Avaliação das aberrações de alta ordem em pacientes com ceratocone inicial / Assessment of high-order aberrations in patients with early keratoconus

RESUMO Objetivo Identificar alterações de aberrometria de alta ordem em diferentes graus de ceratocone. Métodos Estudo transversal, retrospectivo, observacional. Foram analisados 54 pacientes (108 olhos) diagnosticados com ceratocone pelo mesmo especialista em córnea por meio dos critérios ABCD de Belin/Ambrósio Enhanced Ectasia, utilizando-se o tomógrafo Pentacam® HR 70900 (Oculus Wetzlar, Alemanha). Além disso, foram feitas análises qualitativa e quantitativa das aberrações de alta ordem desses mesmos pacientes por meio do OPD-Scan III-NIDEK. Resultados Por meio da avaliação de ambos os olhos dos pacientes com os critérios de Belin-Ambrósio, constatou-se presença de ceratocone em 34 pacientes. Ademais, por meio da análise estatística, constatou-se relação direta entre a asfericidade posterior e o desenvolvimento do ceratocone, com p<0,001 (referência: p<0,05). Por meio da análise do OPD-Scan III-NIDEK, as principais aberrações de alta ordem encontradas nos pacientes com ceratocone foram coma, trefoil e aberração esférica. Conclusão O raio da curvatura posterior é a primeira variável a se alterar com o desenvolvimento do ceratocone, o que se faz perceptível na análise da asfericidade posterior por meio o Pentacam®. Além disso, a alteração da paquimetria e da asfericidade posterior influencia diretamente o desenvolvimento de aberrações de alta ordem em pacientes com ceratocone.

ABSTRACT Objective To identify higher order aberrometry changes in different degrees of keratoconus. Methods Cross-sectional, retrospective, observational study. Fifty-four patients (108 eyes) diagnosed with keratoconus by the same corneal specialist using the Belin/Ambrósio Enhanced Ectasia ABCD criteria were analyzed, using the Pentacam® HR 70900 tomograph (Oculus Wetzlar, Germany). In addition, qualitative and quantitative analysis of higher order aberrations in these patients was performed using the OPD-Scan III-NIDEK. Results Through the evaluation of both eyes of the patients according to the criteria of Benin Ambrósio, the presence of KCN was verified in 34 patients. Furthermore, through statistical analysis, a direct relationship was found between posterior asphericity and the development of KCN; p<0.001 (reference: p<0.05). Through the analysis of the OPD scan, the main higher order aberrations found in patients with KCN were Coma, Trefoil and Spherical Aberration (AE). Conclusion The posterior curvature radius is the first variable to change with the development of the KCN, which is noticeable in the analysis of posterior asphericity in Pentacam. In addition, alterations in pachymetry and posterior asphericity directly influence the development of higher order aberrations in patients with KCN.

Unexpectedly high corneal flap thickness and ectasia after mechanical LASIK / Espessura inesperadamente alta do flap corneano e ectasia após LASIK mecânico

ABSTRACT LASIK is a refractive surgical procedure in which a corneal flap is created to expose the corneal stromal bed. Preoperative estimation of corneal flap thickness is necessary to calculate the percentage tissue altered in LASIK, an important quantitative risk factor for ectasia. The objective of this study was to assess flap thickness and calculate percentage tissue altered to check if unexpectedly thicker flaps and higher percentage tissue altered could pose as risk factors of ectasia. Four subjects (eight eyes) were submitted to mechanical LASIK in 2009 and 2010. Pre and postoperative clinical and tomographic data were reviewed. Mean preoperative estimated percentage tissue altered was 39.18±1.31%, which was borderline for increased ectasia risk when considering the limit of 40%. However, when considering the postoperatively measured flap thickness, the actual mean percentage tissue altered turned out to be 45.17 ± 4.13%, which was significantly higher than predicted preoperatively (p=0.002). Unexpectedly higher postoperative percentage tissue altered may be responsible for corneal ectasia after mechanical LASIK.

