Citomegalia congénita y malformaciones corticales y subcorticales / Congenital cytomegalovirus infection and cortical/subcortical malformations

Introducción: La infección intrauterina por citomegalovirus es la más frecuente de las viriasis/parasitosis intrauterinas que afectan al sistema nervioso central y causan lesiones permanentes tanto en el córtex como en la sustancia blanca subcortical. Son escasos los estudios de resonancia magnética (RM) cerebral.Material y métodos: Seis pacientes (4M y 2V) fueron estudiados desde los primeros meses de vida para hacer el diagnóstico de citomegalia congénita e identificar la presencia de lesiones corticales y subcorticales, utilizando las necesarias secuencias de RM. Resultados: Los 6 pacientes mostraban malformaciones del desarrollo cortical (MDC) (esquisencefalia, polimicrogiria o lisencefalia-paquigiria) desde la época neonatal y alteraciones difusas de la sustancia blanca, que se mantuvieron con pocos cambios durante los dos primeros años y después se iban reduciendo de tamaño en forma de zonas de hiperseñal en T2, circunscritas a determinadas áreas y permanecían con pocos cambios durante algunos años más. Conclusión:La infección intrauterina por citomegalovirus causa lesiones en sustancia gris cortical, que consisten en MDC y en sustancia blanca subcortical. Estas últimas muestran aspecto cambiante, ya que aparecen como áreas difusas y amplias de hiperseñal, que se suelen interpretar como retraso en la mielinización, pero que también pueden ser causadas directamente por el virus de la citomegalia. Estas alteraciones de la sustancia blanca sufren cambios morfológicos a lo largo de los primeros años de vida, dejando atrofia cerebral. Las secuelas neurológicas que dejan estas alteraciones son severas y crónicas (AU)

Introduction: Intrauterine infection due to cytomegalovirus is the most common of the intrauterine viral/parasitic infections that affect the central nervous system and cause permanent lesions in the cortex as well as the subcortical white matter. Studies using brain magnetic resonance imaging (MRI) are limited. Material and methods: Six patients (4 females and 2 males) were studied in the first months of life in order to make a diagnosis of congenital cytomegalovirus, and identify the cortical and subcortical lesions using the necessary MRI sequences. Results: The six patients showed malformations of cortical development (MDC) (schizencephaly, polymicrogyria or lissencephaly-pachygyria) from the neonatal period, and diffuse changes of the white matter, which remained with few changes during the first two years. They then began reducing in size in the form of high signal areas in T2, restricted to certain areas, and were evident for a few years more with little change. Conclusion: Intrauterine infection due to cytomegalovirus causes changes in the cortical grey matter, which consists of MDC, and in the subcortical white matter. The latter show a changing aspect as they appear as diffuse and wide areas of high signal intensity, which is usually due to delay in myelinisation, but could also be caused directly by the cytomegalovirus. These changes in the white matter are subjected to morphological changes throughout the first years of life, leading to brain atrophy. The neurological sequelae of these lesions left by these alterations are severe and chronic (AU)

[Congenital cytomegalovirus infection and cortical/subcortical malformations]. / Citomegalia congénita y malformaciones corticales y subcorticales.

INTRODUCTION: Intrauterine infection due to cytomegalovirus is the most common of the intrauterine viral/parasitic infections that affect the central nervous system and cause permanent lesions in the cortex as well as the subcortical white matter. Studies using brain magnetic resonance imaging (MRI) are limited. MATERIAL AND METHODS: Six patients (4 females and 2 males) were studied in the first months of life in order to make a diagnosis of congenital cytomegalovirus, and identify the cortical and subcortical lesions using the necessary MRI sequences. RESULTS: The six patients showed malformations of cortical development (MDC) (schizencephaly, polymicrogyria or lissencephaly-pachygyria) from the neonatal period, and diffuse changes of the white matter, which remained with few changes during the first two years. They then began reducing in size in the form of high signal areas in T2, restricted to certain areas, and were evident for a few years more with little change. CONCLUSION: Intrauterine infection due to cytomegalovirus causes changes in the cortical grey matter, which consists of MDC, and in the subcortical white matter. The latter show a changing aspect as they appear as diffuse and wide areas of high signal intensity, which is usually due to delay in myelinisation, but could also be caused directly by the cytomegalovirus. These changes in the white matter are subjected to morphological changes throughout the first years of life, leading to brain atrophy. The neurological sequelae of these lesions left by these alterations are severe and chronic.

[Cutaneous hemangiomas and vascular malformations and associated pathology (Pascual-Castroviejo type II syndrome). Study of 41 patients]. / Hemangiomas y malformaciones vasculares cutáneas y patología asociada (síndrome de Pascual-Castroviejo tipo II). Presentación de 41 pacientes.

