Síndrome de uno y medio / One and a half syndrome

Se realizó una revisión bibliográfica con el objetivo de obtener información actualizada acerca de las características, diagnóstico y tratamiento del síndrome del uno y medio. Se emplearon principalmente las bases de datos disponibles en Infomed, Google Scholar y Pubmed. El síndrome del uno y medio es una entidad infrecuente, que se caracteriza por parálisis de la mirada conjugada horizontal y alteración del fascículo longitudinal medial ipsilateral secundario a diversas etiologías, entre las que se incluyen la enfermedad cerebrovascular y la esclerosis múltiple. Clínicamente se presenta con exotropía y nistagmo a la abducción. Su diagnóstico puede ser establecido en la exploración por las alteraciones típicas de los movimientos oculares, mientras que la imagen de resonancia magnética cerebral resulta indispensable para el diagnóstico diferencial y etiológico(AU)

One and a half syndrome is an infrequent condition characterized by conjugate horizontal gaze palsy and ipsilateral medial longitudinal fasciculus alteration secondary to various etiologies, including cerebrovascular disease and multiple sclerosis. Clinically, it presents with exotropia and abduction nystagmus. Its diagnosis may be established during exploration, due to the typical eye movement alterations, whereas brain magnetic resonance imaging is indispensable for differential and etiological diagnosis. A bibliographic review was conducted to obtain updated information about the characteristics, diagnosis and treatment of one and a half syndrome. Use was made of the databases available in Infomed, Google Scholar and Pubmed(AU)

Abordaje pterional transilviano con apoyo neuroendoscópico en colesteatoma cerebral, a propósito de un caso interesante / Transsilvian pterional approach with neuroendoscopic support in cholesteatoma of the brain. A propos of an interesting case

RESUMEN Los quistes epidermoides constituyen el 1 % de los tumores intracraneales y el 7 % de los del ángulo pontocerebeloso. Los colesteatomas son lesiones benignas que se originan de restos de tejidos epitelial ectodérmicos que quedan en el sistema nervioso central, al cerrarse el tubo neural entre la tercera y quinta semana de gestación. Se trata de un paciente remitido de la Consulta de Neurooftalmología con crisis de cefalea y toma de los pares craneales III, IV, V, rama oftálmica desde hace 2 semanas. Se le realizaron estudios imagenológicos donde se constató un tumor hipodenso en región silviana frontotemporal izquierdo. Se interpretó como un quiste arcnoideo. Se le aplicó un bordaje pterional transilviano con apoyo neuroendoscópico y para sorpresa del equipo quirúrgico se abordó un tumor perlado solido identificado macroscópicamente como un colesteatoma silviano. Se resecó la totalidad del tumor cerebral.

ABSTRACT Epidermal inclusion cysts constitute 1% of the intracranial tumors and 7% of the cerebellopontine angle ones.Cholesteatoma are benign lesions originated from the remains of ectodermic epithelial tissues remaining in the central nervous system when the neural tube closes between the third and fifth week of pregnancy. The case deals with a patient remitted from the Neurophthalmologic Consultation with migraine crisis and lesion on the III, IV, V cranial nerves, ophthalmologic branch, for two weeks. Image studies were carried out, showing a hypo dense tumor in the left silvian frontotemporal region. It was taken as an arachnoid cyst. A pterional trans-silvian approach with neuroendoscopic support was applied, and the surgical team was surprised when they found a solid pearly tumor that was macroscopically identified as a silvian cholesteatoma. The cerebral tumor was totally resected.

