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.

Coronary Artery Dilation in Children with Febrile Exanthematous Illness without Criteria for Kawasaki Disease / Dilatação das Artérias Coronárias em Crianças com Doença Exantemática Febril sem Critérios para a Doença de Kawasaki

Abstract Background: Coronary dilatation is the most important complication of Kawasaki disease (KD) and, in addition to some clinical characteristics, is common to KD and febrile exanthematous illnesses (FEIs). Objective: To assess whether children with FEI, who do not meet the criteria for KD, have changes in coronary arteries dimensions. Methods: Echocardiography was performed within the first two weeks of the disease in patients < 10 years with fever and exanthema without other KD criteria. To make a comparison with KD patients, we reviewed the echocardiograms and medical records of patients with a diagnosis of KD of the last five years. Coronary ectasia was assessed using Z scores of coronary arteries. The means of the dimensions of the coronary arteries were compared with a z test and a level of significance of 0.05 was adopted. Results: A total of 34 patients were included, 22 (64.7%) with FEI, and 12(35.2%) with a diagnosis of KD. Using the Z scores of coronary artery, a dilation of any of the coronary artery branches was observed in six (27.2%) patients with FEI. Conclusions: An important percentage of patients with FEI has coronary artery dilation.

Resumo Fundamento: A dilatação das artérias coronárias é a principal complicação da Doença de Kawasaki (DK) e, além de algumas características clínicas, é comum à DK e a doenças exantemáticas febris (DEFs). Objetivo: Avaliar se crianças com DEF e que não têm critério para DK apresentam alterações nas dimensões das artérias coronárias. Métodos: Foi realizada ecocardiografia nas primeiras duas semanas da doença em crianças com idade inferior a 10 anos, que apresentaram febre e exantema e nenhum outro critério de DK. Para comparar com pacientes com DK, fizemos a revisão de ecocardiogramas e prontuários médicos de pacientes com diagnóstico de DK dos últimos cinco anos. Ectasia coronária foi avaliada usando escore Z das artérias coronárias. As médias das dimensões das artérias coronárias foram comparadas pelo teste z, e um nível de significância de 0,05 foi adotado. Resultados: Foram incluídos no estudo 34 pacientes, 22 (64,7%) com diagnóstico de DEF e 12 (35,2%) com diagnóstico de DK. Usando o escore Z das artérias coronárias, observou-se dilatação em algum dos ramos da artéria coronária em seis (27,2%) pacientes com DEF. Conclusão: Uma porcentagem importante dos pacientes com DEFs apresenta dilatação das artérias coronárias.

Value of Contrast Transesophageal Echocardiography in the Detection of Intrapulmonary Vascular Dilatations in Hepatosplenic Schistosomiasis / Valor do Ecocardiograma Transesofágico com Contraste no Diagnóstico da Dilatação Vascular Intrapulmonar na Esquistossomose Hepatoesplênica

Abstract Background: Hepatopulmonary syndrome (HPS), found in cirrhotic patients, has been little studied in hepatosplenic schistosomiasis (HSS) and includes the occurrence of intrapulmonary vascular dilatations (IPVD). Contrast transesophageal echocardiography (cTEE) with microbubbles is more sensitive than contrast transthoracic echocardiography (cTTE) with microbubbles in the detection of IPVD in cirrhosis. Objective: To assess the performance of the cTEE, compared with that of cTTE, in detecting IPVD for the diagnosis of HPS in patients with HSS. Methods: cTEE and cTTE for investigation of IPVD and laboratory tests were performed in 22 patients with HSS. Agitated saline solution was injected in peripheral vein during the cTEE and cTTE procedures. Late appearance of the microbubbles in the left chambers indicated the presence of IPVD. Results of the two methods were compared by the Student's t-test and the chi-square test (p < 0.05). Results: cTEE was performed in all patients without complications. Three patients were excluded due to the presence of patent foramen ovale (PFO). The presence of IPVD was confirmed in 13 (68%) of 19 patients according to the cTEE and in only six (32%, p < 0.01) according to the cTTE. No significant differences in clinical or laboratory data were found between the groups with and without IPVD, including the alveolar-arterial gradient. The diagnosis of HPS (presence of IPVD with changes in the arterial blood gas analysis) was made in five patients by the cTEE and in only one by the cTTE (p = 0.09). Conclusion: In HSS patients, cTEE was safe and superior to cTTE in detecting IPVD and allowed the exclusion of PFO.