RESUMO A LASIK é um procedimento cirúrgico refrativo, no qual um retalho corneano é criado para expor o leito estromal corneano. A estimativa pré-operatória da espessura do retalho corneano é necessária para calcular o percentual de tecido alterado no LASIK, um importante fator de risco quantitativo para ectasia. O objetivo deste estudo foi avaliar a espessura do retalho e calcular o percentual de tecido alterado para verificar se retalhos inesperadamente mais espessos e percentuais de tecido alterado mais altos poderiam representar fatores de risco de ectasia. Quatro indivíduos (oito olhos) foram submetidos à LASIK mecânica em 2009 e 2010. Dados clínicos e tomográficos pré e pós-operatórios foram revisados. A média de percentual de tecido alterado pré-operatória estimada foi de 39,18±1,31%, limítrofe para risco aumentado de ectasia quando considerado o limite de 40%. No entanto, ao considerar a espessura do retalho medida no pós-operatório, o percentual de tecido alterado médio real foi de 45,17±4,13%, ou seja, significativamente maior do que o previsto no pré-operatório (p=0,002). O percentual de tecido alterado pós-operatóriao inesperadamente mais alto pode ser responsável pela ectasia da córnea após LASIK mecânico.

Belin/Ambrósio Enhanced Ectasia Display in cataract surgery and relation to final visual acuity / Belin/Ambrósio Enhanced Ectasia Display na cirurgia de catarata e sua relação com a acuidade visual final

ABSTRACT Objective To analyze and describe the coefficients found on maximum Ambrósio Relational Thickness-Maximum (ART-Max) and Belin/Ambrósio Enhanced Ectasia Display total deviation (BAD-D) in eyes with normal corneal topography subjected to cataract surgery with premium intraocular lens implantation and correlated these data with final visual acuity. Methods ART-Max and BAD-D data from 103 eyes of patients subjected to implantation of diffractive bifocal intraocular lens, with normal corneal topography who achieved visual acuity of 20/20 or 20/25 without correction after cataract surgery were analyzed. The groups with normal and abnormal values were compared using the chi-square test. Results Thirty-two (31.1%) and 71 (68.9%) eyes presented normal and abnormal ART-Max values, respectively. The difference between these groups was significant (p=0.0002). Fifty-five (53.4%) and 48 (46.6%) eyes had normal and abnormal BAD-D, respectively, and intergroup difference was not significant (p=0.9576). Conclusion Among patients with normal corneal topography who underwent premium intraocular and had good final visual acuity of 20/20 or 20/25, suspicious or abnormal indices of ART-Max and BAD-D were frequent, providing evidence that it possibly should not be a contraindication.

RESUMO Objetivo Analisar e descrever os coeficientes numéricos encontrados nos exames Ambrósio Relational Thickness-Maximum (ART-Max) e desvio total do Belin/Ambrósio Enhanced Ectasia Display (BAD-D) em olhos com topografia normal submetidos ao implante de lente intraocular premium na cirurgia de catarata, correlacionando-os com a acuidade visual final pós-operatória. Métodos Foram analisados os resultados de ART-Max e BAD-D de 103 olhos de pacientes submetidos ao implante de lentes bifocais difrativas, que apresentavam exame topográficos normal e alcançaram acuidade visual 20/20 ou 20/25 sem correção visual no pós-operatório final. Para a análise estatística entre os grupos normais e anormais ou suspeitos, utilizou-se o teste do qui-quadrado. Resultados Foram encontrados 32 (31,1%) olhos com ART-Max normal e 71 (68,9%) com ART-Max suspeito/anormal. A diferença entre os grupos foi significativa (p=0,0002). Quanto ao BAD-D, foram encontrados 55 (53,4%) olhos com resultados normais e 48 (46,6%) com resultados suspeitos/anormais. A diferença entre os grupos não foi significativa (p=0,9576). Conclusão Entre os pacientes com topografia normal submetidos ao implante de lentes premium e que alcançaram acuidade visual 20/20 ou 20/25, os índices suspeitos ou anormais de ART-Max e BAD-D eram frequentes, não se configurando em contraindicação para a realização do implante.