Comparison of Astigmatism Induced by Combined Inferior Oblique Anterior Transposition Procedure and Lateral Rectus Recession Alone

PURPOSE: The purpose of this study is to compare the magnitude and axis of astigmatism induced by a combined inferior oblique (IO) anterior transposition procedure with lateral rectus (LR) recession versus LR recession alone. METHODS: Forty-six patients were retrospectively analyzed. The subjects were divided into two groups: those having concurrent inferior oblique muscle overaction (IOOA) and intermittent exotropia (group 1, 20 patients) and those having only intermittent exotropia as a control (group 2, 26 patients). Group 1 underwent combined anterior transposition of IO with LR recession and group 2 underwent LR recession alone. Induced astigmatism was defined as the difference between preoperative and postoperative astigmatism using double-angle vector analysis. Cylinder power, axis of induced astigmatism, and spherical equivalent were analyzed at 1 week, 1 month, and 3 months after surgery. RESULTS: Larger changes in the axis of induced astigmatism were observed in group 1, with 4.5° incyclotorsion, than in group 2 at 1 week after surgery (axis, 84.5° vs. 91°; p < 0.001). However, there was no statistically significant inter-group difference thereafter. Relaxation and rapid regression in the incyclotorsion of induced astigmatism were observed over-time. Spherical equivalent significantly decreased postoperatively at 1 month in both groups, indicating a myopic shift (p = 0.011 for group 1 and p = 0.019 for group 2) but did not show significant differences at 3 months after surgery (p = 0.107 for group 1 and p = 0.760 for group 2). CONCLUSIONS: Combined IO anterior transposition procedures caused an increased change in the axis of induced astigmatism, including temporary incyclotorsion, during the first week after surgery. However, this significant difference was not maintained thereafter. Thus, combined IO surgery with LR recession does not seem to produce a sustained astigmatic change, which can be a potential risk factor of postoperative amblyopia or diplopia compared with LR recession alone.

Apresentação atípica da oftalmopatia de Graves / Atypical presentation of Graves’ ophthalmopathy

A oftalmopatia de Graves é a doença orbitária mais comum e acomete 25 a 50% dos pacientes portadores da Doença de Graves e é mais frequente no sexo feminino, entre a segunda e quinta décadas de vida. A doença apresenta uma fase aguda e uma crônica, evoluindo lenta e progressivamente até estabilizar, sendo raros os casos de resolução espontânea. O tratamento dependerá da fase em que a doença se encontra e está baseado principalmente na corticoterapia via oral e endovenosa e/ou radioterapia, sendo a colchicina empregada em casos isolados. No seguinte relato de caso, abordaremos uma forma atípica de manifestação clínica da Oftalmopatia de Graves em paciente eutireoideia com anticorpos negativos na sua apresentação inicial.

Graves ‘ophthalmopathyis themost commonorbital diseaseand affects25-50% of the patients withGraves’ disease. It`s morecommon in females, between the second andfifth life`s decade. The disease hasanacute andachronic stage, slowly progressing until it stabilizes, with rarecasesof spontaneous resolution.The treatment depends on thestage andthe disease ismainly treated withoralor intravenous corticosteroids with or without radiotherapy;colchicine is usedin individual cases. In the followingcase report, we discuss an atypicalmanifestation ofGraves’ ophthalmopathyin an euthyroid patientwith negative antibodiesin the inicial presentation.

Consideraciones actuales en la insuficiencia de convergencia / Current considerations about convergence insufficiency

La insuficiencia de convergencia es un problema común de coordinación muscular en el cual los ojos tienden a desviarse hacia afuera exoforia con la lectura o cuando se realizan actividades de cerca. Los síntomas más característicos son los astenópicos. Es una de las causas más frecuentes de disconfort ocular. Se diagnostica cuando la exoforia es mayor de cerca y el paciente tiene uno o ambos de los siguientes signos: un punto próximo de convergencia remoto y/o reserva fusional positiva reducida. No existe un consenso para su diagnóstico y tratamiento, aunque muchos coinciden en que la terapia visual y la aplicación de los adelantos técnicos donde los programas de computación juegan un papel importante logran una pronta mejoría en estos pacientes

Convergence insufficiency is a common eye muscle coordination problem in which the eyes tend to drift outward (exophoria) when reading or doing some work at near distance. The most frequent symptoms are the asthenopics. Convergence insufficiency is one of the most common causes of ocular discomfort. The diagnosis is based on exophoria greater at near distance, a remote near point of convergence and decreased convergence amplitudes. There is not any consensus whatsoever about diagnosis and treatment, although many agree on the fact that the visual therapy and the application of technical advances - computer software packages play an important role - manage to attain rapid visual improvement in these patients

