Cortical Deficits are Correlated with Impaired Stereopsis in Patients with Strabismus / 神经科学通报·英文版

In this study, we explored the neural mechanism underlying impaired stereopsis and possible functional plasticity after strabismus surgery. We enrolled 18 stereo-deficient patients with intermittent exotropia before and after surgery, along with 18 healthy controls. Functional magnetic resonance imaging data were collected when participants viewed three-dimensional stimuli. Compared with controls, preoperative patients showed hypoactivation in higher-level dorsal (visual and parietal) areas and ventral visual areas. Pre- and postoperative activation did not significantly differ in patients overall; patients with improved stereopsis showed stronger postoperative activation than preoperative activation in the right V3A and left intraparietal sulcus. Worse stereopsis and fusional control were correlated with preoperative hypoactivation, suggesting that cortical deficits along the two streams might reflect impaired stereopsis in intermittent exotropia. The correlation between improved stereopsis and activation in the right V3A after surgery indicates that functional plasticity may underlie the improvement of stereopsis. Thus, additional postoperative strategies are needed to promote functional plasticity and enhance the recovery of stereopsis.

Ileo-Ileal Intussusception with Meckel Diverticulum in a Filipino adolescent

@#Intussusception refers to the invagination (telescoping) of a part of the intestine into itself. Intussusception occurs primarily in infants and toddlers.1 The peak incidence is between 4 and 36 months of age, and it is the most common cause of intestinal obstruction in this age group. 2 Approximately 1 percent of cases are in infants younger than three months, 30 percent between 3 and 12 months, 20 percent between one and two years, 25 percent between two and three years, and 10 percent between three and four years.3 Although intussusception is most common in infants and young children, it is important to consider this diagnosis in children outside this age range. Approximately 10 percent of cases are in children over five years, and 3 to 4 percent in those over 10 years.3,4 We share here images from an actual case of a 15-year-old Filipino male with an ileo-ileal intussusception that is beyond the typical age range, with an incidental finding of an intraluminal mass that was histomorphologically diagnosed as Meckel’s diverticulum (MD). The diagnosis of intussusception is relatively rare in the patient’s age and the diagnosis of MD in the presence of intussusception is sparsely reported in the Philippines. Intussusception refers to the invagination (telescoping) of a part of the intestine into itself. Intussusception occurs primarily in infants and toddlers.1 The peak incidence is between 4 and 36 months of age, and it is the most common cause of intestinal obstruction in this age group. 2 Approximately 1 percent of cases are in infants younger than three months, 30 percent between 3 and 12 months, 20 percent between one and two years, 25 percent between two and three years, and 10 percent between three and four years.3 Although intussusception is most common in infants and young children, it is important to consider this diagnosis in children outside this age range. Approximately 10 percent of cases are in children over five years, and 3 to 4 percent in those over 10 years.3,4 We share here images from an actual case of a 15-year-old Filipino male with an ileo-ileal intussusception that is beyond the typical age range, with an incidental finding of an intraluminal mass that was histomorphologically diagnosed as Meckel’s diverticulum (MD). The diagnosis of intussusception is relatively rare in the patient’s age and the diagnosis of MD in the presence of intussusception is sparsely reported in the Philippines.

Tratamiento farmacológico del estrabismo / Pharmacological treatment of strabismus

La cirugía de la musculatura extraocular ha sido el estándar de atención para tratamiento quirúrgico del estrabismo por más de un siglo. A pesar del gran desarrollo técnico de la cirugía de estrabismo en la actualidad, la utilización de microscopio quirúrgico, el diseño novedoso del instrumental quirúrgico, la calidad de la sutura no reabsorbible; los avances en equipamiento y fármacos anestésicos, la misma no está exenta de complicaciones quirúrgicas, además del tiempo de recuperación que necesita el paciente para reincorporarse a sus actividades sociales, han propiciado una búsqueda permanente del tratamiento farmacológico para el estrabismo. El objetivo de esta revisión bibliográfica es analizar las distintas alternativas farmacológicas disponibles como tratamiento del estrabismo. Para su confección se consultó textos completos y artículos en idiomas español e inglés, disponible en algunas bases de datos. Concluimos que aunque se han estudiado numerosos fármacos, la toxina botulínica que es la más conocida y utilizada mundialmente, seguida de la bupivacaína. Encontramos otros como la IGF I y II (Insuline Growing Factor), capaces de generar un efecto de reforzamiento de la actividad muscular. Y otros que "debilitan" la musculatura extraocular, incluyen la mAb35-Rubicina, BMP4 (Proteína morfogénica ósea). Se continúa su investigación en la actualidad(AU)