Ectasia coronaria y miocardiopatía hipertrófica: una entidad infrecuente / Coronary artery ectasia and hypertrophic cardiomyopathy: case report

Resumen Se presenta el caso de un paciente de 54 años que consulta por angina de esfuerzo de 2 años de evolución en quien se identifica una dilatación ectásica del árbol coronario con lesiones ateroscleróticas críticas y miocardiopatía hipertrófica septal obstructiva. Una revisión bibliográfica revela que es una asociación infrecuente de la cual solo existen reportes de casos aislados.

Abstract We present the case of a 54-year-old patient who presented with a history of 2 years with angina. Invasive studies revealed critical coronary artery stenosis coexisting with obstructive hypertrophic miopathy. This is a rare association with only isolated case reports.

Slow Flow Phenomenon Impairs the Prognosis of Coronary Artery Ectasia as Well as Coronary Atherosclerosis

Abstract Introduction: Coronary artery ectasia (CAE) is one of the uncommon cardiovascular disorders and its prognosis is still debated. Objective: We aimed to review long-term follow-up data in patients with CAE and to evaluate the prognosis of CAE patients with coronary slow flow phenomenon (CSFP). Methods: This study had a prospective design and 143 patients with CAE were included. The angiographic and demographic characteristics were reviewed in detail. The patients were categorized, based on concomitant coronary artery stenosis detected by angiography, as CCAE group (n=87, ≥30% luminal stenosis) and ICAE group (n=56, <30% luminal stenosis) and also categorized by the coronary flow as CSFP group (n=51) and normal flow coronary ectasia - NCEA group (n=92). All patients were re-evaluated at 6-month intervals. Follow-up data, cardiovascular (CV) mortality, hospitalization and major adverse cardiac events (MACE) were collected. The level of statistical significance was set at 5%. Results: Patients were followed up for an average of 56.9±7.4 months. During the follow-up, statistically significant differences were found in hospitalization, CV mortality and MACE between the CCAE and ICAE groups (P=0.038, P=0.003, P=0.001, respectively). The CSFP and NCEA groups were also compared. There was a statistical difference with respect to hospitalization between the CFSP and NCEA groups (P=0.001), but no difference was observed in terms of MACE and CV mortality (P=0.793 and P=0.279). Conclusion: CSFP accompanying CAE may be a predictor of hospitalization. Significant atherosclerotic plaques coexisting with CAE may be predictive for MACE.

Fibrinogen-to-albumin ratio may be a predictor for ascending aortic aneurysm

SUMMARY OBJECTIVE: The predictive value of the fibrinogen-to-albumin ratio has been evidenced in coronary artery disease. Available data demonstrated that inflammation and oxidative stress are the relevant mechanisms of ascending aortic aneurysm formation and dilatation. The fibrinogen-to-albumin ratio reflects oxidative stress and inflammation. This study investigated the correlation between fibrinogen-to-albumin ratio and ascending aortic aneurysm. METHODS: A total of 250 consecutive patients with ascending aortic aneurysm and 250 consecutive patients with normal ascending aortic diameter were included in the study using comprehensive transthoracic echocardiography. All data and fibrinogen-to-albumin ratio were compared between two groups. RESULTS: The fibrinogen-to-albumin ratio levels were significantly higher in ascending aortic aneurysm group compared with normal ascending aortic diameter group (p<0.001). Also, there was significantly positive correlation between the diameter of the ascending aorta and the fibrinogen-to-albumin ratio (p<0.001). CONCLUSION: Fibrinogen-to-albumin ratio is associated with ascending aortic aneurysm and may serve as blood marker for identifying high-risk patients.

