Síndrome de Morning Glory / Morning Glory Syndrome

Introducción: la anomalía congénita del disco óptico conocida como "morning glory" (MG) es un desarrollo anómalo del nervio óptico, caracterizado por una cavidad amplia en forma de embudo del disco en el punto donde salen las fibras del nervio de la retina. Su presentación más frecuente es unilateral. La mayoría de los casos son aislados y no se asocian con anomalías sistémicas. Objetivo: se presenta el caso de un niño de 1 año con MG y los hallazgos imagenológicos. Caso clínico: presentamos el caso de un paciente masculino de 1 año con sospecha de patología visual; en el estudio se realizó resonancia magnética cerebral (RM) en la que se identificó excavación en forma de embudo de la papila del globo ocular izquierdo, alteración en la morfología del quiasma óptico con desplazamiento inferior asimétrico y engrosamiento del infundíbulo y del tallo hipofisiario. Discusión: el diagnóstico de MG es clínico, las imágenes aportan información adicional sobre anomalías oculares, craneofaciales y vasculares asociadas. En este caso además de la lesión ocular había anomalías de estructuras de línea media como el infundíbulo y el tallo hipofisiario, además de engrosamiento quiasmático. Conclusión: la valoración por imágenes en fundamental, no solo para describir la alteración del nervio óptico, si no para identificar y describir anomalías asociadas para realizar manejo integral del paciente.

Introduction: the congenital optic disc anomaly known as morning glory disc anomaly (MGDA) is an alteration of normal optic nerve development, characterized by an enlarged funnel-shaped cavity of the optic disc, the point in the eye where the optic nerve fibers leave the retina. The anomaly is typically unilateral. Most are isolated cases with no associated systemic anomalies. Objective: a case is presented in a one-year-old boy with MGDA and the corresponding imaging findings. Case reports: we present the case of a one-year-old male patient with suspected visual impairment. Cerebral magnetic resonance imaging (MRI) revealed a papillary funnel-shaped excavation in the left eye, alteration in the morphology and inferior asymmetric displacement of the optic chiasm and thickening of the pituitary infundibular stalk. Discussion: MGDA is diagnosed clinically, however, imaging provides additional information regarding the associated ocular, craniofacial and vascular abnormalities. In this case, in addition to the ocular lesion there were abnormalities of midline structures, such as, the pituitary infundibular stalk, as well as, optic chiasm thickening. Conclusion: imaging is fundamental, not only to describe the anatomic alteration of the optic nerve, but also to identify related abnormalities in order to provide patients a comprehensive management

Características visuales en el albinismo: Revisión sistemática

El albinismo es una condición compleja, en muchos casos incapacitante, afectando a muchas personas alrededor del mundo, incluso puede conducir a la muerte. Los problemas visuales más comunes que pueden existir en el albinismo con fotofobia son defectos refractivos con medidas considerables, problemas a nivel de fondo de ojo, fotofobia y translucencia de iris. La realidad actual estima que 1 de cada 10.000 personas tienen albinismo y pueden presentar estos problemas visuales. Objetivo. Identificar las características visuales en cada tipo de albinismo presentados en los estudios de Latinoamérica durante el periodo 2014-2022. Metodología. Se realizó una revisión sistemática. Como criterios de inclusión se tomó en cuenta el año de publicación de las fuentes. Se utilizaron los siguientes descriptores de búsqueda en español y en inglés: "albinismo", "tipos de albinismo" y "complicaciones albinismo", "albinism", "albinism types", "albinism issues". Resultados. Fueron seleccionados 22 artículos obtenidos de Google Académico, revistas como pubmed, scielo, Elsevier, tesis, de los cuales y basado en controles de calidad se analizaron 12 fuentes. Conclusión. La mayoría de estudios describen tratamientos posibles para el albinismo. Se resaltan hallazgos clínicos que destacan las características en los tipos de albinismo como lo son problemas a nivel del nervio óptico como la hipoplasia, y disminución de la agudeza visual, nistagmus y en ocasiones el problema de posición compensatoria de cabeza.

Albinism is a complex condition, in many cases disabling, affecting many people around the world, and can even lead to death. The most common visual problems that may exist in albinism with photophobia are refractive defects with considerable measurements, problems at the fundus level, photophobia and iris translucency. Current reality estimates that 1 in 10,000 people have albinism and may present with these visual problems. Objective. To identify the visual characteristics in each type of albinism presented in studies in Latin America during the period 2014-2022. Methodology. A systematic review was performed. The year of publication of the sources was taken into account as inclusion criteria. The following search descriptors were used in Spanish and English: "albinism", "types of albinism" and "albinism complications", "albinism", "albinism types", "albinism issues". Results. Twenty-two articles obtained from Google Scholar, journals such as pubmed, scielo, Elsevier, theses were selected, from which and based on quality controls 12 sources were analyzed. Conclusion. Most studies describe possible treatments for albinism. Clinical findings that highlight the characteristics in the types of albinism such as problems at the level of the optic nerve as hypoplasia, and decreased visual acuity, nystagmus and sometimes the problem of compensatory head position are highlighted.

O albinismo é uma condição complexa e, em muitos casos, incapacitante, que afeta muitas pessoas em todo o mundo e pode até levar à morte. Os problemas visuais mais comuns que podem existir no albinismo com fotofobia são defeitos de refração de tamanho considerável, problemas no nível do fundo do olho, fotofobia e translucidez da íris. A realidade atual estima que 1 em cada 10.000 pessoas tem albinismo e pode apresentar esses problemas visuais. Objetivo. Identificar as características visuais em cada tipo de albinismo apresentado em estudos na América Latina durante o período de 2014 a 2022. Metodologia. Foi realizada uma revisão sistemática. O ano de publicação das fontes foi levado em consideração como critério de inclusão. Foram usados os seguintes descritores de busca em espanhol e inglês: "albinismo", "tipos de albinismo" e "complicações do albinismo", "albinismo", "tipos de albinismo", "questões de albinismo". Resultados. Foram selecionados 22 artigos do Google Scholar, revistas como pubmed, scielo, Elsevier, teses, dos quais 12 fontes foram analisadas com base em controles de qualidade. Conclusão. A maioria dos estudos descreve possíveis tratamentos para o albinismo. Os achados clínicos destacam as características dos diferentes tipos de albinismo, como problemas no nervo óptico, como hipoplasia, diminuição da acuidade visual, nistagmo e, às vezes, problemas compensatórios na posição da cabeça.

