Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF-β expression and connective tissue features.

Fibromuscular dysplasia (FMD) is a rare, nonatherosclerotic arterial disease for which the molecular basis is unknown. We comprehensively studied 47 subjects with FMD, including physical examination, spine magnetic resonance imaging, bone densitometry, and brain magnetic resonance angiography. Inflammatory biomarkers in plasma and transforming growth factor β (TGF-β) cytokines in patient-derived dermal fibroblasts were measured by ELISA. Arterial pathology other than medial fibrodysplasia with multifocal stenosis included cerebral aneurysm, found in 12.8% of subjects. Extra-arterial pathology included low bone density (P<0.001); early onset degenerative spine disease (95.7%); increased incidence of Chiari I malformation (6.4%) and dural ectasia (42.6%); and physical examination findings of a mild connective tissue dysplasia (95.7%). Screening for mutations causing known genetically mediated arteriopathies was unrevealing. We found elevated plasma TGF-β1 (P=0.009), TGF-β2 (P=0.004) and additional inflammatory markers, and increased TGF-β1 (P=0.0009) and TGF-β2 (P=0.0001) secretion in dermal fibroblast cell lines from subjects with FMD compared to age- and gender-matched controls. Detailed phenotyping of patients with FMD allowed us to demonstrate that FMD is a systemic disease with alterations in common with the spectrum of genetic syndromes that involve altered TGF-β signaling and offers TGF-β as a marker of FMD.

Delayed post-surgical development of dural arteriovenous fistula after cervical meningocele repair

A 34-year-old female patient presented with an intracranial subarachnoid hemorrhage and was found to have a dural arteriovenous fistula at the site of previous cervical meningocele repair. Subsequent occlusion was achieved with endovascular embolization. To our knowledge, the phenomenon of the development of a spinal dural fistula at the site of a meningocele repair has not been recorded before.

Corticoides versus Placebo o tratamiento conservador en Cefalea post punción dural. Revisión sistemática y metaanálisis

Introducción Se realizó una revisión sistemática de la literatura y metaanálisis para determinar la utilidad, en términos de reducción de la intensidad y la prevención de la cefalea post-punción dural, de los corticoides endovenosos. Materiales y métodos Revisión sistemática y metaanálisis, dos revisores seleccionaron y analizaron los artículos definidos en los términos de entrada, extrajeron características generales de los estudios incluidos, describieron los principales hallazgos y resumieron sus resultados. Se analizó la heterogeneidad por el coeficiente de I2 y Tau2 , la magnitud del efecto se analizó en el coeficiente Z, todos los estadísticos asumieron un nivel de significancia del 95%, en cuadros y figuras (de control de sesgos y Forres Plot) Resultados 75 Referencias fueron seleccionadas, 33 identificadas en la búsqueda y 48 por método de Snowball; Seis concordaron con los criterios de elegibilidad; 69 fueron excluidas por no cumplir con los criterios de inclusión; el enmascaramiento aspecto de mayor riesgo de sesgo (intermedio); en conjunto hubo buen control del riesgo de sesgo entre los estudios incluidos. Los resultados muestran baja heterogeneidad a favor de la intervención con corticoides en el tratamiento de la cefalea post-dural; amplia heterogeneidad y sin efecto para la prevención de la cefalea post-dural. Conclusiones Se recomienda el uso de hidrocortisona o metilprednisolona en la reducción de la intensidad de la cefalea post-dural. No se cuenta con evidencia de dexametasona en la prevención de cefalea postdural. Se requiere de estudios adicionales, con otras intervenciones farmacológicas que sobre la plausibilidad de la condición (cefalea post-dural) pudieran prevenir o disminuir su ocurrencia

A inervação parassimpática colinérgica craniana e as cefaléias primárias : o papel da acetilcolina em um modelo experimental de vasodilatação dural neurogênica

Resumo não disponível

Avaliação da pressão liquórica em pacientes obstétricas submetidas à anestesia subaracnoidea que apresentaram cefaleia pós-punção dural

Pós-graduação em Anestesiologia - FMB

Endovascular Treatment of Tentorial Dural Arteriovenous Fistulae

Tentorial dural arteriovenous fistula (DAVF) is a rare vascular disease, which accounts for less than 4% of all cases of intracranial DAVF Because of the high risk of intracranial hemorrhage, patients with tentorial DAVF need aggressive treatment. Management approaches are still controversial, and endovascular treatment has emerged as an effective alternative. In the current work, we describe our experience with the endovascular approach in the treatment of these deep and complex DAVF of the tentorium. Eight patients were treated between January 2006 and July 2009. Six patients (75%) presented with intracranial hemorrhage related to the DAVF rupture. Four patients (50%) had subarachnoid bleeding and two had intraparenchymal hematoma. Endovascular treatment was performed via the transarterial route alone in five cases (62.5%), by the transvenous approach in two cases (25.0%) and in a combined procedure using both arterial and venous routes in one patient (12.5%). Complete obliteration of the fistula was achieved in all cases. The outcome at 15 months was favorable (modified Rankin scale 0-3) in seven (87.5%) patients. Complete cure of the lesion was confirmed in these cases. This paper reports on the effectiveness of endovascular treatment in tentorial DAVF management The choice of the venous versus the arterial approach is determined by regarding different anatomical dispositions.