Resumo Fundamento: A síndrome hepatopulmonar (SHP), presente em pacientes cirróticos, é pouco estudada na esquistossomose hepatoesplênica (EHE) e inclui a ocorrência de dilatações vasculares intrapulmonares (DVP). O ecocardiograma transesofágico com contraste (ETEc) de microbolhas é mais sensível que o ecocardiograma transtorácico com contraste (ETTc) de microbolhas na identificação de DVP na cirrose. Objetivo: Avaliar o desempenho do ETEc comparado ao ETTc na identificação de DVP para diagnóstico de SHP em pacientes com EHE. Métodos: Incluímos 22 pacientes com EHE submetidos a ETEc e ETTc para pesquisa de DVP, além de exames laboratoriais. Os ETEc e ETTc foram realizados empregando-se solução salina agitada, injetada em veia periférica. A visualização tardia das microbolhas em câmaras esquerdas indicava presença de DVP. Os resultados foram comparados entre os dois métodos pelos testes t de Stu dent e qui-quadrado (significância p < 0,05). Resultados: Todos os 22 pacientes realizaram ETEc sem intercorrências. Foram excluídos três pela presença de forame oval patente (FOP), e a análise final foi realizada nos outros 19. A DVP esteve presente ao ETEc em 13 pacientes (68%) e em apenas seis ao ETTc (32%, p < 0,01). Não houve diferenças significativas nos dados clínicos e laboratoriais entre os grupos com e sem DVP, incluindo a diferença alveoloarterial de oxigênio. O diagnóstico de SHP (presença de DVP com alterações gasométricas) ocorreu em cinco pacientes pelo ETEc e em apenas um pelo ETTc (p = 0,09). Conclusão: Em pacientes com EHE, o ETEc foi seguro e superior ao ETTc na detecção de DVP não identificada ao ETTc, o que possibilitou adicionalmente excluir FOP.

Síndrome de Ehlers-Danlos hipermóvel: um relato de caso / Hypermobile Ehlers-Danlos syndrome: a case report

A síndrome de Ehlers-Danlos é estabelecida por distúrbios hereditários do tecido conjuntivo que tem como manifestações principais a hipermobilidade articular, a hiperextensibilidade da pele e a fragilidade de tecidos, como articulações, ligamentos, pele, vasos sanguíneos e órgãos internos. São reconhecidos 13 subtipos, de acordo com Classificação Internacional de 2017. Dentre estes, abordamos o hipermóvel, cujo diagnóstico é eminentemente clínico, com manifestações sistêmicas distintas. Esse artigo refere-se ao caso de uma paciente diagnosticada com síndrome de Ehlers-Danlos hipermóvel, tendo como intuito a atualização acerca dos novos critérios diagnósticos, assim como o diagnóstico precoce de tal raropatia.

Ehlers-Danlos syndrome is established through hereditary disorders of connective tissue, and has as its manifestations: joint hypermobility, skin hyperextensibility, and fragility of tissues such as joints, ligaments, skin, blood vessels, and internal organs. Thirteen subtypes have been recognized according to the 2017 International Classification. Among these, the hypermobile type, the diagnosis of which is eminently clinical, with distinct systemic manifestations, will be addressed. This article refers to the case of a patient diagnosed with hypermobile Ehlers-Danlos syndrome, with the objective of updating the new diagnostic criteria, as well as the early diagnosis of such a rare disease.