Ecografía de nervio óptico para estimar hipertensión intracraneal en pacientes adultos con neurotrauma

Introducción: El neurotrauma es una condición que puede dar paso a una hipertensión intracraneana, situación que es muy grave. Los métodos diagnósticos de elección son los invasivos, aun así, los no invasivos y entre ellos la ecografía del nervio óptico, ofrecen muchísimas ventajas. Objetivo: Describir elementos esenciales de la ecografía de nervio óptico como método para diagnosticar hipertensión intracraneal en pacientes adultos con neurotrauma. Métodos: Se hizo una revisión de la literatura más reciente sin restricción lingüística o geográfica en las bases de datos PubMed y SciELO, se usaron términos afines al tema del artículo y se realizó una valoración crítica sobre la bibliografía consultada. Resultados: La literatura disponible sobre la ecografía del nervio óptico en la determinación de la hipertensión intracraneal es abundante y la mayoría apunta a sus beneficios como método no invasivo. La principal debilidad del mismo es que no es capaz de dar un valor exacto y esto se debe a que el valor normal del diámetro de la vaina del nervio óptico por cada persona puede variar significativamente. La proporción directa entre el diámetro de la vaina del nervio óptico y la presión intracraneal es un hecho que ningún autor intenta invalidar. Conclusiones: La ecografía del nervio óptico es un método seguro, accesible económicamente, no invasivo, fácil de usar y con un valor predictivo confiable para determinar la hipertensión intracraneal.

Introduction: Neurotrauma is a condition that can lead to intracranial hypertension, which is a very serious situation. The diagnostic methods of choice are the invasive ones, even so, the non-invasive ones offer many advantages, the ultrasound of the optic nerve is among them. Objective: To describe essential elements of optic nerve ultrasound as a method to diagnose intracranial hypertension in adult patients with neurotrauma. Methods: A review of the most recent literature was made without linguistic or geographical restrictions in databases such as PubMed and SciELO, terms related to the theme of the manuscript were used. A critical assessment of the consulted bibliography was made. Results: The available literature on optic nerve ultrasound in the determination of intracranial hypertension is abundant and most points to the benefits as a non-invasive method. However, its main weakness lies in the fact that it is not capable of giving an exact value, due to the fact that the normal value of the diameter of the optic nerve sheath for each person can vary significantly. The direct relationship between optic nerve sheath diameter and intracranial pressure is a fact that no author attempts to invalidate. Conclusions: Optic nerve ultrasound is a safe, affordable, non-invasive, easy-to-use method with a reliable predictive value to determine intracranial hypertension.

Infiltración del nervio óptico como forma inicial de recaída de la leucemia linfoblástica aguda / Optic nerve infiltration as an initial form of relapse of acute lymphoblastic leukemia

Introducción: La infiltración del nervio óptico como forma inicial de recaída de la leucemia linfoblástica aguda es rara, aunque altamente indicativa de que el sistema nervioso central está involucrado. Objetivo: Actualizar el conocimiento sobre infiltración del nervio óptico como forma inicial de recaída de la leucemia linfoblástica aguda. Métodos: Se realizó una revisión de las publicaciones más relevantes relacionadas con el tema durante los últimos años. La búsqueda y la localización de la información se apoyaron en la elección de palabras clave/descriptores que configuraron el perfil de búsqueda. Se utilizó el MeSH Database de PubMed. Se realizó una extensa revisión en Google Académico y otros megabuscadores de revisión sistemática mediante TripDatabase y Cochrane. Conclusiones: La infiltración directa de células tumorales y las alteraciones hematológicas propias de la enfermedad constituyen los mecanismos fundamentales de la fisiopatogenia. El edema del disco óptico es su signo oftalmoscópico más distintivo. La imagen por resonancia magnética de cráneo, el análisis citológico del fluido cerebroespinal y la biopsia de médula ósea negativas no deben excluir el diagnóstico. La terapia combinada que incluye la radiación localizada constituye una buena opción de tratamiento. Un número considerable de ojos recuperan su agudeza visual y muestran resolución del cuadro infiltrativo(AU)

Introduction: Optic nerve infiltration as an initial form of relapse of acute lymphoblastic leukemia is rare, although it is highly indicative of central nervous system involvement. Objective: To update the knowledge about optic nerve infiltration as an initial form of relapse of acute lymphoblastic leukemia. Methods: A review of the most relevant publications related to the subject during the last years was carried out. The search and localization of information was supported by the choice of keywords/descriptors that configured the search profile. The MeSH Database of PubMed was used. An extensive review was performed in Google Scholar and other systematic review mega search engines using TripDatabase and Cochrane. Conclusions: Direct tumor cell infiltration and disease-specific hematologic alterations are the fundamental mechanisms of pathophysiology. Optic disc edema is the most distinctive ophthalmoscopic sign. Negative cranial magnetic resonance imaging, cytologic analysis of cerebrospinal fluid and bone marrow biopsy should not exclude the diagnosis. Combination therapy including localized radiation is a good treatment option. A considerable number of eyes recover visual acuity and show resolution of the infiltrative picture(AU)

Comparative morphological and computed tomographical measurements of the orbit in cattle and pig eyes / Mediciones morfológicas y tomográficas computarizadas comparativas de la órbita en ojos de bovinos y porcinos

SUMMARY: Understanding species intraocular parameters using computed tomography (CT) scanning is initial and crucial step in ophthalmology. There is a lack of studies that have specialized in estimating the cattle and pig eyes using CT scans. Therefore, this study aimed to describe the anatomical and computed tomography features of cattle and pig eyes and its internal structures. Animal heads that not suffer from any diseases related to the eye were disarticulated. CT scan was performed. Moreover, 10 % fixed buffered formalin Specimens were used for the anatomical description of the eye and the optic nerve in particular. The values of length, width of the lens and globe, radio density of the optic nerve, lens, the anterior chamber, the vitreous chamber, and orbital dimensions were measured. Statistically, all parameters of cattle optic nerve increased significantly than that of the pig except for radiodensity, and angle of the optic nerve. Furthermore, all intraocular parameters of cattle increased significantly than that of the pig except the radiodensity of aqueous and vitreous humor. There was no significant difference between species in the opening angle of the orbit. This study's findings represent a first step toward developing CT reference values for cattle and pigs intraocular structural assessments.

