ABSTRACT
No disponible
Subject(s)
Child , Female , Humans , CHARGE Syndrome/complications , Vestibular Diseases/diagnosis , Semicircular Canals/abnormalities , Ear/abnormalitiesABSTRACT
El síndrome del acueducto vestibular dilatado es la patología de herencia autosómica recesiva en la que se produce una hipoacusia progresiva o fluctuante desde la infancia por detención del desarrollo del oído interno, lo que provoca un aumento del saco endolinfático y una ligera displasia coclear. Presentamos 2 casos clínicos cuya peculiaridad es que ambos son unilaterales, ya que suelen ser bilaterales en el 90%de los casos. Una tomografía computarizada (TC) de cortes finos (1 mm)identifica al acueducto vestibular aumentado, pero una TC normal no excluye este diagnóstico, por lo que se debe realizar una resonancia magnética a todo paciente con hipoacusia neurosensorial unilateral para descartar ésta y otras alteraciones que pueden estar asociadas (AU)
Enlarged vestibular aqueduct syndrome is a clinical condition characterized for an autosomal recessive inheritance and a progressive perceptive deafness linked to a broadening of vestibular great aqueduct and a cochlear dysplasia. We report two clinical cases which peculiarity is that though they are usually bilateral in 90% of the cases, both cases which have been presented are unilateral. The enlarged vestibular aqueduct is detected by a CAT Scan (Computerized axial Tomography) of thin cuts (1 mm), but a normal CAT scan does not exclude this diagnosis. So a MRI (Magnetic Resonance Imaging) is needed in the diagnosis of unilateral neurosensorial hypoacusia to rule out this malformation and others that might be related (AU)
Subject(s)
Humans , Male , Female , Child , Magnetic Resonance Spectroscopy , Vestibular Aqueduct/physiopathology , Hearing Loss, Sensorineural/diagnosis , Hearing Loss, Sensorineural/surgery , Vestibular Aqueduct/transplantationABSTRACT
The development of knowledge of paragangliomas has been inextricably linked to the development of all fields of medicine. Throughout history, both knowledge of diseases and technical improvements in diagnosis have served to understand these tumors, although unanswered questions remain. From the XVIII century to the XXI century events sometimes unexpected and other times intensely sought have occurred that have helped to identify the characteristics of these tumors. These events have ranged from description of paragangliomas to their genetic diagnosis. The incidence of paragangliomas is not high. Nevertheless, the variability of these tumors in terms of their localization, forms of presentation, possibilities of metastasis or their hereditary component has been well characterized.
Subject(s)
Head and Neck Neoplasms/epidemiology , Head and Neck Neoplasms/history , Paraganglioma/epidemiology , Paraganglioma/history , History, 18th Century , History, 19th Century , History, 20th Century , HumansABSTRACT
El desarrollo del conocimiento de los paragangliomas ha ido inexorablemente unido al desarrollo de la medicina en todos sus campos. Tanto el propio conocimiento de las enfermedades como la mejora técnica en el diagnóstico han servido, a lo largo de la historia, para llegar a entender esta patología, que presenta, por otra parte, interrogantes sin resolver. Desde el siglo XVIII hasta el siglo XXI se han ido sucediendo acontecimientos, a veces ocasionales y a veces profundamente buscados, que han ido revelando cómo es esta patología, desde la descripción de la enfermedad hasta el diagnóstico genético. Su incidencia no es elevada pero, a pesar de ello, su variabilidad en cuanto a localización, formas de presentación, posibilidad de metastatizar o su componente hereditario está perfectamente estudiada (AU)
The development of knowledge of paragangliomas has been inextricably linked to the development of all fields of medicine. Throughout history, both knowledge of diseases and technical improvements in diagnosis have served to understand these tumors, although unanswered questions remain. From the XVIII century to the XXI century eventssometimes unexpected and other times intensely soughthave occurred that have helped to identify the characteristics of these tumors. These events have ranged from description of paragangliomas to their genetic diagnosis. The incidence of paragangliomas is not high. Nevertheless, the variability of these tumors in terms of their localization, forms of presentation, possibilities of metastasis or their hereditary component has been well characterized (AU)
Subject(s)
Humans , History, 18th Century , History, 19th Century , History, 20th Century , Head and Neck Neoplasms/epidemiology , Head and Neck Neoplasms/history , Paraganglioma/epidemiology , Paraganglioma/history , Portraits as TopicABSTRACT
No disponible
Subject(s)
Female , Child, Preschool , Humans , Torticollis/complications , Vertigo/complications , Torticollis/therapy , Vertigo/therapyABSTRACT
The third branquial arch anomalies represent less than 1% of this deformites. The clinical manifestation can be varied althouth the formation of cysts is the commonest one and may appear at any time in life. We report the clinical case of a 7 year old boy with an neck mass. This mass was diagnosed as a branquial cyst. The patologist found a "timic reminescences". We carry out a review of the literature for this pathology.