Aneurisma gigante de la arteria coronaria derecha, reporte de un caso y revisión de la literatura / Giant aneurysm of the right coronary artery, report of a case and review of the literature

Resumen Los aneurismas de las arterias coronarias se definen como una dilatación localizada que excede el diámetro normal en 1.5 veces. Esta es una condición poco frecuente, su incidencia varía del 0.3 hasta el 5.3% de las angiografías coronarias. Los aneurismas que exceden cuatro veces el diámetro del vaso normal se consideran gigantes. Estos son aún más raros y se presentan en el 0.02 a 0.2% de todos los casos. Existe controversia en cuanto a su fisiopatología, sin embargo, hasta el 50% de los casos se relacionan con la aterosclerosis. Se diagnostican más frecuentemente entre la sexta y séptima décadas de vida. Las principales manifestaciones clínicas están relacionadas con la cardiopatía isquémica. Respecto a su tratamiento, no existe un consenso del manejo en los pacientes adultos, las opciones son: médico, quirúrgico o intervencionismo. Reportamos la presencia de un aneurisma gigante de la coronaria derecha y ectasia gigante del sistema izquierdo con trombosis activa en un hombre con antecedentes de un aneurisma en la aorta abdominal, tratado por vía endovascular, e infarto agudo al miocardio sin elevación del segmento ST no reperfundido. Requirió de estudio de angiotomografía coronaria, el cual permitió la identificación de las características anatómicas de esta enfermedad.

Abstract Coronary artery aneurysms are described as a localized dilatation that exceeds the normal diameter by 1.5 times. This is a rare condition; its incidence varies from 0.3% up to 5.3% of all coronary angiographies. Those aneurysms that exceed 4 times the diameter of a normal artery are considered giant aneurysms, which are even more uncommon, presenting between 0.02% and 0.2% of all cases. There is controversy regarding its pathophysiology, however, up to 50% of the cases are related to atherosclerosis. They are diagnosed more frequently between the sixth and seventh decade of life. The main clinical manifestations are related to ischemic heart disease. Regarding their treatment, there is no general consensus towards its management in adult patients. The options are medical, surgical or percutaneous treatment. We report the presence of a giant aneurysm of the right coronary artery and giant ectasia of the left coronary system with active thrombosis in a man with history of an abdominal aortic aneurysm, with endovascular treatment and a non-ST segment elevation myocardial infarction with no reperfusion strategy, who required a coronary computed tomography, identifying the anatomical characteristics of this disease.

Comparative evaluation of magnetic resonance cholangiopancreatography and perioperative cholangiography in patients with suspect choledocholithiasis / Avaliação comparativa da colangiopancreatografia por ressonância magnética e da colangiografia peroperatória na suspeita de coledocolitíase

ABSTRACT Background: Cholelithiasis is a highly prevalent condition, and choledocholitiasis is a high morbidity complication and requires accurate methods for its diagnosis. Aim: To evaluate the population of patients with suspected choledocholitiasis and check the statistical value of magnetic resonance cholangiopancreatography, ultrasonography, the laboratory and the clinic of these patients comparing them to the results obtained by perioperative cholangiography. Methods: This is a retrospective cohort study, which were evaluated 76 patients with cholelithiasis and suspected choledocholithiasis. Results: It was observed that the presence of dilatation of the biliary tract or choledocholithiasis in the ultrasonography was four and eight times increased risk of perioperative cholangiography for positive choledocholithiasis, respectively. For each unit increased in serum alkaline phosphatase was 0.3% increased the risk of perioperative cholangiography for positive choledocholithiasis. In the presence of dilatation of the bile ducts in the ultrasonography was four times greater risk of positive magnetic resonance cholangiopancreatography for choledocholithiasis. In the presence of pancreatitis these patients had five times higher risk of positive magnetic resonance cholangiopancreatography for choledocholithiasis. On the positive magnetic resonance cholangiopancreatography presence to choledocholithiasis was 104 times greater of positive perioperative cholangiography for choledocholithiasis. Conclusions: The magnetic resonance cholangiopancreatography is a method with good accuracy for propedeutic follow-up for the diagnosis of choledocholithiasis, consistent with the results obtained from the perioperative cholangiography; however, it is less invasive, with less risk to the patient and promote decreased surgical time when compared with perioperative cholangiography.