Comprender los parámetros intraoculares de especies mediante la tomografía computarizada es un paso inicial y crítico en oftalmología. Faltan estudios enfocados en la estimación de ojos de bovinos y porcinos mediante tomografías computarizadas. Por tanto, este estudio tuvo como objetivo describir las características anatómicas y de tomografía computarizada de los bulbos oculares de bovinos y porcinos y sus estructuras internas. Se desarticularon cabezas de animales que no padecieran alguna enfermedad relacionada con el ojo. Se realizó una tomografía computarizada. Se fijaron las muestras en formalina tamponada al 10 % para la descripción anatómica del bulbo ocular y del nervio óptico en particular. Se midieron los valores de longitud del bulbo ocular, ancho del lente y la radio densidad del nervio óptico, lente, cámara anterior, cámara vítrea y dimensiones orbitarias. Estadísticamente, todos los parámetros del nervio óptico del ganado aumentaron significativamente con respecto al del cerdo, excepto la radio densidad y el ángulo del nervio óptico. Además, todos los parámetros intraoculares del ganado bovino aumentaron significativamente respecto a los del cerdo, excepto la radio densidad del humor acuoso y cuerpo vítreo. No hubo diferencia significativa entre especies en el ángulo de apertura de la órbita. Los hallazgos de este estudio representan un primer paso hacia el desarrollo de valores de referencia de tomografía computarizada para evaluaciones estructurales intraoculares de ganado vacuno y porcino.

Contribución de los hallazgos tomográficos al diagnóstico de la hipertensión intracraneal en el traumatismo craneoencefálico grave

Introducción: El traumatismo craneoencefálico es responsable de más de 500 000 visitas a departamentos de urgencias, 95 000 hospitalizaciones y 7 000 muertes en la población infantil. La monitorización clínica se apoya en técnicas imagenológicas, entre otras. Objetivo: Relacionar los hallazgos tomográficos encontrados, a través de la clasificación de Marshall al ingreso y diámetro de la vaina del nervio óptico y su asociación con la presión intracraneal y con los resultados en el paciente pediátrico con traumatismo craneoencefálico grave. Métodos: Estudio descriptivo prospectivo con todos los pacientes pediátricos aquejados de traumatismo craneoencefálico grave entre enero de 2003 y diciembre de 2017. Resultados: De los 41 casos, se encontró relación entre los niveles de presión intracraneal y el grado de luxación de la línea media >10 mm en 10 enfermos (62,5 %) y con el diámetro de la vaina del nervio óptico (>5,5 mm) por tomografía de cráneo en 12 (75,0 %), igualmente existió relación entre esas dos variables con los resultados a los 6 meses de evolución. Conclusiones: La utilización de la tomografía de cráneo simple como herramienta para la determinación de variables utilizadas en el monitoreo no invasivo de la presión intracraneal como son la clasificación tomográfica de Marshall, la luxación de la línea media y la medición del diámetro de la vaina del nervio óptico, permite identificar incrementos de la presión intracraneal. Esto pudiera utilizarse para el tratamiento intensivo de los pacientes pediátricos con traumatismo cráneo encefálico grave, sin necesidad de la utilización de monitoreo invasivo en ellos.

Introduction: Traumatic brain injury is responsible for more than 500,000 visits to the emergency services, 95,000 hospitalizations and 7,000 deaths in children. Clinical monitoring is based on imaging techniques, among others. Objective: To relate the tomographic findings found, through Marshall's classification at the entrance and diameter of the optic nerve sheath and its association with intracranial pressure and with the results in the pediatric patient with severe head trauma. Methods: Prospective descriptive study with all pediatric patients suffering from severe head trauma between January 2003 and December 2017. Results: Of the 41 cases, a relationship was found between the levels of intracranial pressure and the degree of dislocation of the midline >10 mm in 10 patients (62.5%) and with the diameter of the optic nerve sheath (>5.5 mm) by skull tomography in 12 (75.0%); there was also a relationship between these two variables with the results at 6 months of evolution. Conclusions: The use of simple tomography of the skull as a tool for the determination of variables used in the non-invasive monitoring of intracranial pressure such as Marshall tomographic classification, midline dislocation and measurement of the diameter of the optic nerve sheath, allows to identify increases in intracranial pressure. This could be used for the intensive treatment of pediatric patients with severe head trauma, without the need for invasive monitoring in them.

Presión de perfusión ocular en sujetos sin enfermedad ocular / Ocular Perfusion Pressure in Subjects Without Ocular Disease

Objetivo: Describir el comportamiento de la presión de perfusión ocular en sujetos sin enfermedad ocular. Métodos: Se realizó un estudio descriptivo transversal con sujetos sin enfermedad ocular de la consulta externa de Oftalmología del Hospital Universitario "General Calixto García", entre enero y diciembre de 2019. Resultados: La mediana de presión intraocular del ojo derecho osciló entre 13,0-16,0 mmHg, y del ojo izquierdo entre 13,0-15,5 mmHg, durante todo el estudio. El valor máximo para ambos ojos se obtuvo a las 6:00 a. m. (madrugada). La mediana de presión arterial sistólica osciló entre 129,0-138,5 mmHg, y de diastólica entre 79,5-81,5 mmHg. El valor mínimo de presión arterial diastólica fue 53 mmHg a las 6:00 a. m. La mediana de presión de perfusión ocular del ojo derecho osciló entre 46,4 mmHg (12:00 a. m.) y 50,8 mmHg; y del ojo izquierdo entre 47,3 mmHg (6:00 a. m.) y 51,9 mmHg. El valor mínimo específico de presión de perfusión ocular fue 35 mmHg para ambos ojos, a las 6:00 a. m. Conclusiones: La hipertensión arterial es el antecedente patológico personal más frecuente en la población estudiada y en ocasiones se producen cifras fuera de la normalidad, tanto elevadas como disminuidas, capaces de afectar la presión de perfusión ocular, sobre todo la diastólica baja. Sin embargo, las presiones de perfusión ocular se mantuvieron normales, probablemente, por mecanismos de autorregulación individuales(AU)