Measurement of Strabismic Angle Using the Distance Krimsky Test

PURPOSE: To evaluate the correlation of the distance Krimsky test and the alternate prism cover test (APCT) for the distance deviation in patients with horizontal strabismus. METHODS: Forty patients with horizontal strabismus (20 esotropia and 20 exotropia) were included in this study. Patients with a variable angle of deviation, vertical angle over 5 prism diopters, impaired binocular vision, or poor cooperation were excluded. We instructed the patient to look a target 6 meters away, and applied a prism over the patient's dominant eye while flashing a light source 33 centimeters from the middle of both eyebrows. When the corneal light reflexes were located on the center of each cornea, we measured the angle of deviation. We defined this method as 'distance Krimsky test,' and the angle measured by this method was compared with the conventional Krimsky test and APCT at distance. We analyzed the accuracy and intra- and inter-observer reliability. RESULTS: The angle of strabismus measured by the distance Krimsky test showed a significant agreement and correlation with the deviation angle measured by the APCT. We elicited the correlation gradient between the angle measured by the distance Krimsky test and the APCT. In addition, the distance Krimsky test showed significant intra- and inter-observer reliabilities. CONCLUSIONS: The distance Krimsky test is expected to be more useful than the Krimsky test in measuring the distance angle of deviation for patients with strabismus in whom it is difficult to measure the angle of deviation using the APCT. The distance Krimsky test can be an accurate and useful test through the improvement of proficiency of examiners and the establishment of individualized normative data.

A Case of Pediatric Idiopathic Intracranial Hypertension Presenting with Divergence Insufficiency

An 11-year-old female presenting diplopia only at distance was found to have comitant esotropia of 20 prism diopters (PD) at distance and normal alignment at nearer proximity. Other ocular movement, including abduction, was normal and a thorough neurologic examination was also normal. The deviation angle of esotropia was increased to 35 PD in 6 months, and a brain magnetic resonance imaging with venogram at that time demonstrated no intracranial lesion. A lumbar puncture showed increased opening pressure but the cerebrospinal fluid composition was normal. The patient was diagnosed as having idiopathic intracranial hypertension and treated with oral acetazolamide. Three months after treatment, the deviation angle decreased to 10 PD. This is a case report of divergence insufficiency in pediatric idiopathic intracranial hypertension, with an increasing deviation angle of esotropia. Although sixth cranial nerve palsy is a common neurologic manifestation in intracranial hypertension, clinicians should be aware of the possibility of divergence insufficiency. Also, ophthalmoparesis may not be apparent and typical at first presentation, as seen in this case, and therefore ophthalmologists should be aware of this fact, while conducting careful and proper evaluation, follow-up, and intervention.

Comparison of the Clinical Characteristics of Intermittent Exotropia in Children and Adults

PURPOSE: The purpose of this study was to compare and differentiate the clinical characteristics of intermittent exotropia (X(T)) in children and adults. METHODS: This study included 398 patients with X(T): 360 children ranging in age from 1 to 14 years and 38 adults over 15 years of age. Patients with neurological abnormalities or developmental delays were excluded. Clinical characteristics of interest included sex, age on first visit, age of onset, type of onset, duration to surgery, family history, chief complaints, type of fixation, refractive errors, sensory tests, angle of deviation, fundus examination, oblique muscle dysfunction, and other associated ocular disorders. RESULTS: In both groups, an insidious onset was more common than a sudden onset (p=0.033). Outward deviation was the most common chief complaint in both groups, followed by photophobia in the childhood group, and diplopia in the adult group. The most common subtype of X(T) was the basic type. The mean near deviation was 23.6+/-7.9 prism diopters (PD) in the childhood group and 30.7+/-12.2 PD in the adult group (p=0.01). The mean distance deviation was 23.4+/-6.1 PD and 28.3+/-11.2 PD in the childhood and adult groups, respectively (p=0.028). The rate of fusion, measured with the Worth 4-dot test at near and distance was higher in the childhood group, as compared to the adult group (p=0.024 and p=0.048, respectively). CONCLUSIONS: Among X(T) patients, the chief complaints, angle of deviation, and Worth 4-dot tests showed significant differences between the childhood and adult groups. Therefore, these are important factors to consider when assessing adults and children with X(T).

