Ectasia dural e hipotensión endocraneal en síndrome de Marfán / Dural ectasia and intracranial hypotension in Marfan syndrome

INTRODUCCIÓN: El síndrome de Marfán es un trastorno multisistémico del tejido conectivo de herencia autosómica dominante, de expresión variable. La ectasia dural es un compromiso frecuente, pero poco conocido, que puede asociarse a síndrome de hipotensión endocraneana (SHE). OBJETIVO: Pre sentar un caso de cefalea invalidante secundario a SHE, para advertir de esta rara complicación, que debe tenerse presente en niños portadores de conectivopatías, en especial síndrome de Marfán. CASO CLÍNICO: Adolescente femenina de 13 años, portadora de sindrome de Marfán, de diagnóstico clínico según criterios de Ghent 2010, que consultó por cefalea ortostatica invalidante de 6 meses de evolución. La Resonancia Magnetica (RM) de cerebro mostró múltiples signos de hipotensión endocraneana, mientras que la RM de columna total mostró una ectasia dural que determinó la dilatación del saco tecal y remodelación posterior de los cuerpos vertebrales, especialmente a nivel del sacro. Se realizó tratamiento con parche sanguíneo autólogo epidural con buena respuesta clínica. CONCLUSIONES: La ectasia dural, frecuente en el sindrome de Marfán, es una causa predisponente a fuga de líquido cefaloraquideo (LCR), que podría causar cefalea ortostática segundaria al SHE.

INTRODUCTION: Marfan syndrome is an autosomal dominant, multi-systemic connective tissue di sorder of different presentations. Dural ectasia is a common, but little known complication that can be associated with intracranial hypotension syndrome (IHS). OBJECTIVE: To present a case of severe headache secondary to IHS in order to warn about this rare complication, which must be considered in children carriers of connective tissue diseases, especially Marfan syndrome. CLINICAL CASE: 13-year- old female carrier of Marfan syndrome, clinically diagnosed according to the 2010 Ghent criteria, who consulted due to a 6-months history of severe orthostatic headache. Head magnetic resonance imaging (MRI) showed multiple signs of intracranial hypotension, while whole-spine MRI showed dural ectasia that caused the thecal sac dilation and subsequent remodeling of vertebral bodies, es pecially the sacral ones. Treatment with an autologous epidural blood patch was administered with good clinical response. CONCLUSIONS: Dural ectasia, frequent in Marfan syndrome, is a predisposing cause of cerebrospinal fluid (CSF) leakage, which could cause orthostatic headache secondary to IHS.

Metástasis durales: una forma infrecuente de recurrencia en tumores nasosinusales malignos / Dural metastasis: an uncommon form of recurrence in malignant sinonasal tumours

Abstract: Dural metastases are an unusual form of spread in treated sinonasal malignancies. An analysis is presented of 20 cases of dural metastases diagnosed during imaging follow-up in a selection of cases in which anterior craniofacial resection was performed. They included 12 undifferentiated sinonasal carcinomas, 7 olfactory neuroblastomas, and 1 adenoid cystic carcinoma case. Dural metastases appeared on an average of 7.3 years after treatment in olfactory neuroblastoma. The maximum distance from malignancy to dural metastases was 14 cm for olfactory neuroblastoma, and 4.3 cm for undifferentiated sinonasal carcinoma. Dural metastases in the Burr holes were observed in 50% of undifferentiated sinonasal carcinoma, and 29% of olfactory neuroblastomas. Dural metastases presented as a nodular (60%), multinodular (10%), cystic (15%), and plaque (15%) pattern. These are suggestive of a local venous spread mechanism related to tumour rupture during surgery of anterior cranial fossa. Long-term follow-up with cranial inclusion would be indicated, given the possible late and distant presentation of dural metastases.

Resumen: Presentamos las metástasis durales como forma inusual de diseminación de tumores nasosinusales malignos tratados; se revisan 20 casos diagnosticados durante el seguimiento imagenológico a un grupo tratado con resección craneofacial anterior. Evaluamos metástasis durales en 12 carcinomas nasosinusales indiferenciados, 7 neuroblastomas olfatorios y un carcinoma adenoquístico. En neuroblastomas olfatorios aparecieron metástasis durales en promedio 7,3 años postratamiento. La distancia máxima del tumor a la metástasis fue de 14 cm para neuroblastoma olfatorio y de 4,3 cm para carcinoma nasosinusal indiferenciado. Observamos metástasis durales en los agujeros de trepanación en el 50% de los carcinomas nasosinusales indiferenciados y en el 29% de los neuroblastomas olfatorios. Las metástasis durales presentaron patrón nodular (60%), multinodular (10%), quístico (15%) y en placa (15%). Proponemos un mecanismo venoso local de diseminación relacionado a disrupción tumoral o quirúrgica de la fosa craneal anterior. El seguimiento a largo plazo con inclusión craneal estaría indicado por la posible presentación tardía y distante de metástasis durales.