Extraocular musculature surgery has been the standard of care for surgical treatment of strabismus for more than a century. Despite the great technical development of strabismus surgery today, the use of a surgical microscope, the novel design of surgical instruments, the quality of the non-absorbable suture; Advances in anesthetic equipment and drugs, it is not exempt from surgical complications, in addition to the recovery time that the patient needs to return to their social activities, have led to a permanent search for pharmacological treatment for strabismus. The objective of this bibliographic review is to analyze the different pharmacological alternatives available as a treatment for strabismus. For its preparation, full texts and articles in Spanish and English languages were consulted, available in some databases. We conclude that although numerous drugs have been studied, botulinum toxin, which is the best known and used worldwide, followed by bupivacaine. We find others such as IGF I and II (Insuline Growing Factor), capable of generating an effect of reinforcing muscle activity. And others that "weaken" MOE include mAb35-Rubicin, BMP4 (Bone Morphogenic Protein). His research is continuing today(AU)

Efectividad de la cirugía monocular en la exotropía sensorial de gran ángulo / Effectiveness of the surgery monocular in the sensorial exotropia of great angle

La exotropía sensorial se define como una desviación ocular divergente unilateral o bilateral, dada por anomalías oculares congénitas o adquiridas; es más frecuente en adultos. El objetivo de la cirugía de estrabismo en el adulto es restablecer la visión binocular, reduciendo la diplopía y al lograr el alineamiento ocular, mejorar su estética, por lo que antes de realizar la cirugía se debe tratar la causa que provoca la baja visión. El procedimiento quirúrgico de elección es la cirugía monocular, pero si la desviación es grande se realizará cirugía binocular. Se presenta una paciente femenino de 25 años de edad portadora de lentes de contacto por miopía elevada del ojo derecho (9,00 -0.75 x 105º con 0,2 de agudeza visual mejor corregida), exotropía de más de 25º por Hirschberg, limitación de aducción del ojo derecho y por método de oclusor y prismas a 6 metros sin cristales y con sus lentes de contacto, ambos ojos 50 ∆ b interna, no estereopsia y suprime ojo derecho. Por todas las ventajas que presenta la cirugía monocular, se decidió realizarla en el ojo derecho, se colocó anestesia local peribulbar, se realizó recesión amplia del recto lateral a 12 mm y se realizó 8 mm de resección del recto medial; en el postoperatorio se logró 10 ∆ b interna y por momentos ortotropia; refirió diplopía post quirúrgica que resolvió espontáneamente. Los resultados quirúrgicos en la exotropía sensorial son menos alentadores ya que con el tiempo pueden evolucionar hacia la hipercorrección o hacia la recurrencia(AU)

Sensory exotropía is defined as a unilateral or bilateral divergent ocular deviation, given by congenital or acquired ocular anomalies, and is more frequent in adults. The objective of the strabismus surgery in adults is to reestablish binocular vision, reduce diplopía, improve ocular alignment and enhance quality of life; before performing surgery the cause of low vision should be treated. The preferred surgical procedure is a monocular approach, but if there is a large deviation a binocular procedure should be performed. The case presented Is a 25 year-old female with high myopia of the right eye (-9.00 -0.75 x 105º with 0.2 of best corrected visual acuity), exotropía of more than 25º for Hirschberg, limitation of aducción of the right eye and with both the oclusor method and prisms to 6 meters without glasses and with her contact lenses, both eyes have 50∆ of internal base, no estereopsia and suppression of the right eye. For its advantages a monocular approach (of the right eye) was preferred, with peribulbar anesthesia: recession to 12mm of the lateral rectus combined with 8 mm of resection of the medial rectus; in the postoperative she presented a 10∆ intern base deviation and for moments ortotropia; she referred diplopia that solved spontaneously. The surgical results in sensory exotropía are less encouraging since they have higher hypercorrection and recurrence rates(AU)