Importance of anterior segment tomography for the diagnosis of corneal ectasia

Purpose: The methodology currently used for interpretation of the cornea and anterior segment tomography for the diagnosis of corneal ectasia and its susceptibility. Methods: Description of the methodology and clinical interpretation of corneal and anterior segment tomography indexes; report of three cases demonstrating the importance of this new diagnostic tool (Pentacam HR (R)) in ophthalmological practice. Conclusion: The use of corneal and anterior segment tomography seems to be an effective method to increase the sensitivity and specificity for the diagnosis and early detection of corneal ectasia.

Importância da tomografia de córnea para o diagnóstico de ectasia

OBJETIVO: Utilização da metodologia para interpretação da tomografia de córnea e segmento anterior para a detecção e diagnóstico de ectasia corneana. MÉTODOS: Descrição da interpretação clínica dos índices da tomografia de córnea e segmento anterior (Pentacam High Resolution - Oculus, Wetzlar,Germany); relato de 3 casos, demonstrando a importância da propedêutica no exame complementar, através do Pentacam HR®. CONCLUSÃO: Nossa experiência compatibiliza-se com os relatos de literatura na demonstração do incremento de sensibilidade e especificidade para a detecção precoce de susceptibilidade e diagnóstico de ectasia corneana, utilizando a tomografia de córnea e segmento anterior.

[Spontaneous dural arterio-venous fistula at the cavernous sinus: detected by decompensation following endoscopic sinus surgery]

Spontaneous dural arterio-venous fistulas can imperceptibly develop over a long time period before they suddenly develop symptoms like bruit, loss of vision, exophthalmos and conjunctival injection. We present the rare case of an occult, para-infectious, dural arterio-venous fistula which became symptomatic after endoscopic sinus surgery. Conjunctival injection and slight exophthalmos developed due to decompensation of venous drainage probably by intraoperative positioning of the patient, positive pressure ventilation and nasal packing.

Management, risk factors and outcome of cranial dural arteriovenous fistulae: a single-center experience

The role of endovascular interventions in managing dural arteriovenous fistulas (DAVFs) is increasing. Furthermore, in patients with aggressive DAVFs, different surgical interventions are required for complete obliteration or disconnection. Our objective was to evaluate the management of patients with intracranial DAVFs treated in our institution to identify the parameters that may help guide the long-term management of these lesions.

Endovascular and surgical treatment of spinal dural arteriovenous fistulas

INTRODUCTION: The aim of this retrospective study was to evaluate the clinical outcome of patients with spinal dural arteriovenous fistulas (SDAVFs) that were treated with surgery, catheter embolization, or surgery after incomplete embolization. METHODS: The study included 21 consecutive patients with SDAVFs of the thoracic, lumbar, or sacral spine who were treated in our institution from 1994 to 2007. Thirteen patients were treated with catheter embolization alone. Four patients underwent hemilaminectomy and intradural interruption of the fistula. Four patients were treated by endovascular techniques followed by surgery. The clinical outcome was assessed using the modified Aminoff-Logue scale (ALS) for myelopathy and the modified Rankin scale (MRS) for general quality of life. Patient age ranged from 44 to 77 years (mean 64.7 years). RESULTS: Surgical as well as endovascular treatment resulted in a significant improvement in ALS (-62.5% and -31.4%, respectively, p < 0.05) and a tendency toward improved MRS (-50% and -32%, respectively) scores. Patients that underwent surgery after endovascular treatment due to incomplete occlusion of the fistula showed only a tendency for improvement in the ALS score (-16.7%), whereas the MRS score was not affected. CONCLUSION: We conclude that both endovascular and surgical treatment of SDAVFs resulted in a good and lasting clinical outcome in the majority of cases. In specific situations, when a secondary neurosurgical approach was required after endovascular treatment to achieve complete occlusion of the SDAVF, the clinical outcome was rather poor. The best first line treatment modality for each individual patient should be determined by an interdisciplinary team.