AIM: To describe the clinical, diagnostic and therapeutic features of this angiomatous neurocutaneous syndrome, which is the most frequent one, and to report a personal series of 41 patients. PATIENTS AND METHODS: Forty one patients--31 females and 10 males--were studied during childhood and then, several patients were followed during many years, which allowed us to learn about the evolution of the abnormalities. The cutaneous lesions were classified as hemangiomas in 30 patients (73%) and as vascular malformations in 11 patients (27%). RESULTS: A cerebellar anomaly (unilateral hemispheric hypoplasia and Dandy-Walker malformation) was seen in 13 patients (31.5%) cerebral cortical dysplasia in 4 patients (10%), aortic arch coarctation in 6 patients (15%), and congenital cardiopathy in 5 patients (12%). The most frequent abnormalities were intracranial and/or extracranial vascular malformations. Persistence of the trigeminal artery was observed in 7 patients (17%), absence or severe hypoplasia of an internal carotid artery in 13 patients (32%), absence of a vertebral artery in 7 patients (17%), hypoplasia of intracranial arteries in 6 patients (15%) and aneurysmal enlargement of carotid or vertebral arteries in 5 patients (12%). Also were observed 4 patients (10%) with intracranial hemangioma, 2 (5%) with hemangioma in mediastinum, and 3 (7.5%) with intestinal hemangioma, all of which disappeared during the first years of life. Aneurysmal enlargement of the carotid and vertebral arteries and intracranial branches also disappeared after a process of progressive narrowing of the arterial lumen that caused complete obstruction of these arteries. At the same time the cutaneous hemangioma regressed. During this process, collateral vascularization through branches of the external carotid artery and of the non-affected branches of the contralateral intracranial arteries developed. CONCLUSION: This neurocutaneous syndrome is the most frequent one and it is associated with several types of vascular and non-vascular abnormalities which can involve any organ of the body. Internal and external hemangiomas and hemangiomatous lesions progress and tend to regress concomitantly.

Hemangiomas y malformaciones vasculares cutáneas y patología asociada (síndrome de Pascual-Castroviejo tipo II). Presentación de 41 pacientes / Cutaneous hemangiomas and vascular malformations and associated pathology (Pascual-Castroviejo type II syndrome). Study of 41 patients

Objetivo. Describir la historia, los hechos diagnósticos y terapéuticos de este síndrome neurocutáneo de naturaleza angiomatosa, que es el más frecuente de los trastornos neurocutáneos. Al mismo tiempo, presentamos una serie personal de 41 pacientes. Pacientes y métodos. Se estudiaron 41 pacientes –31 mujeres y 10 varones– cuando eran niños y se han seguido a varios de ellos durante muchos años, por lo que ha podido conocerse la evolución de sus anomalías. Las lesiones cutáneas correspondían a hemangiomas en 30 pacientes (73%) y a malformaciones vasculares en 11 (27%). Resultados. Las malformaciones cerebelosas (hipoplasia de un hemisferio y malformación de Dandy-Walker) aparecían en 13 pacientes (31,5%). Cuatro pacientes (10%) presentaban displasias corticales. Seis pacientes (15%) presentaban coartación aórtica y cinco (12%) cardiopatía congénita de diverso tipo. Las malformaciones vasculares intracraneales y/o extracraneales eran las anomalías asociadas más frecuentes, y se ha encontrado: persistencia de la arteria trigeminal en siete pacientes (17%), ausencia o hipoplasia grave de la arteria carótida interna en 13 (32%), ausencia de la arteria vertebral en siete (17%), hipoplasia de arterias intracraneales en seis (15%), dilatación aneurismática de carótidas o vertebrales en cinco (12%). También se encontraron cuatro hemangiomas intracraneales, dos en mediastino y tres intestinales, que desaparecieron durante los primeros años de vida. La dilatación aneurismática de las arterias carótidas y vertebrales y de sus ramas intracraneales desaparecieron y, tras algunos años de regresión coetánea con la de los hemangiomas faciales, se estrecharon gravemente hasta obstruirse totalmente, al mismo tiempo que se formaba una circulación colateral transdural a través de las ramas de la arteria carótida externa. Conclusión. Este síndrome neurocutáneo, el más frecuente de todos, se asociaba a varios tipos de patología vascular y de otros órganos. Las lesiones hemangiomatosas internas y externas progresaban y regresaban casi simultáneament (AU)

