high-risk strata for ischemic stroke must undergo a 6-vessel cerebral angiography and further hemodynamic evaluation to indicate need for cerebral revascularization to prevent ischemic stroke. Non-progressive vasculopathy associated with PHACES can itself be hemodynamically relevant for neurosurgical intervention. This vasculopathy is distinct from moyamoya angiopathy, which can occur in conjunction with PHACES, resulting in concurrent progressive vasculopathy that would otherwise be absent

Estudo da Sobrevida dos Doentes com o Diagnóstico de Glioblastoma Multiforme Operados no Hospital Garcia de Orta

O glioblastoma multiforme (GBM) é o tumor primário do sistema nervoso central mais frequente nos adultos, estimando-se uma incidência de 5,26/10^5, superior na 6ª e 7ª décadas de vida. Apesar de constituírem apenas cerca de 2% de todos os tumores, estão associados a uma elevada morbilidade e mortalidade, não existindo dados sobre a sobrevida destes doentes na população portuguesa. Objectivo: Este estudo teve como objectivo estimar a sobrevida dos casos de GBM diagnosticados no Hospital Garcia de Orta entre 2009 e 2013. Realizou-se um estudo de seguimento retrospectivo usando informação registada (até março de 2015) no processo clínico dos doentes com diagnóstico de GBM, entre janeiro de 2009 e dezembro de 2013, no serviço de neurocirurgia do Hospital Garcia de Orta. Caracterizaram-se os doentes de acordo com sexo, idade, manifestação clínica inicial, tratamento cirúrgico e evolução índice de Karnofsky (KPS). Usando o método de Kaplan-Meier, estimou-se o tempo médio de sobrevida (e intervalo de confiança a 95 %) após o diagnóstico de acordo com o tratamento cirúrgico efectuado, procedendo-se à sua comparação através da prova de Mantel-Haenszel. Foram identificados 155 doentes, 51 % (79) do sexo masculino, com idade média de 62,8. Os déficites focais (35,3 %), cefaleia (16,5 %) e alterações cognitivas/mnésicas (15,1%) foram as manifestações clínicas inaugurais mais frequentes. Em relação ao tratamento cirúrgico 31,9 % (49) dos doentes apenas realizaram biopsia e 22,1% (34) fizeram remoção total. O índice de KPS à data da alta foi de 72,8; 45,8 aos 3 meses; 36,2 aos 6 meses e 27,4 aos 12 meses. O tempo médio de sobrevida foi de 9,6 meses (IC95: 5,1 a 8,2 meses) com uma mediana de 6,7 meses, observando-se menor sobrevida quando efectuada apenas biopsia (5,5 meses, IC 95: 2,2 a 4,9 meses) e maior tempo de sobrevida quando feita remoção total (16,1 meses; IC 95: 12,8 a 18,9 meses). A sobrevida dos doentes com GBM operados no Hospital Garcia de Orta é sobreponível ao registado na literatura. Verifica-se uma correlação positiva entre o grau de remoção cirúrgica e a sobrevida total, como descrito noutras séries

Variations in management of A3 and A4 cervical spine fractures as designated by the AO Spine Subaxial Injury Classification System

© 2022 The authors.OBJECTIVE Optimal management of A3 and A4 cervical spine fractures, as defined by the AO Spine Subaxial Injury Classification System, remains controversial. The objectives of this study were to determine whether significant management variations exist with respect to 1) fracture location across the upper, middle, and lower subaxial cervical spine and 2) geographic region, experience, or specialty. METHODS A survey was internationally distributed to 272 AO Spine members across six geographic regions (North America, South America, Europe, Africa, Asia, and the Middle East). Participants’ management of A3 and A4 subaxial cervical fractures across cervical regions was assessed in four clinical scenarios. Key characteristics considered in the vignettes included degree of neurological deficit, pain severity, cervical spine stability, presence of comorbidities, and fitness for surgery. Respondents were also directly asked about their preferences for operative management and misalignment acceptance across the subaxial cervical spine. RESULTS In total, 155 (57.0%) participants completed the survey. Pooled analysis demonstrated that surgeons were more likely to offer operative intervention for both A3 (p < 0.001) and A4 (p < 0.001) fractures located at the cervicothoracic junction compared with fractures at the upper or middle subaxial cervical regions. There were no significant variations in management for junctional incomplete (p = 0.116) or complete (p = 0.342) burst fractures between geographic regions. Surgeons with more than 10 years of experience were more likely to operatively manage A3 (p < 0.001) and A4 (p < 0.001) fractures than their younger counterparts. Neurosurgeons were more likely to offer surgical stabilization of A3 (p < 0.001) and A4 (p < 0.001) fractures than their orthopedic colleagues. Clinicians from both specialties agreed regarding their preference for fixation of lower junctional A3 (p = 0.866) and A4 (p = 0.368) fractures. Overall, surgical fixation was recommended more often for A4 than A3 fractures in all four scenarios (p < 0.001). CONCLUSIONS The subaxial cervical spine should not be considered a single unified entity. Both A3 and A4 fracture subtypes were more likely to be surgically managed at the cervicothoracic junction than the upper or middle subaxial cervical regions. The authors also determined that treatment strategies for A3 and A4 subaxial cervical spine fractures varied significantly, with the latter demonstrating a greater likelihood of operative management. These findings should be reflected in future subaxial cervical spine trauma algorithms.N

An international validation of the AO spine subaxial injury classification system

Purpose To validate the AO Spine Subaxial Injury Classification System with participants of various experience levels, subspecialties, and geographic regions. Methods A live webinar was organized in 2020 for validation of the AO Spine Subaxial Injury Classification System. The validation consisted of 41 unique subaxial cervical spine injuries with associated computed tomography scans and key images. Intraobserver reproducibility and interobserver reliability of the AO Spine Subaxial Injury Classification System were calculated for injury morphology, injury subtype, and facet injury. The reliability and reproducibility of the classification system were categorized as slight (? = 0-0.20), fair (? = 0.21-0.40), moderate (? = 0.41-0.60), substantial (? = 0.61-0.80), or excellent (? = > 0.80) as determined by the Landis and Koch classification. Results A total of 203 AO Spine members participated in the AO Spine Subaxial Injury Classification System validation. The percent of participants accurately classifying each injury was over 90% for fracture morphology and fracture subtype on both assessments. The interobserver reliability for fracture morphology was excellent (? = 0.87), while fracture subtype (? = 0.80) and facet injury were substantial (? = 0.74). The intraobserver reproducibility for fracture morphology and subtype were excellent (? = 0.85, 0.88, respectively), while reproducibility for facet injuries was substantial (? = 0.76). Conclusion The AO Spine Subaxial Injury Classification System demonstrated excellent interobserver reliability and intraobserver reproducibility for fracture morphology, substantial reliability and reproducibility for facet injuries, and excellent reproducibility with substantial reliability for injury subtype