Caracterização de ectasia subclínica com análise integrada da tomografia e biomecânica da córnea / Characterization of subclinical ectasia with integrated corneal tomography and biomechanics assessments

RESUMO O artigo tem como objetivo descrever de forma prospectiva diferentes casos de ectasias altamente assimétricas (very asymmetric ectasia, VAE) para diferenciar formas subclínicas ou "frustas" do ceratocone (forme fruste keratoconus - FFKC) de casos de doença ectásica unilateral. O Caso 1 é um paciente de 39 anos, que admitiu ter coçado intensamente apenas o olho direito (OD) na juventude, se apresentando com ectasia unilateral tratada com sucesso por meio de implante de anel intraestromal em OD. O olho esquerdo (OE) apresentou-se normal ao exame completo por meio de propedêutica multimodal e acuidade visual não corrigida (AVsc) de 20/20, estável por mais de 5 anos, com TBI (tomography and biomechanical index) de 0.02. No Caso 2 é um paciente de 15 anos com ectasia clínica em OD, e OE com topografia normal, mas alterações tomográficas e biomecânicas, incluindo o TBI 0,56, caracterizarando a doença subclínica (FFKC). O Caso 3 é a mãe do paciente do Caso 2, de 46 anos, que se apresentou com presbiopia, sem qualquer histórico oftalmológico relevante. A AVsc foi de 20/20 em cada olho, topografia de Placido com leve encurvamento inferior, mas sem sinais definitivos de ectasia. A avaliação biomecânica e tomográfica revelou sinais de ceratocone em ambos os olhos, com TBI de 1,0 e 0,99. Esses três casos estão de acordo com a definição do consenso global e a hipótese de dois acertos (two-hit hypothesis), que ceratocone é uma doença bilateral, mas ectasia pode ocorrer por causa estritamente mecânica unilateralmente (ou em qualquer olho). A relevância da propedêutica multimodal é destacada, destacando-se a integração do estudo biomecânico e tomográfico com imagens de Scheimpflug.

ABSTRACT The article aims to prospectively describe different cases of highly asymmetric ectasia (very asymmetric ectasia, VAE) to differentiate subclinical or "frustrated" forms of keratoconus (forme fruste keratoconus - FFKC) from cases of unilateral ectatic disease. Case 1 is a 39-year-old patient with unilateral ectasia treated with an intrastromal ring implant. The contralateral eye was normal due to multimodal propaedeutics, stable for more than 3 years, with a TBI of 0.02. The patient admitted to having intensely scratched only his right eye in his youth. In Case 2, a 15-year-old patient with clinical ectasia in the right eye, had a left eye with normal topography and tomographic and biomechanical changes characterizing FFKC. Case 3 is the mother of the case 2 patient, aged 46, who presented with presbyopia, without any relevant ophthalmological history. Uncorrected visual acuity of 20/20 in each eye, Placido topography with slight lower curving, but without definitive signs of ectasia. The biomechanical and tomographic evaluation revealed signs of keratoconus in both eyes. These three cases are in accordance with the definition of the global consensus: keratoconus is a bilateral disease, but ectasia can occur because of strictly mechanical unilateral (in any eye). The relevance of multimodal refractive imaging is highlighted, with a focus on integrating biomechanical and tomographic assessments with Scheimpflug images.