Surgical Treatment for Falcotentorial Meningiomas

Among intracranial meningiomas, falcotentorial meningiomas, occurring at the junction of the falx cerebri and tentorial dural folds, are extremely rare. Because of their deep location, they are surrounded by critical structures, and have been regarded as one of the most challenging lesions for surgical treatment. In this study, we describe our surgical strategy for falcotentorial meningiomas and provide a review of our experience.

Histological analysis of the repair of dural lesions with silicone mesh in rats subjected to experimental lesions / Análise histológica do reparo de lesões da dura com tela de silicone em ratos submetidos à lesão experimental

ABSTRACT Objective To evaluate inflammatory reaction, fibrosis and neovascularization in dural repairs in Wistar rats using four techniques: simple suture, bovine collagen membrane, silicon mesh and silicon mesh with suture. Methods Thirty Wistar rats were randomized in five groups: the first was the control group, submitted to dural tear only. The others underwent durotomy and simple suture, bovine collagen membrane, silicon mesh and silicon mesh with suture. Animals were euthanized and the spine was submitted to histological evaluation with a score system (ranging from zero to 3) for inflammation, neovascularization and fibrosis. Results Fibrosis was significantly different between simple suture and silicon mesh (p=0.005) and between simple suture and mesh with suture (p=0.015), showing that fibrosis is more intense when a foreign body is used in the repair. Bovine membrane was significantly different from mesh plus suture (p=0.011) regarding vascularization. Inflammation was significantly different between simple suture and bovine collagen membrane. Conclusion Silicon mesh, compared to other commercial products available, is a possible alternative for dural repair. More studies are necessary to confirm these findings.

RESUMO Objetivo : Avaliar reação inflamatória, fibrose e neovascularização da reparação da lesão dural em ratos Wistar, comparando quatro diferentes técnicas: pontos simples, membrana de colágeno bovino, tela de silicone e tela de silicone associada a pontos simples. Métodos : Trinta ratos Wistar foram randomizados em cinco grupos: o primeiro foi um grupo controle, submetido somente à durotomia. Os outros também foram submetidos à durotomia, porém sofreram sutura simples, reparo com membrana de colágeno bovino, tela de silicone e tela de silicone com sutura. Os animais foram sacrificados, e a coluna foi submetida à avaliação histológica com um escore (variando de zero a 3) para inflamação, neovascularização e fibrose. Resultados : A fibrose foi significativamente diferente, comparando-se sutura simples e tela de silicone (p=0,005) e sutura simples e tela com fio de sutura (p=0,015), demonstrando que a fibrose foi mais intensa quando um corpo estranho foi utilizado na reparação. Membrana bovina foi significativamente diferente da tela mais sutura (p=0,011) em relação à vascularização. A inflamação foi significativamente diferente entre os grupos submetidos à sutura simples e ao reparo com membrana de colágeno bovino. Conclusão : A tela de silicone, comparada com produtos similares com disponibilidade comercial, é uma possível alternativa como protetor de dura-máter. Mais estudos são necessários para comprovar esses resultados.

Efficacy of Percutaneous Epidural Neuroplasty Does Not Correlate with Dural Sac Cross-Sectional Area in Single Level Disc Disease

PURPOSE: Percutaneous epidural neuroplasty (PEN) is a minimally invasive treatment. The efficacy of PEN has been relatively well investigated; however, the relationship between the clinical effectiveness of PEN and the severity of spinal canal stenosis by disc material has not yet been established. The purpose of this study was to compare clinical outcomes of PEN according to the dural sac cross-sectional area in single level disc disease. MATERIALS AND METHODS: This study included 363 patients with back pain from single level disc disease with and without radiculopathy. Patients were categorized into groups according to spinal canal compromise by disc material: Category 1, less or more than 50%; and Category 2, three subgroups with lesser than a third, between a third and two thirds, and more than two thirds. Clinical outcomes were assessed according to the Visual Analog Scale (VAS) score for back pain and leg pain and Odom's criteria at 1, 3, 6, 12, and 24 months after treatment. RESULTS: The demographic data showed no difference between groups according to spinal canal compromise by disc material except age (older age correlated with more spinal canal compromise). The dural sac cross-sectional area did not correlate with the VAS scores for back and leg pain after PEN in single level disc disease in Groups 1 and 2. Odom's criteria after PEN were also not different according to dural sac cross-sectional area by disc material. CONCLUSION: PEN is an effective procedure in treating single level lumbar disc herniation without affecting dural sac cross-sectional area.