Objective: To describe the behavior of ocular perfusion pressure in subjects without ocular disease. Methods: A cross-sectional descriptive study was performed with subjects without ocular disease from the Ophthalmology outpatient clinic of the University Hospital "General Calixto García", between January and December 2019. Results: The median intraocular pressure of the right eye ranged between 13.0-16.0 mmHg, and of the left eye between 13.0-15.5 mmHg, throughout the study. The maximum value for both eyes was obtained at 6:00 a. m. (early morning). The median systolic blood pressure ranged from 129.0-138.5 mmHg, and diastolic from 79.5-81.5 mmHg. The minimum diastolic blood pressure value was 53 mmHg at 6:00 a.m. The median ocular perfusion pressure of the right eye ranged from 46.4 mmHg (12:00 a.m.) to 50.8 mmHg; and of the left eye from 47.3 mmHg (6:00 a.m.) to 51.9 mmHg. The minimum specific ocular perfusion pressure value was 35 mmHg for both eyes, at 6:00 a.m. Conclusions: Arterial hypertension is the most frequent personal pathologic antecedent in the population studied, and both elevated and decreased out-of-normal figures capable of affecting ocular perfusion pressure, especially low diastolic, occasionally occur. However, ocular perfusion pressures remained normal, probably due to individual autoregulatory mechanisms(AU)

Ultrasonografía del diámetro de la vaina del nervio óptico en paciente con meningoencefalitis complicada / Ultrasonography of the optic nerve sheath diameter in patient with complicated meningoencephalitis

RESUMEN La ultrasonografía del diámetro de la vaina del nervio óptico es un método no invasivo para monitorizar la presión intracraneal. Se ha utilizado en múltiples patologías neurocríticas, incluyendo la infección complicada del sistema nervioso central. Se presenta el caso de una paciente femenina de 47 años, quien ingresó en la Unidad de Cuidados Intensivos luego de presentar progresión al estado comatoso secundario a cuadro de meningoencefalitis bacteriana. Al ingreso se constata midriasis bilateral arreactiva, ausencia parcial de reflejos del tallo encefálico y bradicardia. Ante la sospecha clínica de hipertensión intracraneal, se indica ultrasonografía del diámetro de la vaina del nervio óptico en plano axial. Se realizaron tres mediciones para cada ojo, mostrando un valor de 6,3, 6,6 y 6,00 mm en ojo derecho, y 6,8, 6,6 y 6,8 mm en el izquierdo (promedio biocular: 6,5 mm). Con esta medición se complementó el diagnóstico sospechado. Esta técnica representa un proceder seguro y no invasivo. Su uso completa los datos recogidos en el examen clínico. El punto de corte universalmente aceptado para el diagnóstico es de 5,0 mm o más para un valor de presión intracraneal > 20 mmHg.

ABSTRACT The optic nerve sheath ultrasonography is a non-invasive method for monitoring intracranial pressure. It has been used in multiple neurocritical pathologies, including the complicated infection of the central nervous system. The case of a 47-year-old female patient is presented; she was admitted to the Intensive Care Unit after presenting progression to comatose state secondary to bacterial meningoencephalitis. On admission, bilateral arreactive mydriasis, partial absence of brainstem reflexes and bradycardia are confirmed. Given the clinical suspicion of intracranial hypertension, ultrasonography of the optic nerve sheath diameter in the axial plane is indicated. Three measurements were made for each eye, showing a value of 6.3, 6.6 and 6.00 in the right eye, and 6.8, 6.6 and 6.8 in the left one (biocular average: 6.5 mm). With this measurement the suspected diagnosis was completed. This technique represents a secure and non-invasive procedure. Its use completes the data collected in the clinical examination. The universally accepted cut-off point for diagnosis is 5.0 mm or more for an intracranial pressure value of > 20 mmHg.

Evaluation of third ventriculostomy outcome by measuring optic nerve sheath diameter in adult hdyrocephalus.

OBJECTIVE: It is difficult to demonstrate the success of the procedure in patients with third ventriculostomy. We evaluated that optic nerve sheath diameter (ONSD) measurement, which can reflect intracranial pressure, may be a criterion for decision of endoscopic third ventriculostomy (ETV) success. METHODS: 28 adult patients suffering long overt standing ventriculomegaly (LOVA) who performed ETV were included in this retrospective study. The patients were divided into two groups as successful (group A) and failed ETV group (group B) according to their postoperative evaluation. ONSD was measured on pre- and post-operative computed tomography (CT) and Evan's index (EI), diameter of third ventricule (V3), the patency of ETV stoma and periventricular edema were evaluated by magnetic resonance imaging (MRI). RESULTS: The mean ONSD was measured as 6.39±0.92mm for the right eye, 6.50±0.91mm for the left eye on preoperative CT. The mean ONSD by CT (after surgery) was 4.89±0.87mm for the right eye, 5.02±0.1mm for the left eye (p<0.05). Postoperative group A and group B were compared according to ONSD measurement; mean ONSD in group A was 4.52±0.69mm for the right and 4.59±0.9mm for the left, mean ONSD in group B was 5.82±0.51mm for the right and 6.1±0.32mm for the left (p<0.05). The best ONSD value for detecting failed ETV was 5.40mm (sensitivity 90%, specifity 75%, AUROC 0.938) for right and 5.91mm (sensitivity 90%, specifity 75%, AUROC 0.950) for left. EE was measured as 0.39±0.12mm on preoperative MRI and 0.39±0.12mm on postoperative MRI (p=0.3). V3 was measured as 14.7±2.47mm on preoperative MRI and 10.47±1.99mm on postoperative MRI (p<0.05). CONCLUSION: The statistical values obtained from study show that the ONSD measurement can help in the postoperative evaluation of patients, who had a ETV surgery.