Laser topoguiado como tratamento de ectasia pós lasik / Topoguided laser as treatment of ectasia after lasik

RESUMO O laser in situ keratomileusis (LASIK) é o método cirúrgico refrativo mais realizado no mundo com excelentes resultados, porém tal método não é isento de riscos. A ectasia pós LASIK é uma complicação grave e se caracteriza por um aumento progressivo da curvatura e afinamento da córnea, resultando em astigmatismo irregular e diminuição da acuidade visual. Sua incidência na literatura atual é de aproximadamente 0,033%. O principal tratamento dessa complicação é o implante de anel intra-estromal com cross-linking. Neste artigo descrevemos um caso de uma mulher de 45 anos submetida a sucessivas tentativas de tratamento de ectasia pós LASIK, com implante de anel intra-estromal bilateral, sem sucesso em um dos olhos. Foi feito o uso do excimer laser topoguiado através do photorefractive keratectomy (PRK) sem o cross-linking, que optamos por não realizar pela estabilidade há anos, pelo risco alto de haze e pela ablação apenas na lamela (que era espessa).A paciente obteve melhorasignificativa da visão e da qualidade de vida. Mantemos o acompanhamento a cada seis meses desde então com a tomografia da córnea.

ABSTRACT The laser in situ keratomileusis (LASIK) is the most widely used refractory surgical method in the world with excellent results, but this method is not without risks. Post-LASIK ectasia is a serious complication and is characterized by a progressive increase in curvature and thinning of the cornea, resulting in irregular astigmatism and decreasing visual acuity. Its incidence in the current literature is approximately 0.033%. The main treatment for this complication is the implantation of an intra-stromal ring with crosslinking. In this article, we describe a case of a 45-year-old woman who underwent successive attempts to treat post-LASIK ectasia, with bilateral intra-stromal ring implantation, with no success in the eyes. Excimer laser was used, made through photorefractive keratectomy (PRK) without cross-linking and the patient obtained significant improvement in vision and in quality of life.

A Associação da Relação Proteína C-Reativa/Albumina em Pacientes com Ectasia da Artéria Coronária Isolada / Association of C-Reactive Protein to Albumin Ratio in Patients with Isolated Coronary Artery Ectasia

Resumo Fundamento A ectasia da artéria coronária (EAC) é definida como a dilatação difusa ou localizada do lúmen da artéria coronária com diâmetro de 1,5 a 2,0 vezes o diâmetro da artéria coronária normal adjacente. A relação proteína C-reativa/albumina (CAR, sigla em inglês) é um marcador inflamatório útil que tem sido documentado em doença arterial coronariana. Objetivo Analisar a associação entre a EAC e a CAR. Métodos Um protocolo caso-controle foi utilizado neste estudo. Foram incluídos 102 pacientesconsecutivos com EAC isolada sem estenose (56 homens e 46 mulheres; idade média de 60,4 ± 8,8 anos). O grupo controle era constituido pelo mesmo número de pacientes pareados por sexo e idade com artérias coronárias normais (55 homens e 47 mulheres; idade média de 61,2 ± 9,1 anos). Características clínicas, achados laboratoriais e histórico de uso de medicamentos foram registrados. Foram realizados teste t de Student, teste U de Mann-Whitney, teste do qui-quadrado, análise de regressão linear e logística. Foi considerado estatisticamente significativo p bilateral < 0,05. Resultados A CAR estava aumentada nos pacientes com EAC em comparação com os controles (32 e 16; p < 0,001). Além disso, foi verificado que a CAR era um preditor independente da EAC (razão de chances = 2,202; intervalo de confiança 95%, 1,184 - 5,365; p < 0,001). Conclusão No presente estudo, determinamos que os níveis da CAR estavam significativamente mais altos no grupo EAC que no grupo controle e a CAR estava significativamente correlacionada com a EAC. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0)