Aim. To describe the clinical, diagnostic and therapeutic features of this angiomatous neurocutaneous syndrome, which is the most frequent one, and to report a personal series of 41 patients. Patients and methods. Forty one patients –31 females and 10 males– were studied during childhood and then, several patients were followed during many years, which allowed us to learn about the evolution of the abnormalities. The cutaneous lesions were classified as hemangiomas in 30 patients (73%) and as vascular malformations in 11 patients (27%). Results. A cerebellar anomaly (unilateral hemispheric hypoplasia and Dandy-Walker malformation) was seen in 13 patients (31.5%) cerebral cortical dysplasia in 4 patients (10%), aortic arch coarctation in 6 patients (15%), and congenital cardiopathy in 5 patients (12%). The most frequent abnormalities were intracranial and/or extracranial vascular malformations. Persistence of the trigeminal artery was observed in 7 patients (17%), absence or severe hypoplasia of an internal carotid artery in 13 patients (32%), absence of a vertebral artery in 7 patients (17%), hypoplasia of intracranial arteries in 6 patients (15%) and aneurysmal enlargement of carotid or vertebral arteries in 5 patients (12%). Also were observed 4 patients (10%) with intracranial hemangioma, 2 (5%) with hemangioma in mediastinum, and 3 (7.5%) with intestinal hemangioma, all of which disappeared during the first years of life. Aneurysmal enlargement of the carotid and vertebral arteries and intracranial branches also disappeared after a process of progressive narrowing of the arterial lumen that caused complete obstruction of these arteries. At the same time the cutaneous hemangioma regressed. During this process, collateral vascularization through branches of the external carotid artery and of the non-affected branches of the contralateral intracranial arteries developed. Conclusion. This neurocutaneous syndrome is the most frequent one and it is associated with several types of vascular and non-vascular abnormalities which can involve any organ of the body. Internal and external hemangiomas and hemangiomatous lesions progress and tend to regress concomitantly (AU)

[Orbital vasculonervous network and orbital surgical compartments by high field magnetic resonance]. / Dotación vasculonerviosa y espacios anatomoquirúrgicos de la órbita por resonancia magnética de alto campo.

PURPOSE: To elucidate the possibilities and indications of high-resolution magnetic resonance imaging (MRI) in the study of the orbit and its contents. METHODS: Orbital anatomy was studied in sliced specimens of fifteen fresh frozen cadavers and the results were compared with those obtained in thirty asymptomatic subjects who underwent a magnetic resonance with 1.5 Tesla equipment. The information obtained was used to interpret the findings in twenty-two patients with various orbital diseases. RESULTS: High-resolution MRI allows visualization of structures difficult to assess previously, like the cerebrospinal fluid (CSF) surrounding the optic nerve, the complete intraorbital route and the exit of the third cranial nerve, the ophthalmic artery and the intraorbital relationships of the sixth cranial nerve, which can be clearly differentiated from the lateral rectus muscle. CONCLUSIONS: High-resolution MRI is a very useful tool for the study of the orbit and its content. It provides accurate diagnoses through non-invasive procedures and facilitates the planning of the surgical approaches by improving the visualization of pathologic orbital structures. lcarlos@correo.uniovi.es

Dotación vasculonerviosa y espacios anatomoquirúrgicos de la órbita por resonancia magnética de alto campo / Orbital vasculonervous network and orbital surgical compartments by high field magnetic resonance

Objetivo: Conocer las posibilidades de la Resonancia Magnética de alto campo en el estudio de la órbita y su contenido. Métodos: Se han realizado cortes anatómicos en 15 cadáveres a fin de analizar las estructuras orbitarias y vecinas, comparando éstas con los hallazgos obtenidos en 30 sujetos sanos a los que fue realizada una Resonancia Magnética (RM) con equipo dotado de 1.5 TESLA. Asimismo se incorporaron al estudio comparativo un grupo de 22 pacientes portadores de patología orbitaria diversa. Resultados: Se pueden identificar con este tipo de sistema de exploración estructuras hasta ahora difícilmente asequibles, tales como el L.C.R. que acompaña al fascículo óptico, la salida y recorrido completos del III par craneal, la arteria oftálmica y las relaciones del VI par craneal en el interior de la órbita, que con este método puede ser bien diferenciable de la estructura del músculo recto externo. Conclusiones: La Resonancia Magnética de alto campo o de alta resolución es un procedimiento de exploración muy útil para el estudio de la órbita y su contenido, lo cual permite avanzar en el diagnóstico por métodos escasamente invasivos y facilita la preparación de los abordajes quirúrgicos, al mostrar con buena nitidez el estado de las diferentes estructuras orbitarias afectadas por patología (AU)

Purpose: To elucidate the possibilities and indications of high-resolution magnetic resonance imaging (MRI) in the study of the orbit and its contents. Methods: Orbital anatomy was studied in sliced specimens of fifteen fresh frozen cadavers and the results were compared with those obtained in thirty asymptomatic subjects who underwent a magnetic resonance with 1.5 Tesla equipment. The information obtained was used to interpret the findings in twenty-two patients with various orbital diseases. Results: High-resolution MRI allows visualization of structures difficult to assess previously, like the cerebrospinal fluid (CSF) surrounding the optic nerve, the complete intraorbital route and the exit of the third cranial nerve, the ophthalmic artery and the intraorbital relationships of the sixth cranial nerve, which can be clearly differentiated from the lateral rectus muscle. Conclusions: High-resolution MRI is a very useful tool for the study of the orbit and its content. It provides accurate diagnoses through non-invasive procedures and facilitates the planning of the surgical approaches by improving the visualization of pathologic orbital structures (AU)