Neurological manifestations of neurofibromatosis type 1: our experience.

INTRODUCTION: Neurofibromatosis type 1 (NF1) is a progressive multisystem disorder following an autosomal dominant inheritance pattern that presents with multiple neurological manifestations. METHODS: We reviewed medical histories of patients with NF1 followed up at our hospital's paediatric neurology department from May 1990 to 31 December 2018. We collected data on neurological symptoms. RESULTS: A total of 128 patients with NF1 were identified. Mean age (SD) at NF1 diagnosis was 4.43 (3.38) years (range, 0.5-14.5 years). There was a slight female predominance (53.1%). Macrocephaly (head circumference over 2 SDs above average for age) was present in 37.5% of cases. Attention-deficit/hyperactivity disorder was recorded in 28.9% of patients (37): combined type in 20 patients, predominantly inattentive in 15, and predominantly impulsive/hyperactive in 2. Other manifestations included headache (18.6%), cognitive impairment (7.8%), motor deficit (6.2%), and epilepsy (4.68%). Brain MRI was performed in 85 patients, revealing T2-weighted hyperintensities in the basal ganglia and/or cerebellum in 60 patients (70.5%), Chiari malformation type 1 in 4 cases, and arachnoid cysts in 3. Optic nerve gliomas were identified by MRI in 22 patients (25.8%). Other MRI findings included plexiform neurofibromas (9.3%) and central nervous system gliomas (3.1%). CONCLUSIONS: The neurological manifestations identified in our sample are consistent with those reported in the literature. Effective transfer strategies from paediatric neurology departments and subsequent clinical follow-up by adult neurology departments are needed to prevent loss to follow-up in adulthood.

Mensuración ecográfica del complejo nervio óptico-vaina en la hipertensión intracraneal / Ultrasonographic imaging of the optic nerve-sheath complex in intracranial hypertension

Objetivo: Determinar la relación entre el grosor del complejo nervio óptico-vaina, mensurado por ecografía y la hipertensión intracraneal. Métodos: Se realizó un estudio observacional descriptivo y longitudinal en 144 órbitas de 72 pacientes con diagnóstico clínico de hipertensión intracraneal. Una vez alcanzada la mejoría clínica, se les practicó ultrasonido orbitario al inicio del diagnóstico, el cual permitió mensurar la vaina meníngea, el nervio óptico, el complejo nervio óptico-vaina y la altura de la papila. Resultados: En la totalidad de los casos el grosor inicial de la vaina fue ≥ 3 mm, el del complejo nervio óptico-vaina > 5 mm, y la altura de la papila > 0,8 mm, mientras que el del nervio óptico no superó los 3 mm. Tras alcanzar la mejoría clínica se demostró disminución de todas estas variables, con excepción del nervio óptico, cuyo diámetro casi no se modificó. Algunas diferencias evidenciadas entre la primera y la segunda medición ecográfica fueron estadísticamente significativas. Conclusión: Por tanto, este proceder se ratifica como parte del monitoreo neurológico integral en pacientes con hipertensión intracraneal sospechada o confirmada(AU)

Objective: To determine the relationship between the thickness of the optic nerve- sheath complex as measured by ultrasound and intracranial hypertension. Methods: A descriptive and longitudinal observational study was performed in 144 orbits of 72 patients with a clinical diagnosis of intracranial hypertension. Once clinical improvement was achieved, orbital ultrasound was performed at the beginning of the diagnosis, which allowed measuring the meningeal sheath, the optic nerve, the optic nerve-sheath complex and the height of the papilla. Results: In all cases the initial thickness of the sheath was ≥ 3 mm, that of the optic nerve-sheath complex > 5 mm, and the height of the papilla > 0.8 mm, while that of the optic nerve did not exceed 3 mm. After reaching clinical improvement, a decrease in all these variables was demonstrated, with the exception of the optic nerve, whose diameter was almost unchanged. Some differences between the first and second ultrasound measurements were statistically significant. Conclusion: Therefore, this procedure is ratified as part of the full neurological monitoring in patients with suspected or confirmed intracranial hypertension(AU)

Relación entre la medición por tomografía y ecografía del diámetro de la vaina del nervio óptico como estimador no invasivo de la presión intracraneal.

OBJECTIVE: To compare the ONSD measured by ultrasound and tomography in patients with a diagnosis of intracranial hypertension. METHOD: Prospective, transversal, observational, analytical study. 105 patients were included, divided into two groups: healthy (control group) and patients presenting clinical data of intracranial hypertension (study group). ONSD was measured by ultrasound and tomography. The Kruskal-Wallis test was used to compare the ONSD between the patients, and the Spearman test was used to assess the correlation between USG and CT. A value of p <0.05 was considered statistically significant. RESULTS: Of the 105 patients, 58.1% were men and 41.9% women. The study group included 14 patients with TBI, CVD, intracranial neoplasia, or neuroinfection. The highest median of ONSD by Ultrasound was in the CVD group, followed by TBI, neoplasia and neuroinfection and the lowest was in the control group (7.5, 7.0, 6.8, 6.8 and 5.2 mm respectively); these differences being statistically significant (p < 0.001). In the correlation analysis between Ultrasound and CT, a good statistically significant positive correlation was found (rho = 0.893, p < 0.001). CONCLUSIONS: The ultrasound evaluation of ONSD has proven to be a reliable test for the diagnosis and non-invasive monitoring of intracranial hypertension.