RESUMO Racional: A colelitíase é afecção de alta prevalência, sendo a coledocolitíase complicação de elevada morbidade e que necessita de métodos acurados para seu diagnóstico. Objetivo: Avaliar o perfil populacional de pacientes com suspeita de coledocolitíase e verificar o valor estatístico da colangiopancreatografia por ressonância magnética, da ultrassonografia, dos exames laboratoriais e da clínica desses pacientes e compará-los aos resultados obtidos pela colangiografia peroperatória. Métodos: Trata-se de estudo longitudinal, de coorte, retrospectivo, no qual foram avaliados 76 pacientes com diagnóstico de colelitíase e suspeita de coledocolitíase. Resultados: Observou-se que na presença de dilatação das vias biliares ou coledocolitíase na ultrassonografia havia risco quatro e oito vezes maior, respectivamente, de colangiografia peroperatória positiva para coledocolitíase. Para cada unidade de aumento na fosfatase alcalina sérica houve aumento em 0,3% no risco de colangiografia peroperatória positiva para coledocolitíase. Na presença de dilatação das vias biliares na ultrassonografia ou clínica de pancreatite havia risco quatro e cinco vezes maior, respectivamente, de colangiopancreatografia por ressonância magnética positiva para coledocolitíase. Na presença de colangiopancreatografia por ressonância magnética positiva para coledocolitíase o risco foi 104 vezes maior de colangiografia peroperatória positiva para coledocolitíase. Conclusão: A colangiopancreatografia por ressonância magnética para seguimento propedêutico tem boa acurácia para o diagnóstico de coledocolitíase, e concordante com os resultados obtidos na colangiografia peroperatória. O método é menos invasivo, com menores riscos ao paciente e com diminuição do tempo cirúrgico dispendido para realização da colangiografia peroperatória.

Dilatação fluxo-mediana da artéria braquail: aspectos técnicos, aplicabilidade e reprodutividade em ginecologia e obstetrícia / Flow-mediated dilatation of brachial artery: technical aspects, applicability and reproducibility in gynecology and obstetrics

A avaliação da dilatação fluxo-mediada da artéria braquial (DILA) tem-se incorporada na prática clínica como método capaz de contribuir no estudo das doenças arterioescleróticas. Estudos demonstraram que os valores de DILA são reduzidos em pacientes com pré-eclâmpsia, e que a redução desses valores está diretamente ligada à morbidade da doença. Outros estudos correlacionam diabetes gestacional e disfunção endotelial com redução do DILA nestas pacientes. Ainda, a terapia de reposição hormonal tem sido demonstrada como fator de melhora nos valores do DILA em pacientes na pós-menopausa. O objetivo deste estudo foi realizar uma revisão bibliográfica sobre a utilização do DILA em Ginecologia e Obstetrícia. Foi realizado um levantamento bibliográfico nas bases da Bireme, Scielo e PubMed, de trabalhos publicados entre 2013 e 2017. Podemos concluir que métodos não invasivos e de fácil execução, como o DILA, podem ter impactos relevantes ao identificar grupos de riscos e, assim, viabilizar medidas protetivas.(AU)

The evaluation of the flow-mediated dilation of the brachial artery (FMD) has been incorporated into clinical practice as a method capable of contributing to the study of arteriosclerotic diseases. Studies have shown that FMD values are reduced in patients with preeclampsia, and that the reduction in these values is directly associated with disease morbidity. Other studies correlate gestational diabetes and endothelial dysfunction with reduction of FMD in these patients. Furthermore, hormone replacement therapy has been demonstrated as an improvement fator in FMD values in postmenopausal patients. The objective of this study was to perform a bibliographic review on the use of FMD in Gynecology and Obstetrics. A bibliographical survey was carried out on the Bireme, Scielo and PubMed databases, of works published between 2013 and 2017. We can conclude that non-invasive and easy-to-execute methods, such as FMD, can have relevant impacts when identifying gropups of risk and thus make feasible measures protective.(AU)