Abstract Background Coronary artery ectasia (CAE) is defined as diffuse or localized dilatation of coronary artery lumen with a diameter of 1.5 to 2.0 times the adjacent normal coronary artery. The C-reactive protein to albumin ratio (CAR) is a useful inflammatory marker, which has been documented in coronary artery disease. Objective To analyze the association of CAE and CAR. Methods A case-control protocol was used in this study. We included 102 consecutive patients with isolated CAE without stenosis (56 men and 46 women; mean age 60.4 ± 8.8 years). The control subjects consisted of an equal number of sex and age matched patients with normal coronary arteries (55 men and 47 women; mean age 61.2 ± 9.1 years). Clinical features, laboratory findings, and medication use history were recorded. Student's t test, Mann-Whitney U test, chi-square test, and linear and logistic regression analysis were performed. A 2-sided p < 0.05 was statistically considered significant. Results The CAR was increased in patients with CAE compared to the controls (32 and 16; p < 0.001). In addition, the CAR was found to be an independent predictor of CAE (OR = 2.202; 95% CI 1.184 - 5.365; p < 0.001). Conclusion In the present study, we determined that CAR levels were significantly higher in the CAE group than in the control group, and the CAR was significantly correlated with CAE. (Arq Bras Cardiol. 2020; [online].ahead print, PP.0-0)

Infarto agudo de miocardio secundario a enfermedad coronaria ectásica y agenesia de la arteria coronaria derecha / Acute myocardial infarction secondary to coronary artery ectasia and right coronary artery agenes

Se presenta el caso de un paciente de sexo masculino de 42 años de edad que cursa con episodio de infarto agudo del miocardio sin elevación del segmento ST, en quien se documenta enfermedad coronaria ectásica moderada a grave y como hallazgo incidental agenesia de la arteria coronaria derecha, entidad poco frecuente. Se realiza una revisión de los aspectos más relevantes de la literatura en ectasia coronaria y una breve descripción del hallazgo incidental.

Herein, we report a 42-year-old male patient with acute myocardial infarction presenting without ST-segment elevation. Moderate to severe coronary ectasia was documented, and a rare case of right coronary artery agenesis was incidentally detected. A literature review on the most relevant aspects of coronary ectasia and a brief description of the incidental finding were conducte.

Diagnóstico do ceratocone: um artigo de revisão / A review of keratoconus diagnosis

Resumo Objetivo: Realizar uma revisão sobre o diagnóstico do ceratocone, com enfoque nos métodos propedêuticos disponíveis. Métodos: Foi realizada uma pesquisa no banco de dados PubMed com as palavras chave: Keratoconus, diagnosis, topography e tomography. Por se tratar de uma revisão, não houve restrição de período para a publicação dos artigos selecionados. Foram também utilizados o manual Prefered Practice Pattern(PPP) da academia americana de oftalmologia, assim como o site "eyewiki.aao.org". Resultados: Dos 641 artigos encontrados no PubMed, assim como os usados como referência para o PPP, 36 foram selecionados por serem considerados mais relevantes para o tema proposto. O site "eyewiki.aao.org" foi utilizado como referência para as figuras. Conclusão: O diagnóstico do ceratocone evoluiu consideravelmente desde quando foi primeiramente descrito. É desejável que seja feito em suas fases precoces devido ao alto potencial de morbidade desta doença. Uma possível integração entre os múltiplos índices diagnósticos, investigação genética, biologia molecular e inteligência artificial é almejado para uma maior acurácia diagnóstica.

Abstract Objective: To Perform a review on the diagnosis of keratoconus, focusing on the available propaedeutic methods. Methods: A search was performed in the PubMed database using the key words: Keratoconus, diagnosis, topography and tomography. As it is a review, there was no restriction regarding the publication period of the selected articles. Furthermore, both the preferred practice pattern(PPP) manual of the American Academy of Ophthalmology, as well as the website "eyewiki.aao.org" were used as reference. The project was submitted to the research ethics committee of the Federal University of São Paulo / UNIFESP / SP 2018 (# 2,568,770). Results: Out of the 641 papers found in PubMed, in adition to those used as a reference for PPP, 36 were selected while considered more relevant to the adopted theme. The website "eyewiki.aao.org" was used as a reference for the images. Conclusion: The diagnosis of keratoconus has evolved considerably since it was first described. It is desirable to diagnose it on the early stages due to its high potential of morbility. A possibility of an integration between the various diagnostic indices, genetic research, molecular biology and artificial intelligence is recommended for greater diagnostic accuracy.