OBJETIVO: Comparar el diámetro de la vaina del nervio óptico (DVNO) medido por ultrasonografía (USG) y tomografía computarizada (TC) en pacientes con diagnóstico de hipertensión intracraneal. MÉTODO: Estudio prospectivo, transversal, observacional y analítico. Se incluyeron 105 pacientes divididos en dos grupos: sanos (grupo control) y pacientes que presentaran datos clínicos de hipertensión intracraneal (grupo de estudio). Se midió el DVNO por USG y TC. Para comparar el DVNO entre los pacientes se utilizó la prueba de Kruskal-Wallis, y para evaluar la correlación entre USG y TC se utilizó la prueba de Spearman. Un valor de p < 0.05 fue considerado estadísticamente significativo. RESULTADOS: De los 105 pacientes, el 58.1% eran hombres y el 41.9% mujeres. El grupo de estudio incluyó 14 pacientes con traumatismo craneoencefálico (TCE), evento vascular cerebral (EVC), neoplasia intracraneal o neuroinfección. La mayor mediana de DVNO por USG la tuvo el grupo de EVC, seguido de los pacientes con TCE, neoplasia y neuroinfección, y la menor la tuvo el grupo control (7.5, 7.0, 6.8, 6.8 y 5.2 mm, respectivamente), siendo estas diferencias estadísticamente significativas (p < 0.001). En el análisis de correlación entre USG y TC se encontró una buena correlación positiva estadísticamente significativa (rho = 0.893, p < 0.001). CONCLUSIONES: La evaluación por USG del DVNO ha demostrado ser una prueba confiable para el diagnóstico y el monitoreo no invasivo de la hipertensión intracraneal.

Correlation between optic nerve sheath diameter and extracorporeal life support time.

INTRODUCTION: The objective of the study was to analyse the correlation between extracorporeal life support (ECLS) and aortic cross-clamp times and optic nerve sheath diameter (ONSD). PATIENTS AND METHODS: Study in a cohort of patients aged 0-15 years that underwent ECLS for cardiac surgery after obtention of signed informed consent. We calculated a sample size of 23 participants. First, we obtained 3 vertical and 3 horizontal measurements of the ONSD for each eye and calculated the mean of both eyes for each measurement to be used in the analysis. The measurements were made at admission and at 6 and 24 h post surgery. We retrieved the ECLS time and the aortic cross-clamp time were from the operative report. RESULTS: We analysed data for 23 participants, 52.2% female, with a median age of 14 months. The median ECLS time was 60 min; the median aortic cross-clamp time was 32 min. The median baseline ONSD was 3.1 mm. ONSD values had increased a median of 0.015 mm at 6 h post surgery (P = .03). We found a positive correlation between ECLS time and ONSD values (r = 0.476, P < 0,05). The ONSD values returned to baseline by 24 h post surgery. None of the patients developed signs or symptoms of increased intracranial pressure. CONCLUSION: Our study found a correlation between ECLS time and ONSD at 24 h post surgery. We found variations in the ONSD even in patients without signs or symptoms of increased ICP. Further research is required to identify the factors related to these variations.

Septo-optic dysplasia: Ophthalmological abnormalities in a series of 5 cases.

Septo-optic dysplasia (SOD) is a rare congenital condition of unknown cause, with a characteristic triad that includes optic nerve hypoplasia, pituitary function abnormalities and midline brain defects, in addition to a broad spectrum of symptoms and associations. A total of five clinical cases are presented, four of which met the complete classic triad. All of them showed a wide variety of ophthalmological, endocrinological and neurological alterations. Within the ophthalmological spectrum of SOD, papillary hypoplasia and ocular motility alterations (nystagmus, strabismus) stand out. Other less frequent ones may also appear, such as pupillary alterations, microphthalmia and coloboma. Given the suspicion of SOD, brain MRI scan should be performed, as well as consultation with the paediatric department in order to complete the study and indicate, if necessary, systemic treatment.

Optic nerve sheath diameter with intracranial pressure monitoring: a non-invasive method to follow children with craniosynostosis.

Craniosynostosis (CS) is associated with increased intracranial pressure (ICP) and this elevation is of vital importance in children. Bedside optic nerve sheath diameter (ONSD) on ocular ultrasonography has begun to be increasingly used in recently. A patient who was diagnosed with CS, standard anesthesia monitoring and anesthesia were performed. Before and after the surgery, ONSD measurement was performed to follow the changes in ICP. ONSD measurement can be used as an effective, non-invasive, repeated, and easy-to-apply method to monitor the changes in the ICP in pediatric patients with CS who are planned to undergo craniectomy.

La craneosinostosis (SC) se asocia con un aumento de la presión intracraneal (PIC) y esta elevación es de vital importancia en los niños. El diámetro de la vaina del nervio óptico al lado de la cama (ONSD) en la ecografía ocular ha comenzado a usarse cada vez más recientemente. A un paciente al que se le diagnosticó SC, se le realizó seguimiento anestésico estándar y anestesia. Antes y después de la cirugía, se realizó la medición ONSD para seguir los cambios en la PIC. La medición ONSD se puede utilizar como un método eficaz, no invasivo, repetido y fácil de aplicar para monitorear los cambios en la PIC en pacientes pediátricos con SC que se planea someter a craniectomía.

Ultrasonografía del diámetro de la vaina del nervio óptico en el monitoreo de la presión intracraneal / Ultrasound of optic nerve sheath diameter for monitoring intracranial pressure

Introducción: El neuromonitoreo no invasivo en pacientes críticos representa una opción de primera línea para el manejo de complicaciones fatales derivadas del aumento de la presión intracraneal. En esta modalidad se incluye la ultrasonografía del diámetro de la vaina del nervio óptico, la cual representa una técnica rápida, fácil de realizar y disponible a la cabecera del paciente. Objetivos: Describir aspectos fundamentales y actualizados sobre el uso de la ultrasonografía en el monitoreo de la presión intracraneal a través de la medición del diámetro de la vaina del nervio óptico en los diferentes escenarios neuroclínicos. Métodos: Se realizó una revisión de la literatura publicada en bases de datos como: PubMed/Medline, SciELO y Google académico entre los meses mayo y julio de 2020. Se revisaron publicaciones en inglés y español. Se seleccionaron 46 bibliografías que cumplieron con los criterios de inclusión. Se describen aspectos fundamentales como la anatomía ecográfica del nervio óptico, descripción de la técnica y su uso en entidades neurocríticas como el traumatismo craneoencefálico, ictus, muerte encefálica, entre otros. Conclusiones: La ecografía de la vaina del nervio óptico representa una alternativa no invasiva ampliamente aceptada para la medición del incremento de la presión intracranial. Con un diámetro de 5,0 hasta 5,9 mm o más se puede asumir el diagnóstico de hipertensión intracraneal con alta sensibilidad y especificidad, aunque debe individualizarse su uso en cada patología neurocrítica. La curva de aprendizaje para la realización del proceder es de breve tiempo y satisface las habilidades necesarias(AU)