Exodeviated Ophthalmoplegia in a Patient with Progressive Supranuclear Palsy

We report a patient with progressive supranuclear palsy (PSP) with his serial photographs before the onset of ocular symptoms and after the onset with two year intervals. These photographs show his progressive eyeball deviations toward complete exotropia. There were no effective voluntary eyeball movements, Bell's phenomenon, doll's eye movements, and vestibulo-ocular reflexes. These signs indicate the involvement of the oculomotor nuclear complex by the disease. We suggest that PSP may cause not only 'supranuclear' but also 'nuclear' complete ophthalmoplegia with exodeviation of the eyes.

Bilateral Brown's syndrome in a mother and her son: case report / Síndrome de Brown bilateral em mãe e filho: relato de caso

This case report describes clinical data from mother and son with bilateral Brown's syndrome and highlights possible genetically determined predispositions.

Este relato de caso descreve achados clínicos de mãe e filho com síndrome de Brown bilateral e discute a possibilidade de predisposição genética.

Vertical Rectus Muscles Transposition in Large Exotropia with Medial Rectus Muscle Transection Following Endoscopic Sinus Surgery

PURPOSE: To evaluate the effect of transposition procedures on the vertical rectus muscle (VRM) in the patients who underwent a medial rectus muscle (MR) transection after endoscopic sinus surgery (ESS). METHODS: In 4 patients with exotropia (XT) and a lack of adduction after ESS, orbital CT or MRI revealed a complete transection of the midportion of the MR. Full-tendon VRM transposition was performed within 3 months after injury (early surgery) in 2 patients with 40delta XT. Two patients with 70delta and 85delta XT underwent an X-type augmented Hummelsheim procedure, which involved pulling each half-tendon and crossing it through the undersurface of the severed MR to the other end of the MR insertion, concurrently with an ipsilateral lateral rectus (LR) recession 11 months and 36 months after ESS, respectively. The adduction deficits were divided into -1 through to -8. The patients were followed up for more than than 1.5 years. RESULTS: Postoperatively, 3 patients showed orthophoria and no diplopia in the primary position. The adduction deficits improved to -3.5 or -4. One patient who underwent an X-type augmented Hummelsheim procedure showed a residual XT of 25delta. CONCLUSIONS: VRM transposition is effective in correcting a large XT secondary to a MR transection after ESS. When a longstanding large-angle XT with severe contracture of the ipsilateral LR and massive scarring of the adjacent tissues is present, the X-type augmented Hummelsheim procedure coupled with an ipsilateral LR recession had an augmenting effect.

Achados oftalmológicos em pacientes com múltiplas deficiências / Ophthalmologic findings in multiple handicapped patients

OBJETIVO: Relatar os achados oftalmológicos em portadores de múltiplas deficiências. MÉTODOS: Foram estudados 274 usuários do Sistema Unico de Saúde atendidos no Departamento de Oftalmologia Pediátrica e Estrabismo da Fundação Altino Ventura (FAV), no período de junho a setembro de 2004. RESULTADOS: A freqüência dos pacientes quanto ao gênero foi de 58,5 por cento para o masculino e 41,5 por cento para o feminino. A variação das idades foi de 0,1 a 20 anos com mediana de 5. A maioria (61,3 por cento) dos pacientes apresentava boa acuidade visual, contudo 38,7 por cento apresentava baixa de visão (< 20/80). As heterotropias foram vistas em 66 pacientes (24,0 por cento). Os transtornos refracionais mais freqüentes foram astigmatismo (53,2 por cento) e hipermetropia (29,0 por cento). CONCLUSÃO: Crianças com múltiplas deficiências necessitam de diagnóstico e tratamento oftalmológico precoce, possibilitando um melhor desenvolvimento global. A integração de uma equipe multidisciplinar constituída de pediatras, oftalmologistas pediátricos e profissionais de visão subnormal pode assegurar melhor qualidade na reabilitação visual.