Alteraciones estructurales y funcionales en pacientes con ambliopía / Structural and Functional Alterations in Patients with Amblyopia

Objetivo: Determinar los resultados motores post cirugía refractiva corneal con láser de excímero en pacientes miopes con o sin astigmatismo asociado. Método: Se realizó un estudio preexperimental del tipo antes y después, en el que fueron incluidos 81 pacientes (162 ojos) tratados con LASEK-MMC (65 pacientes) o PRK-MMC (16 pacientes) seguidos por tres meses. Se les realizó un examen oftalmológico completo y se evaluaron las siguientes variables: punto próximo de convergencia, la amplitud de convergencia, divergencia y el alineamiento ocular precirugía y poscirugía. Resultados: Con la cirugía se observó un incremento significativo del punto próximo de convergencia (LASEK-MMC: p = 0,000 y PRK-MMC: p = 0,021). La amplitud de convergencia de cerca y de lejos, así como la amplitud de divergencia tendieron a disminuir, pero la diferencia no fue significativa (p > 0,05). Aumentó el porcentaje de pacientes con exodesviaciones de cerca, después de la cirugía LASEK-MMC (26,2 por ciento → 60,0 por ciento, p = 0,000) y de la PRK-MMC (18,8 por ciento → 81,3 por ciento, p = 0,000), y predominaron las heteroforias pequeñas en ambos casos (LASEK-MMC: 52,3 por ciento y PRK-MMC: 75,0 por ciento). Conclusiones: Con la cirugía fotoablativa con láser, aumentaron las exodesviaciones en el posoperatorio, pero se trató fundamentalmente de heteroforias pequeñas, aunque un número reducido de pacientes desarrolló nuevas exodesviaciones(AU)

Objective: To determine the motor results after corneal refractive surgery with excimer laser in myopic patients with or without associated astigmatism. Method: A pre-experimental study of the before and after type was carried out, in 81 patients (162 eyes) treated with LASEK-MMC (65 patients) or PRK-MMC (16 patients) followed up for three months. A complete ophthalmological examination was performed and the variables were evaluated near point of convergence, amplitude of convergence, divergence, and pre-surgery and postsurgery ocular alignment. Results: A significant increase in the near point of convergence was observed with surgery (LASEK-MMC: p = 0.000 and PRK-MMC: p = 0.021). The amplitude of convergence near and far, as well as the amplitude of divergence tended to decrease, but the difference was not significant (p > 0.05). The percentage of patients with near exodeviations increased after LASEK-MMC surgery (26.2 percent → 60.0 percent, p = 0.000) and PRK-MMC (18.8 percent → 81.3 percent, p = 0.000), and small heterophoria predominated in both cases (LASEK-MMC: 52.3 percent and PRK-MMC: 75.0 percent). Conclusions: The laser photoablative surgery increased postoperative exodeviations, but they were mainly small heterophoria, although a small number of patients developed new exodeviations(AU)

Resultados motores en cirugía fotoablativa en pacientes miopes / Motor Outcomes of Photoablative Surgery in Myopic Patients