Introduction: Noninvasive neuromonitoring in critically ill patients is a first-line option for the management of fatal complications derived from increased intracranial pressure. This modality includes ultrasound of optic nerve sheath diameter, which is a quick technique, easy to perform and available at the bedside. Objectives: To describe significant and state-of-the-art aspects regarding the use of ultrasound for monitoring intracranial pressure through measurement of the optic nerve sheath diameter in different neuroclinical settings. Methods: A review was carried out, between May and July 2020, of the literature published in databases such as PubMed/Medline, SciELO and Google Scholar. Publications in English and Spanish were reviewed. Forty-six bibliographic sources were chosen, as long as they met the inclusion criteria. Fundamental aspects are described, such as the ultrasound anatomy of the optic nerve, the technique procedures and its use in neurocritical entities such as head trauma, stroke and brain death, among others. Conclusions: Ultrasound of the optic nerve sheath is a widely accepted noninvasive choice for measurement of increased intracranial pressure. With a diameter of 5.0 to 5.9 mm or more, the diagnosis of intracranial hypertension can be assumed with high sensitivity and specificity, although its use should be individualized in each neurocritical pathology. The learning curve for carrying out the procedure is short and satisfies the necessary skills(AU)

Medición de la vaina del nervio óptico mediante tomografía computarizada como predictor de mortalidad en pacientes con traumatismo craneoencefálico grave al ingreso a la Unidad de Cuidados Intensivos / Optic nerve sheath measurement through computerized scan as death predictor in patients with serious cranioencephalic trauma on entry to the Intensive Care Unit / Medição da bainha do nervo óptico por tomografia computadorizada como preditor de mortalidade em pacientes com trauma cranioencefálico grave internados na Unidade de Terapia Intensiva

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Abstract: Introduction: Traumatic brain injury (TBI) is a medical-surgical condition characterized by brain involvement secondary to a traumatic lesion. Patients with severe TBI are at high risk of mortality and this will depend on different factors such as the presence of intracranial hypertension, age, origin of the injury and score on the Glasgow coma scale. Measurement of the optic nerve sheath diameter (ONSD) appears to be a good indirect indicator of intercranial hypertension and therefore, a good predictor of mortality. Objective: To determine the most appropriate cut-off point, as well as the measurement of the ONSD usefulness as a prognostic indicator of mortality in patients with severe TBI in the Intensive Care Unit (ICU). Material and methods: This is an analytical, descriptive, and retrospective study. The universe of study consists of all the case/files with TBI. For the sample selection, all available records of patients with severe TBI sent to the ICU during the period from March 1 to August 31, 2021, will be included. Within the inclusion criteria patients with a Glasgow scale score of < 8 points on entry and with a computerized scan done. The dependent variables to considerer are the outcome understood as death or survival of the patient, the days hospitalized in the ICU, the presence of complications; among the dependent variables is the diameter of the optic nerve sheath measured by computerized tomography. Intervening variables were also considered such as the presence of comorbidities and overweight/obesity, the age and sex of the patient. The project consisted of four phases: 1) request for authorization and access to files, 2) application of selection criteria, 3) performance of ONSD measurements and 4) creation of the database. Finally, once the database is formed, the statistical analysis will proceed; for the descriptive part, prevalence's, means (standard deviation) and medians (percentiles) will be calculated for the variables by sex and by outcome, subsequently the diagnostic capacity of the ONSD will be analyzed through the area under the ROC curve (receiving operating characteristics) for the outcome. Afterwards the performance of this and other cut-off points are compared using the Youden index. Results: Sixty records of TBI patients admitted to the ICU were studied, 51 were men (85%), 45 patients survived (75%) and 15 patients died (25%). The average age was of 50.5 ± 10.6 years, the average Glasgow score on admission was 6.6 ± 1.6 points, the average BMI was 26.42 ± 4.10 kg/m2, and the average number of days spent in the ICU was 9.03 ± 6.4. The diameter of the optic nerve was not a predictor of mortality, but if the Glasgow coma scale was, with an AUC of 0.775 (95% CI: 0.648-0.901, p = 0.002), the best cut-off point was 7 with a sensitivity of 93% and specificity of 54%. The bivariate linear regression model points to low Glasgow coma score and long hospital stay as predictors of mortality. Conclusions: The results of this study infer that, consistent with current scientific evidence, the sociodemographic characteristics of our population are similar to those reported by other authors, with men over 50 years of age being the most affected by this entity. On the other hand, the measurement of the diameter of the optic nerve sheath has been considered a good prognostic indicator of intracranial hypertension, which in turn is associated with increased mortality. However, in the present study there is no association between the diameter of the optic nerve sheath and the prognosis of mortality.