Subject(s)
Branchial Region/abnormalities , Branchial Region/pathology , Branchioma/pathology , Branchial Region/surgery , Branchioma/surgery , Child , Humans , Magnetic Resonance Imaging , MaleABSTRACT
Las anomalías derivadas del desarrollo del tercer arco y bolsa branquiales representan menos del 1% del total de estas malformaciones. Su forma de manifestación puede ser variada, aunque la más frecuente es la forma quística y pueden aparecer en cualquier etapa de la vida. Presentamos el caso clínico correspondiente a un paciente de 7 años con una tumoración laterocervical derecha de un año de evolución, diagnosticado de quiste branquial y con un estudio anatomopatológico tras la extirpación de restos tímicos. De igual modo realizamos una revisión de la literatura de estos casos
The third branquial arch anomalies represent less than 1% of this deformites. The clinical manifestation can be varied althouth the formation of cysts is the commonest one and may appear at any time in life. We report the clinical case of a 7 year old boy with an neck mass. This mass was diagnosed as a branquial cyst. The patologist found a "timic reminescences". We carry out a review of the literature for this pathology
Subject(s)
Male , Child , Humans , Branchial Region/abnormalities , Branchial Region/pathology , Branchioma/pathology , Branchial Region/surgery , Branchioma/surgery , Magnetic Resonance ImagingABSTRACT
Los condrosarcomas laríngeos son neoplasias poco frecuentes que suponen solamente un pequeño porcentaje de todos los tumores primarios de la laringe y que ha sido estimado en menos de un 2%. Con frecuencia, la clínica que presentan es anodina pese a su progresivo crecimiento hasta que la funcionalidad de la laringe se ve severamente comprometida. Esta neoplasia se caracteriza por una agresividad local variable y frecuentes metástasis, que comúnmente se localizan en pulmones y hueso, dependiendo del tipo histológico. Presentamos un caso de un condrosarcoma laríngeo agresivo con enfermedad metastásica exclusivamente cerebral como causa de muerte, hecho extremadamente infrecuente en estos tumores. Aportamos, además, una revisión de la literatura, analizando especialmente las controversias sobre el manejo de esta entidad
Chondrosarcomas of the larynx are rare neoplasms that account only for a very small percentage of all the primary laryngeal neoplasms that has been estimated in less than 2%. They often show initially with indolent symptoms despite their progressive growth until great compromise ofthe laryngeal function is produced. This neoplasm is characterised by a variable local aggressive behaviour and frequent of distant metastasis, that are commonly located in lungs and bone, deppending on the hystologic grade. A case of an aggressive laryngeal chondrosarcoma with exclusive brain metastasic disease as cause of death, a very uncommon event in these neoplasms, is reported. We also provide a review ofthe literature focusing on the controversies about the management of tlIis condition
Subject(s)
Humans , Chondrosarcoma/pathology , Laryngeal Neoplasms/pathology , Neoplasm Metastasis/pathology , Brain Neoplasms/secondary , Chondrosarcoma/therapy , Laryngeal Neoplasms/therapy , Neoplasm Metastasis/therapy , Brain Neoplasms/therapyABSTRACT
The parapharyngeal space-occupying lessions may develop from any of the structures located inside it. Uncommon although possible ethiologies for them are anatomic variations of the course of the internal carotid artery. These anatomic variants mainly arise from degenerative, mostly age-amplified, causes and lead to elongations, angulations, kinking and tortuosities in the course of the vessel that appear as impressions or protusions of the pharyngeal wall. Utherly unfrecuently lead to loops that may show as large parapharyngeal tumors. This fact should be noticed previously to any invasive procedure to be performed on the patient specially when evidenced in concommitance with an infectious process at the moment of diagnosis. A case of an impressive internal carotid artery loop that conditioned an important parapharyngeal mass in a patient with an associated pharyngeal infection is reported to warn about this rare parapharyngeal space-occupying condition and its potential danger.