Objetivo: Determinar los resultados motores post cirugía refractiva corneal con láser de excímero en pacientes miopes con o sin astigmatismo asociado. Método: Se realizó un estudio preexperimental del tipo antes y después, en el que fueron incluidos 81 pacientes (162 ojos) tratados con LASEK-MMC (65 pacientes) o PRK-MMC (16 pacientes) seguidos por tres meses. Se les realizó un examen oftalmológico completo y se evaluaron las siguientes variables: punto próximo de convergencia, la amplitud de convergencia, divergencia y el alineamiento ocular precirugía y poscirugía. Resultados: Con la cirugía se observó un incremento significativo del punto próximo de convergencia (LASEK-MMC: p = 0,000 y PRK-MMC: p = 0,021). La amplitud de convergencia de cerca y de lejos, así como la amplitud de divergencia tendieron a disminuir, pero la diferencia no fue significativa (p > 0,05). Aumentó el porcentaje de pacientes con exodesviaciones de cerca, después de la cirugía LASEK-MMC (26,2 por ciento → 60,0 por ciento, p = 0,000) y de la PRK-MMC (18,8 por ciento → 81,3 por ciento, p = 0,000), y predominaron las heteroforias pequeñas en ambos casos (LASEK-MMC: 52,3 por ciento y PRK-MMC: 75,0 por ciento). Conclusiones: Con la cirugía fotoablativa con láser, aumentaron las exodesviaciones en el posoperatorio, pero se trató fundamentalmente de heteroforias pequeñas, aunque un número reducido de pacientes desarrolló nuevas exodesviaciones(AU)

Objective: To determine the eye movement outcomes after excimer laser corneal refractive surgery in myopic patients with or without associated astigmatism. Methods: A preexperimental, before-and-after study was carried out with 81 patients (162 eyes) treated with laser-assisted subepithelial keratectomy (LASEK) (65 patients) or photorefractive keratectomy (PRK) (16), with intraoperative application of mitomycin-C (MMC) in both cases, and followed for three months. A complete ophthalmologic examination was performed and the following variables were evaluated before and after the surgery: near convergence point, convergence amplitude, divergence and ocular alignment. Results: With surgery, a significant increase in the near point of convergence was observed (LASEK-MMC: P=0.000; PRK-MMC: P=0.021). The near and far convergence amplitude tended to decrease, but the difference was not significant (P > 0.05). The percentage of patients with near exodeviations increased after LASEK-MMC (26.2 percent → 60.0 percent, P=0.000) and PRK-MMC (18.8 percent → 81.3 percent, P=0.000), while small heterophorias predominated in both cases (LASEK-MMC: 52.3 percent and PRK-MMC: 75.0 percent). Conclusions: With laser photoablative surgery, exodeviations increased after surgery, but these were primarily small heterophorias, although a small number of patients developed new exodeviations(AU)

Quality of life in children with strabismus / Qualidade de vida em crianças com estrabismo

ABSTRACT Purpose: To evaluate the quality of life of children with strabismus and understand their consequences in the functional and psychosocial aspects of the childhood. Methods: A semi-structured interview adapted of Adult Strabismus-20 questionnaire was conducted with parents or the legal responsible person of children aged between 3 and 13 years old attended at the public health care. A likert-scale of five points was used to measure the impact of strabismus in childhood, from psychosocial and functional aspects. Results: 97 children were included in the study. 61,4% of the children had esotropia (ET), while 35.9% had exotropia (XT). In each group, the magnitude of deviation was classified in smaller (<) or equal/greater (≥) then 30 prismatic diopters. When analyzing the functional and the psychosocial scores of the groups separated by the type of strabismus and magnitude of deviation, the average score of the group with esotropia and the patients with larger deviations were worse. The correlation value between the psychosocial and functional scores was 200.656 (p <0.01). Conclusion: Strabismus in childhood is associated with many negative effects. These consequences should be considered when deciding for surgical treatment on early ages. The strabismus treatment may change positively how these children perceive themselves, resulting in benefits on quality of life from both the functional and psychosocial perspectives.

