RESUMEN
An 81-year-old male presented with a soft mass on his neck noted in the supine position. After undergoing an operation for an inguinal hernia, the mass was noted to persist regardless of the patient's position. Computed tomography and magnetic resonance imaging revealed phlebectasia of the external jugular vein with an associated thrombosis. Under general anesthesia, the external jugular vein was ligated and removed, along with the thrombus. A histological section revealed that the tunica media of the external jugular vein was absent, and the smooth muscle layer and elastic fibers were attenuated in the fusiform area of the vein. Due to the risk of propagation of the clot and pulmonary embolism, resection of the external jugular vein is advised in cases of phlebectasia of the external jugular vein.
Asunto(s)
Venas Yugulares/cirugía , Trombectomía/métodos , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/cirugía , Anciano de 80 o más Años , Humanos , Venas Yugulares/anomalías , Venas Yugulares/patología , Ligadura , Imagen por Resonancia Magnética , Masculino , Músculo Liso Vascular/patología , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Túnica Media/anomalías , Tromboembolia Venosa/patologíaRESUMEN
CHF1/Hey2 null mice generated in different laboratories have discrepant cardiovascular phenotypes. To determine the effect of genetic background on phenotype, we backcrossed our knockout strain more than eight generations to the inbred strains BALB/c and C57BL/6. Knockout mice on these backgrounds showed disparate phenotypes. Mice on both backgrounds demonstrated ventricular septal defects (VSDs), tricuspid stenosis and mitral valve thickening, but at varying frequencies, suggesting a general defect in endocardial cushion remodeling. Additional defects seen exclusively on the C57BL/6 background included biventricular wall thinning and left ventricular enlargement, implying a more severe myocardial defect than previously observed. In addition, aortas and pulmonary arteries from these null mice had thinner walls. Intercrossing of the CHF1/Hey2 null mice on a C57BL/6 background with a C57BL/6 MLC2v-CHF1/Hey2 transgenic line overexpressing CHF1/Hey2 in the atrial and ventricular myocardium also rescued the VSD and myocardial phenotypes, but did not affect vascular wall thickness. Our results indicate that CHF1/Hey2 provides an important myocardial signal to the endocardial cushion for proper septation and valve formation and also plays an important role in maturation of the myocardium and vasculature.
Asunto(s)
Factores de Transcripción con Motivo Hélice-Asa-Hélice Básico/deficiencia , Anomalías Cardiovasculares/genética , Epigénesis Genética , Animales , Aorta/anomalías , Factores de Transcripción con Motivo Hélice-Asa-Hélice Básico/genética , Defectos de la Almohadilla Endocárdica/genética , Eliminación de Gen , Corazón/crecimiento & desarrollo , Ventrículos Cardíacos/anomalías , Ratones , Ratones Endogámicos BALB C , Ratones Endogámicos C57BL , Ratones Noqueados , Válvula Mitral/anomalías , Mutación , Miocardio/metabolismo , Fenotipo , Proteínas Represoras/genética , Estenosis de la Válvula Tricúspide/genética , Túnica Media/anomalíasRESUMEN
The authors report a case of spontaneous dissection of both carotid arteries, followed by spontaneous dissection of the left anterior descending coronary artery after a few days. No major abnormalities of collagen production were found. This case may be underlain by a dysplasia of common precursors of the carotid and coronary walls.
Asunto(s)
Disección Aórtica/etiología , Disección de la Arteria Carótida Interna/etiología , Aneurisma Coronario/etiología , Túnica Media/anomalías , Adulto , Disección Aórtica/complicaciones , Disección de la Arteria Carótida Interna/diagnóstico por imagen , Linaje de la Célula , Colágeno/análisis , Anticonceptivos Orales Combinados/efectos adversos , Anticonceptivos Hormonales Orales/efectos adversos , Aneurisma Coronario/complicaciones , Angiografía Coronaria , Desogestrel/efectos adversos , Etinilestradiol/efectos adversos , Matriz Extracelular/química , Femenino , Humanos , Infarto del Miocardio/etiología , Cresta Neural/patología , Tomografía Computarizada EspiralRESUMEN
A 14-month-old infant was found to have a giant aneurysm of the ascending aorta at the time he underwent angiography. At the time of autopsy, a saccular chamber was found to take the place of the aorta. Histologic examination of the aneurysm revealed absence of the medial layer. Thus, medial genesis may be another cause of aneurysm of the ascending aorta.