An experimental model for the study of craniofacial deformities / Modelo experimental para o estudo de deformidades craniofaciais

PURPOSE: To develop an experimental surgical model in rats for the study of craniofacial abnormalities. METHODS: Full thickness calvarial defects with 10x10-mm and 5x8-mm dimensions were created in 40 male NIS Wistar rats, body weight ranging from 320 to 420 g. The animals were equally divided into two groups. The periosteum was removed and dura mater was left intact. Animals were killed at 8 and 16 weeks postoperatively and cranial tissue samples were taken from the defects for histological analysis. RESULTS: Cranial defects remained open even after 16 weeks postoperatively. CONCLUSION: The experimental model with 5x8-mm defects in the parietal region with the removal of the periosteum and maintenance of the integrity of the dura mater are critical and might be used for the study of cranial bone defects in craniofacial abnormalities.

OBJETIVO: Desenvolver um modelo experimental em ratos para o estudo de deformidades craniofaciais. MÉTODOS: Foram realizados defeitos ósseos de espessura total com diâmetro de 5 x 8 mm e 10 x 10mm na calota craniana em 40 ratos, machos, NIS Wistar, com peso de 320 a 420 g divididos igualmente em dois grupos. O periósteo foi retirado e a dura-máter mantida intacta. Os animais foram sacrificados na 8ª e 16ª semana de pós-operatório e amostras de tecido ósseo foram extraídas para realização da análise histológica. RESULTADOS: Os defeitos cranianos permaneceram abertos mesmo após 16 semanas após a cirurgia. CONCLUSÃO: O modelo experimental com defeitos de 5x8mm na região parietal, com remoção do periósteo e manutenção da integridade da dura-máter são considerados críticos, e poderá ser utilizado para o estudo dos defeitos ósseos cranianos nas anomalias craniofaciais.

Síndrome lumbociático de etiología distinta a hernia discal / Etiology of lumbosciatic syndrome different from lumbar herniated disk

La causa más frecuente de lumbociática es la hernia de disco y la patología asociada de estenosis foraminal, espondilolistesis y entesopatía de la articulación facetaria, incluyendo quistes sinoviales. Existen una serie de condiciones que pueden presentar un cuadro clínico similar, y el problema es detectar estas causas infrecuentes en un universo muy grande de pacientes con patología discal. Esto crea una situación potencialmente peligrosa, en la cual se podría interpretar la sintomatología secundaria, por ejemplo a un tumor, como producida por una hernia discal, por otro lado asintomática. En base a una historia clínica cuidadosa y al uso racional de los exámenes complementarios, se puede sospechar aquellos casos que pudieran albergar esta patología de baja incidencia, pero de gran importancia clínica. Se revisan las causas más importantes que pueden provocar un síndrome lumbociático y que deben incluirse en el diagnóstico diferencial, como el síndrome piriforme, tumores intradurales y del nervio ciático y fístulas durales.

The most frequent etiology of sciatic pain is herniation of the nucleus pulposus and associated entesopathic diseases, including synovial cysts. There are several conditions that can present with a similar clinical picture, and the clinician is confronted with the problem of detecting this infrequent occurrences. This creates a potentially dangerous condition of thinking that an asymptomatic disc herniation is causing the symptoms that are originated higher by a tumor for example. With a careful history and judicious use of ancillary examinations, specially NMR, most of the cases can be suspected. The principal causes of non-discal sciatica are reviewed, including piriform syndrome, tumors of the spine and sciatic nerve, and dural fistulae.

Intracranial Dural Metastasis of Ewing's Sarcoma: a Case Report

Although intracranial dural metastasis of Ewing's sarcoma is a very rare finding, its imaging characteristics are similar to those of its primary form in the central nervous system. Thus, this tumor must be considered in the differential diagnosis of extra-axial dural masses.