PURPOSE: To report the visual findings in patients with multiple handicaps. METHODS: Two hundred and seventy-four patients cared for at the Pediatric and Strabismus Ophthalmology Department of the "Fundação Altino Ventura" - Brazilian National Health System, were examined from June to September 2004. Age varied from 0.1 to 20 years with a median of 5. RESULTS: The majority of the patients (61.3 percent) presented good visual acuity; however low visual acuity (< 20/80) was observed in 38.7 percent of the patients. Heterotropias were observed in 66 patients (24.0 percent); astigmatism (53.2 percent) and hyperopia (29.0 percent) were more frequent. CONCLUSION: Children with multiple handicaps need an early ophthalmologic diagnosis and treatment for better global development. The integration of a multidisciplinary team with pediatricians, pediatric ophthalmologists and specialists in low vision, may assure a better visual rehabilitation.

Correçäo cirúrgica do estrabismo horizontal em portadores de alta miopia / Surgical correction of horizontal strabismus in patients with high myopia

Objetivo: Análise dos resultados cirúrgicos da correçäo dos estrabismos horizontais em portadores de alta miopia, em pacientes do Departamento de Oftalmologia da Santa Casa de Misericórdia de Säo Paulo. Métodos: Foram estudados os prontuários de 24 pacientes esotrópicos e 17 exotrópicos, portadores de miopia maior que 6,00 DE operados para correçäo do estrabismo. Consideramos como bons resultados cirúrgicos desvios residuais entre esotropia e exotropia de 10. Resultados: Observou-se grande incidência de maus resultados entre os pacientes esotrópicos altos míopes. Conclusäo: Concluímos que existe uma tendência a piores resultados cirúrgicos nos pacientes esotrópicos com miopia maior que -6,00 DE, em comparaçäo com esotrópicos com erro refrativo entre -0,75 DE e+ 3,50 DE.

Clinical characteristics of CHARGE syndrome

CHARGE syndrome, first described by Pagon, was named for its six major clinical features. They are: coloboma of the eye, heart defects, atresia of the choanae, retarded growth and development including CNS anomalies, genital hypoplasia and/or urinary tract anomalies, and ear anomalies and/or hearing loss. We experienced three cases of CHARGE syndrome who displayed ocular coloboma, heart defects, retarded growth and development, and external ear anomalies, and we also review the previously reported literature concerning CHARGE syndrome.

Exploración motora, observación inicial; demostración de la desviación / Motor exploration, initial observation

Se describen los datos de la observación inicial, desvición ocular, características faciales, rotación de la cabeza. Se analiza para la demostración de la desviación, la prueba de la oclusión/desoclusión, la cual ilustra en: presencia o ausencia de desviación, dirección de la desviación (endotroppia, exotropia, hipertropia, hipotropia), estado sensorial binocular (foria=fusión; foria-tropia=fusión intermitente; tropia=supresión), y el estado sensorial monocular (alternancia=no ambliopía; monocularidad=ambliopía).

Exotropias / Exotropia

O Autor discute a exotropia intermitente demonstando os passos necesários para o seu diagnóstico e tratamento. O Controle da exotropia intermitente é feito com recursos clínicos que visam combater a supressäo e aumentar a convergência acomodativa para obter um bom desenvolvimento da visiao binocular e após 6 anos a cirurgia que e feita em duas etapas. Na primeira obtem-se uma hipercorreçäo de 20 diptrias para criar diplopia e na segunda realiza-se uma pequena miotomia para relaxar o RM