Avaliação das dimensões da aorta torácica ao ecocardiograma transtóracico bidimensional: correlação com fatores de risco cardiovasculares / Transthoracic echocardiographic assessment of thoracic aorta: correlation with cardiovascular risk factors

Fundamentos: As dimensões da aorta torácica têm tido fraca correlação com fatores de risco cardiovasculares, como hipertensão arterial sistêmica (HAS), diabetes mellitus (DM) e doença arterial coronariana (DAC). Objetivos: Correlacionar o diâmetro da aorta torácica avaliado pelo ecocardiograma transtorácico (ETT) com HAS, DM, dislipidemia, DAC, tabagismo, idade, sexo, peso, altura, índice de massa corpórea (IMC) e superfície corporal (SC), além de uso contínuo de medicamentos com ação protetora cardiovascular. Métodos: Estudo observacional, transversal e retrospectivo. Foram selecionados 203 indivíduos (62,1 ± 15,3 anos; 57,1% do sexo feminino) que realizaram ETT com a avaliação da aorta torácica em 6 sítios: (1) anel valvar aórtico; (2) seio de valsalva; (3) junção sino tubular; (4) aorta proximal ascendente; (5) arco aórtico e (6) aorta descendente. Resultados: Idade (p < 0,05), sexo masculino (p < 0,001), peso (p < 0,001), altura (p < 0,05) e SC(p < 0,001) foram associados a maiores diâmetros da aorta torácica em todos os sítios de avaliação. A análise multivariada identificou que idade, sexo masculino e SC, conjuntamente, explicam a variação dos diâmetros no anel aórtico em 17,3%, no seio de valsalva, em 30,7%, na junção sino tubular em 17,7%, na ascendente proximal em 21,9%, no arco aórtico em 19,8% e na aorta descendente em 21,4%. Não houve associação entre os diâmetros da aorta e os fatores de risco avaliados e uso contínuo de betabloqueadores, inibidores da enzima conversora de angiotensina ou bloqueadores dos receptores da angiotensina. Conclusões: Idade, sexo masculino e superfície corpórea se correlacionaram positiva e significativamente com os diâmetros da aorta torácica

Background: Thoracic aorta dimensions have been poorly correlated with cardiovascular risk factors such as systemic arterial hypertension (SAH), diabetes mellitus (DM) and coronary artery disease (CAD). Objectives: To correlate the thoracic aorta diameter assessed by transthoracic echocardiography (TTE) with SAH, DM, dyslipidemia, CAD, smoking, age, gender, weight, height, body mass index (BMI) and body surface area (BSA) and continuous use of drugs with cardiovascular protective action. Methods: Observational, cross-sectional and retrospective study. The study included 203 individuals (62.1 ± 15.3 years of age; 57.1% female) who underwent TTE with thoracic aorta evaluation at 6 sites: (1) aortic valve annulus; (2) sinus of Valsalva; (3) sinotubular junction; (4) ascending proximal aorta; (5) aortic arch and (6) descending aorta.Results: Age (p < 0.05), male gender (p < 0.001), weight (p < 0.001), height (p < 0.05), and BSA (p < 0.001) were associated with greater thoracic aorta diameters at all sites evaluated. Multivariate analysis identified that age, male gender and BSA, together, explain the variation of aortic annulus diameters in 17.3%, in the sinus of Valsalva in 30.7%, in the sinotubular junction in 17.7%, in the proximal ascending aorta in 21.9%, in the aortic arch in 19.8% and in the descending aorta in 21.4%. There was no association between aortic diameters and the risk factors assessed and continuous use of beta-blockers, angiotensin-converting enzyme inhibitors or angiotensin receptor blockers. Conclusions: Age, male gender and body surface area correlated positively and significantly with the thoracic aorta diameters