Enxertos homólogos de esclera e dura-máter em olhos de coelhos: análise histopatológica comparativa / Homologous scleral and dura mater grafts in rabbit eyesa comparative histopathological analysis

OBJETIVO: Avaliar a reação tecidual à implantação de enxertos homólogos de esclera e dura-máter. MÉTODOS: Foram realizadas cirurgias experimentais em 41 coelhos albinos da raça Nova Zelândia, sendo que dois coelhos foram selecionados, ao acaso, para serem doadores dos enxertos a serem utilizados no experimento. Trinta coelhos foram divididos em dois grupos: grupo D e grupo E, sendo submetidos à implantação dos tecidos homólogos e nove animais constituíram o grupo controle (grupo DE), submetidos à cirurgia sem enxertia. Os olhos foram enucleados e amostras foram colhidas em duas, seis e 12 semanas após o experimento, constituindo os subgrupos I, II e III. RESULTADOS: Foi realizada análise histopatológica qualitativa, além de estudo semiquantitativo comparativo da vascularização e infiltrado inflamatório na esclera e dura-máter transplantadas. Procedeu-se, também, a histomorfometria das medidas do lado dos enxertos com sistema analisador de imagens. Não houve diferença estatisticamente significante comparando-se a vascularização, infiltrado inflamatório e medidas do lado, entre os subgrupos I de esclera em relação à dura-máter, assim como nos subgrupos II. Não foram comparados os subgrupos III, pois foram observados somente fragmentos dos enxertos de dura-máter. CONCLUSÕES: O enxerto de dura-máter mostrou intensa reabsorção e progressiva substituição por tecido conjuntivo frouxo. O enxerto de esclera mostrou discreta reabsorção na periferia com formação de membrana fibrosa mais evidente, integrando este à esclera do hospedeiro.

PURPOSE: To evaluate tissue reaction to implantation of homologous scleral and dura mater grafts. METHODS: Experimental surgeries were performed on 41 albine New Zealand rabbits; two rabbits were selected at random to be graft donors. Thirty rabbits were divided into two groups: group D and group E, receiving grafts of homologous tissues. The remaining nine animals comprised the control group (DE) and were submitted to surgery but with no graft. The eyes were enucleated and samples collected two, six and 12 weeks after the experiment, comprising subgroups I, II and III. RESULTS: A qualitative histopathological analysis was performed together with a comparative semi-quantitative study on vascularization and inflammatory infiltrate in the transplanted sclera and dura mater. Histomorphometry was conducted based on measurements of the grafts with an image analyzing system. There was no statistically significant difference when comparing vascularization, inflammatory infiltrate and measurements between subgroup I in relation to sclera and dura mater, nor in subgroup II. Subgroup III was not compared, since only fragments of dura mater grafts were considered. CONCLUSIONS: The dura mater graft presented intense absorption and progressive replacement of loose connective tissue. And the scleral graft showed discreet absorption in the periphery with formation of a more evident fibrous membrane, integrating the graft into the host sclera.

Hypertrophic spinal pachymeningitis presenting as compressive myelopathy: a case report.

Hypertrophic pachymeningitis (HP) is a rare chronic inflammatory process that causes thickening of the dura leading to compressive myelopathy. HP has diverse etiologies like infections, chronic inflammatory processes, collagen vascular diseases and malignancy. We report one such case of HP who presented with compressive myelopathy, underwent decompressive surgery and died due to complications of surgery with the original disease process.

Cavernous Angioma in the Falx Cerebri: A Case Report

Intracranial cavernous angiomas are benign vascular malformations and can be divided into intra-axial and extra-axial lesions. Extra-axial cavernous angiomas are relatively rare and usually arise in relation to the dura mater and mimick meningiomas. We report a case of cavernous angioma that occured in the falx cerebri of a 22-yr-old female patient with the special focus on neuroradiologic findings. This is the fourth case of cavernous angioma in the falx cerebri reported in the literature to our knowledge.

Extensive anterior cranial fossa idiopathic hypertrophic pachymeningitis: a case report and review of the literature.

Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory and fibrosing process of unknown etiology. This entity is characterized by fibrosis and thickening of the dura mater and resulting in neurological syndrome. The authors report a 72 year-old woman who presented with progressive bifrontal headache, bilateral visual loss and transient episode of confusion. Neurological examination revealed bilateral optic atrophy, apathy and no focal neurological deficit. Investigations showed anemia of chronic disease, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. No specific inflammatory diseases or malignancy such as systemic lupus erythematosus, syphilis, hematologic malignancy were found MRI of the brain revealed thickened and enhanced dura mater and leptomeninges at the inferior aspect of bilateral frontal lobes as well as vasogenic edema of the frontal lobes. Cerebrospinal fluid showed mild pleocytosis, high protein level and normal glucose level. Meningeal biopsy revealed nonspecific inflammatory process of the dura and leptomeninges. There was no granuloma formation or evidence of vasculitis. Special stain for tuberculous bacilli, fungus and malignancy were all negative. The diagnosis of "idiopathic hypertrophic pachymeningitis" was made. The patient was treated with oral prednisolone 45 mg/day. Her headache was improved, but the profound vision loss in both eyes remained unchanged after 2 years of follow-up. Prednisolone was tapered within 18 months. Idiopathic hypertrophic cranial pachymeningitis usually involves dura at tentorium cerebelli, cavernous sinus and base of the skull. The extensive involvement at the anterior cranial fossa is extremely rare.