Resumo: Introdução: O traumatismo cranioencefálico (TCE) é uma condição médico-cirúrgica caracterizada por lesão cerebral secundária a uma lesão traumática. Pacientes com TCE grave apresentam alto risco de mortalidade e isso dependerá de diversos fatores, como presença de hipertensão intracraniana, idade, origem da lesão e pontuação na Escala de Coma de Glasgow. A medida do diâmetro da bainha do nervo óptico (DBNO) parece ser um bom indicador indireto de hipertensão intracraniana e, portanto, um bom preditor de mortalidade. Objetivo: Determinar o ponto de corte mais adequado, bem como a utilidade da medida do DBNO como indicador prognóstico de mortalidade em pacientes com TCE grave na Unidade de Terapia Intensiva. Material e métodos: Trata-se de um estudo analítico, descritivo e retrospectivo. O universo de estudo é composto por todos os prontuários de casos/pacientes com TCE grave. Para a seleção da amostra foram incluídos todos os prontuários disponíveis de pacientes com TCE grave encaminhados à Unidade de Terapia Intensiva no período de 1o de março a 31 de agosto de 2021, dentro dos critérios de inclusão foram considerados pacientes com escala de Glasgow < 8 pontos na admissão e com uma tomografia computadorizada realizada. As variáveis ​​dependentes consideradas são o desfecho entendido como óbito ou sobrevida do paciente, os dias de internação na UTI, a presença de complicações; dentro das variáveis ​​independentes está o diâmetro da bainha do nervo óptico medido por tomografia computadorizada. Também foram consideradas variáveis ​​intervenientes, como presença de comorbidades e sobrepeso/obesidade, idade e sexo do paciente. O projeto consistiu em três fases: a) Pedido de autorização e acesso aos prontuários, b) Aplicação dos critérios de seleção, c) Desenvolvimento da base de dados. Por fim, uma vez formada a base de dados, procedeu-se à análise estatística. Para a parte descritiva, foram calculadas as prevalências, médias (desvio padrão) e medianas (percentis) das variáveis ​​por sexo e por desfecho. Posteriormente, a capacidade diagnóstica do DBNO foi analisada pela área sob a curva ROC (Receiving Operating Characteristics) para o resultado. Posteriormente, o desempenho deste e de outros pontos de corte foi comparado pelo índice de Youden. Resultados: Foram estudados 60 prontuários de pacientes com TCE que deram entrada na UTI, 51 eram homens (85%), 45 pacientes sobreviveram (75%) e 15 pacientes morreram (25%). A média de idade foi de 50.5 ± 10.6 anos, a média de Glasgow na admissão foi de 6.6 ± 1.6 pontos, a média de IMC foi de 26.42 ± 4.10 kg/m2 e a média de dias de internação na UTI foi de 9.03 ± 6.4. O diâmetro do nervo óptico não foi preditor de mortalidade, mas a Escala de Coma de Glasgow sim, com AUC de 0.775 (IC 95%: 0.648-0.901, p = 0.002), o melhor ponto de corte foi 7 com sensibilidade de 93% e especificidade de 54%. O modelo de regressão linear bivariada aponta para baixo escore de coma de Glasgow e longa permanência hospitalar como preditores de mortalidade. Conclusões: Os resultados deste estudo inferem que, de acordo com as evidências científicas atuais, as características sociodemográficas de nossa população são semelhantes às relatadas por outros autores, sendo os homens com aproximadamente 50 anos de idade os mais acometidos por essa entidade. Por outro lado, a medida do diâmetro da bainha do nervo óptico tem sido considerada um bom indicador prognóstico de hipertensão intracraniana, que por sua vez está associada ao aumento da mortalidade. No entanto, no presente estudo não há associação entre o diâmetro da bainha do nervo óptico e o prognóstico de mortalidade.

Metastatic involvement of optic nerve in patient with breast and lung neoplasia.

Metastases are the most common adult intraocular tumors. However, those located in the optic nerve are very uncommon and are usually associated with spread to other locations such as the central nervous system, which darkens the prognosis. There is a case of a 67-year-old woman who reports progressive vision loss in the right eye of 15 days of evolution. The ophthalmological examination shows a relative afferent pupil defect in this eye and a pseudoedema of the papilla with retinal hemorrhages in the fundus. Personal history and characteristics of the optic nerve suggest the diagnosis of metastatic infiltration.

Evaluation of third ventriculostomy outcome by measuring optic nerve sheath diameter in adult hdyrocephalus.

OBJECTIVE: It is difficult to demonstrate the success of the procedure in patients with third ventriculostomy. We evaluated that optic nerve sheath diameter (ONSD) measurement, which can reflect intracranial pressure, may be a criterion for decision of endoscopic third ventriculostomy (ETV) success. METHODS: 28 adult patients suffering long overt standing ventriculomegaly (LOVA) who performed ETV were included in this retrospective study. The patients were divided into two groups as successful (group A) and failed ETV group (group B) according to their postoperative evaluation. ONSD was measured on pre- and post-operative computed tomography (CT) and Evan's index (EI), diameter of third ventricule (V3), the patency of ETV stoma and periventricular edema were evaluated by magnetic resonance imaging (MRI). RESULTS: The mean ONSD was measured as 6.39±0.92mm for the right eye, 6.50±0.91mm for the left eye on preoperative CT. The mean ONSD by CT (after surgery) was 4.89±0.87mm for the right eye, 5.02±0.1mm for the left eye (p<0.05). Postoperative group A and group B were compared according to ONSD measurement; mean ONSD in group A was 4.52±0.69mm for the right and 4.59±0.9mm for the left, mean ONSD in group B was 5.82±0.51mm for the right and 6.1±0.32mm for the left (p<0.05). The best ONSD value for detecting failed ETV was 5.40mm (sensitivity 90%, specifity 75%, AUROC 0.938) for right and 5.91mm (sensitivity 90%, specifity 75%, AUROC 0.950) for left. EE was measured as 0.39±0.12mm on preoperative MRI and 0.39±0.12mm on postoperative MRI (p=0.3). V3 was measured as 14.7±2.47mm on preoperative MRI and 10.47±1.99mm on postoperative MRI (p<0.05). CONCLUSION: The statistical values obtained from study show that the ONSD measurement can help in the postoperative evaluation of patients, who had a ETV surgery.

Coexistence of idiopathic intracranial hypertension and optic disc drusen in a latin american patient: case report.

Optic nerve drusen are acellular concretions of calcium located in the parenchyma of the optical nerve head described as the most common cause of pseudo-papilloedema, which makes it difficult to differentiate from a true optical disc oedema. Despite it being rare, the drusen of the optic nerve and the papilloedema secondary to idiopathic intracranial hypertension can coexist in the same patient. The case is presented of a 34 year-old woman referred to the Ophthalmology Department with visual discomfort, headaches, and pulsatile tinnitus of two months onset. In the physical examination there was bilateral blurring of the optic disc margin with absence of spontaneous venous pulsation. The diagnosis of idiopathic intracranial hypertension was made by lumbar puncture with the measurement of the opening pressure, and due to the findings of the magnetic resonance scan of the brain and the eye sockets. The optic nerve drusen was an incidental finding in an optical coherence tomography angiography, which would later be confirmed with an eye ultrasound. To our knowledge, this the first case reported in Latin America of the coexistence of optic nerve drusen and papilloedema secondary to idiopathic intracranial hypertension.