Subject(s)
Carotid Artery, Internal/abnormalities , Pharyngeal Diseases/etiology , Aged , Aged, 80 and over , Female , HumansABSTRACT
Las tumoraciones del espacio parafaríngeo pueden originarse a partir de cualquiera de las estructuras que por él discurren. Una infrecuente aunque posible etiología de las mismas son las variantes anatómicas del trayecto de la arteria carótida interna. Dichas variantes anatómicas surgen generalmente de causas degenerativas y conducen a elongamientos, tortuosidades o suaves rizos que originan improntas en la pared faríngea. Muy raramente, se producen bucles que pueden originar importantes tumoraciones faríngeas. Este hecho debería ser reconocido previamente a cualquier tipo de procedimiento invasivo en el paciente, especialmente cuando concurre en su diagnóstico un proceso infeccioso. Presentamos un caso de gran bucle de arteria carótida interna que condicionó una importante tumoración parafaríngea en un paciente con infección faríngea asociada para llamar la atención sobre esta rara entidad como causa de tumoración parafaríngea y su potencial peligro
The parapharyngeal space-occupying lessions may develop from any of the structures located inside it. Uncommon although possible ethiologies for them are anatomic variations of the course of the internal carotid artery. These anatomic variants mainly arise from degenerative, mostly age-amplified, causes and lead to elongations, angulations, kinking and tortuosities in the course of the vessel that appear as impressions or protusions of the pharyngeal wall. Utherly unfrecuently lead to loops that may show as large parapharyngeal tumors. This fact should be noticed previously to any invasive procedure to be performed on the patient specially when evidenced in concommitance with an infectious process at the moment of diagnosis .A case of an impressive internal carotid artery loop that conditioned an important parapharyngeal mass in a patient with an associated pharyngeal infection is reported to warn about this rare parapharyngeal space-occupying condition and its potential danger
Subject(s)
Female , Aged , Humans , Carotid Artery, Internal/abnormalities , Pharyngeal Neoplasms/etiology , Carotid Stenosis/complications , Pharyngeal Diseases/complications , Respiratory Tract Infections/complicationsABSTRACT
Chondrosarcomas of the larynx are rare neoplasms that account only for a very small percentage of all the primary laryngeal neoplasms that has been estimated in less than 2%. They often show initially with indolent symptoms despite their progressive growth until great compromise of the laryngeal function is produced. This neoplasm is characterised by a variable local aggressive behaviour and frequent of distant metastasis, that are commonly located in lungs and bone, deppending on the hystologic grade. A case of an aggressive laryngeal chondrosarcoma with exclusive brain metastasic disease as cause of death, a very uncommon event in these neoplasms, is reported. We also provide a review of the literature focusing on the controversies about the management of this condition.
