[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.

[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