Paquimeningite hipertrófica idiopática craniana associada a pseudotumor orbitário: relato de caso / Cranial idiopathic hypertrophic pachymeningitis associated with orbital pseudotumor: case report

Hypertrophic pachymeningitis is a rare disorder characterized by meningeal thickness, that can be caused by infection, tumoral infiltration, inflammatory disorders or idiopathic. We report the case of a 40 year-old man that presented with longstanding headache and progressive bilateral visual loss and proptosis. Cranial and orbital magnetic resonance imaging revealed diffuse dural thickness and bilateral extraconal orbital lesion. Extensive investigation did not reveal any systemic condition. Histopathological study after meningeal and orbital biopsy disclosed a chronic inflammatory process compatible respectively with idiopathic hypertrophic pachymeningitis (IHP) and orbital pseudotumor. This case emphasizes that orbital involvement can occur in IHP and that its early identification is of great importance in order to improve the visual prognosis of this condition.

Paquimeningite hipertrófica se caracteriza por espessamento das meninges, podendo ser decorrente de infecção, infiltração tumoral, doença inflamatória ou idiopática. Relatamos sobre um homem de, 40 anos, com queixa de cefaléia de longa data e perda progressiva da visão em ambos os olhos acompanhadas de proptose bilateral. A imagem por ressonância magnética de crânio e órbitas revelou espessamento dural difuso e lesão orbitária bilateral. Extensa investigação não revelou qualquer afecção sistêmica. Estudo anatomopatológico realizado após biópsias de meninges e da massa orbitária evidenciou processo inflamatório crônico compatível com paquimeningite hipertrófica idiopática (PHI) e com pseudotumor orbitário respectivamente. Este caso evidencia que o acometimento orbitário pode ocorrer na PHI e que a sua identificação precoce é de fundamental importância para o prognóstico visual.

Temporal bone carcinoma with intradural extension.

A case of temporal bone carcinoma having intradural extension is reported. To the best of our knowledge, no such case has been reported so far.

Idiopathic hypertrophic cervical pachymeningitis: a case report with 5 year follow up.

Idiopathic hypertrophic pachymeningitis is an extremely rare entity. It usually affects cranial meninges. The spinal form is further uncommon and presents as a chronic progressive disease. We describe a 42 year old female with isolated idiopathic hypertrophic cervical pachymeningitis who had a relapsing remitting course under observation for five years. Laminectomy and immunosuppressive therapy produced temporary and partial relief. The long term course and relevant literature is reviewed.

Calcified falx meningioma.

A totally calcified mid third falcine meningioma in an elderly male patient is presented. An uneventful enmasse excision was performed. Advantages of positioning on ipsilateral side for paramedian extracerebral lesions are highlighted.

Diagnóstico y análisis de las fístulas arteriovenosas durales mediante TC y RM / Dural arteriovenous fistulae: CT and MR diagnostic features

Existen múltiples clasificaciones de las fístulas arteriovenosas durales (FAVD). A fin de identificar las susceptibles de sufrir complicaciones y de este grupo, los hallazgos en TC y RM se estudiaron 23 pacientes con diagnóstico de fístula arteriovenosa dural, con drenaje venoso leptomeníngeo. El rango de edad osciló entre 20 y 75 años. Los pacientes fueron estudiados con TC, RM y Angiografía por sustracción digital (ASD). Además en 4 pacientes se realizó AngioRM. La TC y la IRM permitieron diagnosticar dilataciones vasculares en 20 pacientes (86 por ciento). De estos 20, en 14 (70 por ciento) se detectaron complicaciones entre ellas: hipertensión venosa focal (5 pacientes), infarto venoso secundario a la hipertensión venosa (3), hematomas intraparenquimatosos (2), hemorragia subaracnoidea (2), compresión de pares craneales (1) y necrosis diencefálica (1 paciente). Se detectaron, además 2 pacientes con trombosis del seno longitudinal superior. La TC y la IRM resultaron ser métodos muy útiles en el diagnóstico y análisis de las complicaciones de las FAVD, con una sensibilidad y especificidad cercana al 90 por ciento en la detección de las venas leptomeníngeas dilatadas que son las responsables de las complicaciones