Subject(s)
Chondrosarcoma/surgery , Laryngeal Neoplasms/surgery , Chondrosarcoma/diagnostic imaging , Chondrosarcoma/pathology , Humans , Laryngeal Neoplasms/diagnostic imaging , Laryngeal Neoplasms/pathology , Male , Middle Aged , Tomography, X-Ray ComputedABSTRACT
Angiomyolipoma is an uncommon hamartomatous renal tumor very prone to spontaneous bleeding. However it is possible to find it in extrarenal sites, being the liver the most frequent one. The rest of sites recorded in literature are exceptional. In fact, to our knowledge, this is the first case reported of cervicothoracic settlement of an angiomyolipoma. This rare location, together with its marked tendency to bleed bleeding determinate a complicated diagnoses a difficult surgical approach.
Subject(s)
Angiomyolipoma/surgery , Head and Neck Neoplasms/surgery , Adult , Humans , Male , Surgical Procedures, Operative/methodsABSTRACT
El angiomiolipoma es un raro tumor hamartomatoso con asiento predominantemente en el riñón, con marcada tendencia al sangrado espontáneo. Sin embargo, es posible encontrarlo con localización extrarrenal, siendo en este caso el hígado su principal lugar de asiento. El resto de localizaciones descritas son excepcionales. De hecho, hasta dónde conocemos, éste es el primer caso descrito en la literatura médica de angiomiolipoma cervicotorácico. Esta peculiar localización, unida a su marcada tendencia al sangrado, determinan un complicado diagnóstico y un difícil abordaje quirúrgico (AU)
Angiomyolipoma is an uncommon hamartomatous renal tumor very prone to spontaneous bleeding. However it is possible to find it in extrarenal sites, being the liver the most frequent one. The rest of sites recorded in literature are exceptional. In fact, to our knowledge, this is the first case reported of cervicothoracic settlement of an angiomyolipoma. This rare location, together with its marked tendency to bleed bleeding determinate a complicated diagnoses a difficult surgical approach (AU)
Subject(s)
Male , Humans , Adult , Angiomyolipoma/surgery , Head and Neck Neoplasms/surgery , Surgical Procedures, Operative/methodsABSTRACT
Los plasmocitomas son neoplasias que derivan de las células plasmáticas, que incluyen el mieloma múltiple (MM), el plasmocitoma solitario de hueso y el plasmocitoma extramedular (PEM). Los PEM son tumores solitarios que consisten en una proliferación de células plasmáticas localizadas en lugares distintos del hueso. Un porcentaje variable puede desarrollar con posterioridad un MM. Representan más de un 4 por ciento de los tumores no epiteliales del tracto respiratorio superior. Generalmente se desarrollan en el tejido submucoso de vías aéreas superiores (80 por ciento de los casos), con predilección por la nasofaringe, las fosas nasales, los senos paranasales y las amígdalas. Presentamos los casos clínicos de tres pacientes diagnosticados de PEM localizados en vías aerodigestivas superiores en nuestro Servicio. En los tres casos la resección o la biopsia de la tumoración permitió su estudio histológico en el que se observó una proliferación de células plasmáticas. Los estudios séricos y de médula ósea permitieron concluir el diagnóstico. El tratamiento se realizó con cirugía y radioterapia en 2 casos, y exclusivamente con radioterapia en el tercero de ellos. Los pacientes son sometidos a revisiones periódicas, no habiéndose observado recurrencia de la enfermedad. Llevamos a cabo una revisión de los aspectos clínicos, diagnósticos terapéuticos y pronósticos de este tipo de tumores (AU)
No disponible
Subject(s)
Middle Aged , Aged , Male , Humans , Plasmacytoma , Nose Neoplasms , Oropharyngeal Neoplasms , Ear NeoplasmsABSTRACT
No disponible
No disponible
Subject(s)
Middle Aged , Aged , Male , Humans , Plasmacytoma , Nose Neoplasms , Oropharyngeal Neoplasms , Ear NeoplasmsABSTRACT
Tuberculous otitis media (TOM) is a rare cause of chronic suppurative infection of the middle ear. Due to that the symptoms and signs are often indistinguishable from those of nontuberculosis chronic otitis media and the fact that the index of suspicion is low, there is frequently a considerable delay prior to diagnosis. This can lead to irreversible complications such as facial nerve paralysis and labyrinthitis. Medical therapy with antituberculous drugs is usually effective. We report three cases with TOM diagnosticated and followed up in our Service from january 1993 to july 2001. Their charts were retrospectively reviewed for relevant historical data, physical findings, complementary studies, treatment and clinical response. We performed a review of the literature, emphasizing that TOM should be considered in the differential diagnosis of chronic otitis media.