Prenatal natural history of isolated fetal mild bilateral pyelectasis

OBJECTIVE: To analyze the prenatal outcomes in a cohort of fetuses with mild bilateral pyelectasis and determine whether performing serial ultrasounds is a good follow-up strategy. METHODS: A prospective longitudinal study was conducted on 62 fetuses with mild bilateral pyelectasis. Fetal mild bilateral pyelectasis was considered when the renal pelvis measured (in millimeters) ≥5.0 to 10.0, ≥7.0 to 10.0, and ≥10.0 to 15 at ≤23 weeks 6 days, 24 to 31 weeks 6 days, and ≥32 weeks, respectively, with no uretero-calyceal dilatation. Ultrasounds were performed every 3 weeks to assess whether the mild bilateral pyelectasis regressed, remained unchanged (Group 1) or progressed (Group 2). RESULTS: Group 1 consisted of 53 fetuses (85.4%), and progression was observed in 9 cases (Group 2, 14.6%). The initial renal pelvis diameter was significantly larger in fetuses with progression (p=0.028). Statistically significant differences in the renal pelvis diameter were also found at weeks 31 and 35 for both kidneys (p<0.05). The cases requiring intrauterine procedures or early delivery were not observed. CONCLUSION: Fetal mild bilateral pyelectasis with no calyceal dilatation is a benign condition that can be managed in the postnatal period. The initial renal pelvis diameter and the diameter in week 31 or 35 were valuable parameters for identifying cases that would eventually need specific postnatal procedures.

La coronarioectasia difiere en los factores de riesgo clásicos de enfermedad coronaria / Compared to usual coronary artery disease risk factors are different in those with coronary ectasia

Introducción: La coronarioectasia (CE), es una infrecuente forma de enfermedad coronaria, en que dilataciones coronarias coexisten con placas ateroes-cleróticas. Puede presentarse como cuadros agudos o crónicos, aún sin estenosis significativa. Distintas series lo han asociado a perfiles variados de factores de riesgo cardiovascular. Métodos: Se efectuó un estudio analítico de cohorte retrospectivo, evaluando las coronariografías realizadas en nuestro centro entre Junio de 2009 a Julio de 2015. Se definió CE como dilatación >1,5 veces comparado con el diámetro de la arteria de referencia. Se estudiaron factores de riesgo cardiovascular clásicos y se compararon con un grupo control elegido de forma aleatoria. Resultados: De 9648 coronariografías, 64 presentaban CE. La mayor parte eran hombres, de menor edad, con menos hipertensión arterial, diabetes e hiperlipidemia, comparados con los controles. En la mayoría de los casos la CE afectaba a 3 vasos (83,3%) y en solo 5 casos (28%) coexistía con estenosis. El análisis multivariado señaló como factores de riesgo significativos a la edad < 55 años (OR: 2,63, IC: 1,4 -4,9, p<0,05), Obesidad (OR: 3,2; IC:1,7-5,8, p<0,05) e Hiperlipidemia (OR: 0,09; IC: 0,016-0,54). Considerando los pacientes que se presentaron como SCA se observó que los pacientes con CE fueron más jóvenes (45,9 años; DE: 5,9 v/s 48,8 años; DE: 5,3; p=0,02), y con menos hiperlipidemia (OR:0,2; IC:0,06-0,7, p=0,01). Respecto a la obesidad, esta fue más preva-lente en pacientes con CE (OR: 2,49; IC: 0,956-6,4. p=ns). Conclusión: La CE es una entidad poco frecuente, que puede producir SCA aun en ausencia de estenosis significativa. Son pacientes más jóvenes y con menos antecedente de dislipidemia, por lo que en su patogenia aparentemente participan factores diferentes a los de la enfermedad ateroesclerótica obstructiva.