Atypical Virchow-Robin SpacesMimicking Cystic Primary Brain Tumor ­ Clinical Report and Literature Review

The Virchow-Robin spaces (VRSs), which are often incidentally observed in modern structural neuroimaging examinations, are small cystic cavities that usually surround the small arteries and arterioles at the level of basal ganglia, the anterior perforated substance and the thalamic-mesencephalic junction. Typically, they have similar physicochemical characteristics to cerebral spinal fluid (CSF) and there is no contrast enhancement on brain CT andMRI images. Its real meaning is unknown, although some contemporary studies have suggested that it might be related to certain traumatic brain injury or several other central nervous system (CNS) disorders, as degenerative diseases. Occasionally, some wide and atypical VRS may be mistaken for primary cystic brain tumors, especially in the context of large and symptomatic lesions, multiple clustered cysts, cortical lesions and if there is adjacent reactive gliosis. The present paper reports four patients who were affected by atypical VRS mimicking brain tumors that required imaging follow-up or even a biopsy to confirm the diagnosis or to indicate the correct approach. Although it is not so unusual, one of them occurred concomitantly and adjacent to a diffuse glioma (co-deleted 1p19q, WHO-GII).

Corneal suture for acute corneal hydrops secondary to post-LASIK ectasia: a case report / Sutura corneana em hidrópsia aguda secundária à ectasia pós LASIK: relato de caso

ABSTRACT Corneal ectasia is one of the main complications of keratorefractive procedures. In this report, we describe a case of corneal ectasia after laser-assisted in situ keratomileusis), which progressed with acute hydrops and aqueous leakage and required a suture for correction.

RESUMO A ectasia corneana é uma das principais complicações das cirurgias refrativas. Neste caso, descrevemos um caso de ectasia corneana induzida por laser-assisted in situ keratomileusis, que evoluiu com hidrópsia aguda e extravasamento de humor aquoso, necessitando de sutura corneana.

Enfermedad aneurismática coronaria / Coronary aneurysm disease

Resumen La enfermedad aneurismática coronaria se define como la dilatación de un segmento arterial coronario más 1,5 veces el diámetro de las arterias adyacentes de tamaño normal. Puede ser difusa, y afectar todo el trayecto de la arteria, o localizada y afectar sólo un segmento arterial específico. Puede encontrarse entre un 3 a un 8% de los estudios angiográficos y entre un 0,22% a un 1,4% de las necropsias clínicas. Se presenta 3 reportes de casos, donde la presentación clínica, los hallazgos angiográficos y el manejo farmacológico es diverso como corresponde a esta entidad.

Abstract Coronary aneurysm disease is defined as the dilation of a coronary artery segment more than 1.5 times the diameter of the adjacent arteries of normal size. It may be diffuse, and affect the whole trajectory of the artery, or localised and only affect a specific arterial segment. It can be found in between 3% and 8% of angiograph studies, with between 0.22% and 1.4% in clinical post-mortems. Three case reports are presented in which the clinical presentation, the angiographic findings, and the pharmacological management are shown to be as diverse as this condition itself.

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.

Giant aneurysm of the non-coronary sinus of Valsalva

Abstract Aneurysms of the sinuses of Valsalva are defined as dilatation of the aortic root region between the aortic annulus and the sinotubular junction. Isolated aneurysms of the sinus of Valsalva are rare cardiovascular pathologies. They may be congenital, especially secondary to connective tissue disorders or in conjunction with congenital cardiac defects, or acquired such as secondary to infections or trauma. Small sized aneurysm without rupture in asymptomatic patients may be followed; however, latter cases require intervention and surgery is the gold standard treatment modality. In this report, a 41-year-old male patient was reported with giant aneurysm of the non-coronary sinus of Valsalva whom underwent aortic root sparing surgical aortic sinus of Valsalva reconstruction.