Subject(s)
Otitis Media/cerebrospinal fluid , Tuberculosis/cerebrospinal fluid , Adult , Aged , Amoxicillin/therapeutic use , Antitubercular Agents/therapeutic use , Cerebrospinal Fluid Otorrhea/cerebrospinal fluid , Cerebrospinal Fluid Otorrhea/microbiology , Drug Combinations , Ear Diseases/cerebrospinal fluid , Ear Diseases/drug therapy , Ear Diseases/microbiology , Female , Humans , Isoniazid/therapeutic use , Male , Mycobacterium Infections/drug therapy , Mycobacterium Infections/microbiology , Mycobacterium tuberculosis/isolation & purification , Otitis Media/drug therapy , Otitis Media/microbiology , Penicillins/therapeutic use , Proteus Infections/drug therapy , Proteus Infections/microbiology , Proteus mirabilis/isolation & purification , Pyrazinamide/therapeutic use , Rifampin/therapeutic use , Staphylococcal Infections/drug therapy , Staphylococcal Infections/microbiology , Staphylococcus aureus/isolation & purification , Tuberculosis/drug therapy , Tuberculosis/microbiologyABSTRACT
La otitis media tuberculosa (OMT) es una causa rara de otorrea crónica. La escasa especificidad de su sintomatología, a menudo indistinguible de la de otras causas de otitis media crónica, y el bajo índice de sospecha, hacen que en muchas ocasiones su diagnóstico se retrase e incluso se realice tras la aparición de complicaciones, como la parálisis facial o la laberintitis. Habitualmente responde bien al tratamiento con fármacos antituberculosos. Presentamos 3 casos de otitis media tuberculosa diagnosticado y seguidos en nuestro Servicio, desde enero de 1993 a julio de 2001. Se revisan retrospectivamente las 3 historias clínicas haciendo hincapié en los datos clínicos, la exploración, los exámenes complementarios, el tratamiento y la respuesta clínica. Realizamos una revisión bibliográfica del tema recalcando la necesidad de incluir la OMT en el diagnóstico diferencial de la otitis media crónica (AU)
No disponible
Subject(s)
Adult , Aged , Male , Female , Humans , Staphylococcus aureus , Staphylococcal Infections , Rifampin , Tuberculosis , Mycobacterium tuberculosis , Penicillins , Otitis Media , Proteus Infections , Proteus mirabilis , Pyrazinamide , Antitubercular Agents , Ear Diseases , Drug Combinations , Amoxicillin , Isoniazid , Mycobacterium Infections , Cerebrospinal Fluid OtorrheaABSTRACT
Down beat nystagmus is a central nystagmus. In the literature reviewed the two most common causes that can produce it are cerebellar degenerations and Chiari malformation. The site of lesion causing this nystagmus appears to be the brainstem or the cerebellum, although the definitive etiopathogeny remains controversial. We report the case of a 73-year-old woman with sudden unsteadiness of gait and down beating nystagmus, without any other vestibular or neurologic signs. Simple radiology and MRI evidenciated a Chiari malformation type 1 associated to plastybasia.