Background: Coronary ectasia (CE) is an uncommon condition where coronary artery dilatation coexists with atherosclerotic plaques. It may present as either acute or chronic syndromes even in the absence of coronary artery stenosis. Differences in risk factors associated to CE compared to those associated to usual CAD have been described. Methods: We retrospectively analyzed coronary arteriograms performed between June 2009 and July 2015. CE was defined as the presence of dilatation >1.5 times the diameter of the unaffected vessel. Cardiovascular risk factors were compared in CE vs a random sample of non-CE patients. Results: Out of 9648 coronary arteriograms 64 showed CE (9.5%). Compared to controls, CE patients were males, younger and hat lower prevalence or hypertension, diabetes and hyperlipidemia. CE was present in all 3 main vessels in 83.3% of CE patients and co-existed with significant stenosis in only 28%. Multivariate analysis showed that significant differences in risk factors were age <55 years (OR: 2.63; CI: 1.4 to 4.9, p <0.05), obesity (OR: 3 2; CI: 1.7 to 5.8, p <0.05) and hyperlipidemia (OR: 0.09; CI: 0.016 to 0.54). In patients presenting with an acute coronary syndrome, those with CE were younger (45,9 years; SD: 5,9 v/s 48,8 years; SD: 5,3; p=0,02), y and a lower prevalence or hyperlipidemia (OR:0,2; IC:0,06-0,7, p=0,01). Conclusion: CE is an infrequent condition in CAD. It may me associated to either acute or chronic syndromes. They are younger, have a lower prevalence of dyslipidemia suggesting that risk factors other than traditionally recognized in obstructive CAD influence de development of CE.

Internal Jugular Vein Phlebectasia : A Rare Case of Lateral Neck Swelling.

Internal Jugular vein Phlebectasia (IJP) is a rare disease in which there is a fusiform or saccular dilatation of Internal Jugular vein, usually presenting as a compressible neck mass that becomes prominent on coughing, straining or Valsalva manoeuvre. Colour Doppler is the non invasive diagnostic tool which clinches the diagnosis. It is important to keep this diagnosis in mind in case of lateral neck swellings to avoid invasive investigations and inappropriate treatment as management of asymptomatic case is conservative.

Unilateral internal jugular phlebectasia.

Jugular phlebectasia is a rare cause of cervical swelling in children. It is a fusiform dilatation of any part of the jugular venous system and can involve the external, internal or anterior jugular veins. Previous reports suggest that the entity is often ignored or misdiagnosed. Unilateral internal jugular phlebactasia presenting as an intermittent neck swelling in a ten-year-old girl is reported. The clinical features are analyzed and the appropriate use of noninvasive imaging modalities is highlighted. The literature is also briefly reviewed.

Hepatopulmonary Syndrome Induced by Common Bile Duct Ligation in a Rabbit Model: Correlation between Pulmonary Vascular Dilatation on Thin-Section CT and Angiography and Serum Nitrite Concentration or Endothelial Nitric Oxide Synthase (eNOS) 1 Expression

OBJECTIVE: To investigate the correlation between radiologic vascular dilatation and serum nitrite concentration and eNOS expression in the endothelial cell and pneumocyte in a rabbit model of hepatopulmonary syndrome induced by common bile duct ligation (CBDL). MATERIALS AND METHODS: Thin-section CT scans of the lung and pulmonary angiography were obtained 3 weeks after CBDL (n=6), or a sham operation (n=4), and intrapulmonary vasodilatation was assessed. The diameter and tortuosity of peripheral vessels in the right lower lobe by thin-section CT and angiography at the same level of the right lower lobe in all subjects were correlated to serum nitrite concentration and eNOS (endothelial nitric oxide synthase) expression as determined by immunostaining. RESULTS: The diameters of pulmonary vessels on thin-section CT were well correlated with nitrite concentrations in serum (r = 0.92, p < 0.001). Dilated pulmonary vessels were significantly correlated with an increased eNOS expression (r = 0.94, p < 0.0001), and the severity of pulmonary vessel tortuosity was found to be well correlated with serum nitrite concentration (r = 0.90, p < 0.001). CONCLUSION: The peripheral pulmonary vasculature in hepatopulmonary syndrome induced by CBLD was dilated on thin-section CT and on angiographs. Our findings suggest that peripheral pulmonary vascular dilatations are correlated with serum nitrite concentrations and pulmonary eNOS expression.