RESUMO Objetivo: Avaliar a qualidade de vida de crianças com estrabismo e entender suas consequências em aspectos funcionais e psicossociais da infância. Métodos: Uma entrevista semiestruturada adaptada do questionário Adult Strabismus-20 foi realizada com os pais ou responsáveis legais das crianças entre 3 e 13 anos atendidas na rede pública de saúde. Uma escala do tipo Likert de cinco pontos foi usada para mensurar o impacto do estrabismo na infância, do ponto de vista funcional e psicossocial. Resultados: Foram incluídas no estudo 97 crianças, sendo 61,4% com esotropia e 35,9% com exotropia. Em cada grupo, a magnitude do desvio foi classificada em menor ou maior que 30 dioptrias prismáticas. Quando analisados os escores funcionais e psicossociais de cada grupo separados por tipo e magnitude do desvio, a média do escore do grupo com esotropia e dos pacientes com maior desvio foi pior. O valor de correlação entre os escores funcionais e psicossociais foi de 0,656 (p<0,01). Conclusão: Estrabismo na infância está associado a muitos efeitos negativos. As consequências devem ser levadas em consideração quando da decisão por tratamento cirúrgico em idades precoces. O tratamento do estrabismo pode alterar de forma positiva a percepção que a criança tem de si mesma, resultando em benefícios na qualidade de vida, tanto em aspectos funcionais quanto psicossociais.

Novo gancho muscular milimetrado para cirurgia de estrabismo / A new millimeter muscle hook for strabismus surgery

RESUMO Objetivo: Avaliar um novo tipo de gancho muscular (gancho milimetrado de Felício) e sua eficácia em cirurgias de estrabismo. Métodos: Buscando uma abordagem independente, com a mínima participação do auxiliar, o novo instrumento foi usado em cirurgias de retrocesso e ressecção, para comparar sua eficácia e segurança com a técnica tradicional. Participaram do estudo 14 pacientes divididos em dois grupos. Resultados: O grupo operado por meio da técnica tradicional teve média de idade foi de 14,7 anos, e o grupo que usou o novo gancho teve média de 17 anos. Ambos os grupos obtiveram redução semelhante do estrabismo inicial, sendo, em média, de 87,84% no grupo tradicional e de 93,04% com o novo gancho, porém sem relevância estatística (p=0,274). Conclusão: O gancho milimetrado de Felício mostrou-se opção útil ao cirurgião na realização da cirurgia de estrabismo com redução da importância do auxiliar, de forma segura e reprodutível.

ABSTRACT Objective: To evaluate a new type of muscle hook (Felício's millimeter hook) and its effectiveness in strabismus surgeries. Methods: Seeking an independent approach, with minimal assistance from the assistant, the new instrument was used in retrocession and resection surgeries, to compare its efficacy and safety with the traditional technique. Results: 14 patients participated in the study, divided into two groups. The group who underwent surgery with the traditional technique had a mean age of 14.7 years and the group using the new hook, 17 years. Both groups obtained a similar reduction in initial strabismus, with an average of 87.84% in the traditional group and 93.04% with the new hook, but without statistically significant difference (p=0.274). Conclusion: Felicio's millimeter hook proved to be a useful option for the surgeon in performing strabismus surgery with a reduction in the importance of the assistant, in a safe and reproducible way.

NEXMIF mutations in intellectual disability and epilepsy: A report of 2 cases and literature review / 中南大学学报(医学版)

More than 100 genes located on the X chromosome have been found to be associated with X-linked intellectual disability (XLID) to date, and NEXMIF is a pathogenic gene for XLID. In addition to intellectual disability, patients with NEXMIF gene mutation can also have other neurological symptoms, such as epilepsy, abnormal behavior, and hypotonia, as well as abnormalities of other systems. Two children with intellectual disability and epilepsy caused by NEXMIF gene mutation were treated in the Department of Pediatrics, Xiangya Hospital, Central South University from March 8, 2017 to June 20, 2020. Patient 1, a 7 years and 8 months old girl, visited our department because of the delayed psychomotor development. Physical examination revealed strabismus (right eye), hyperactivity, and loss of concentration. Intelligence test showed a developmental quotient of 43.6. Electroencephalogram showed abnormal discharge, and cranial imaging appeared normal. Whole exome sequencing revealed a de novo heterozygous mutation, c.2189delC (p.S730Lfs*17) in the NEXMIF gene (NM_001008537). During the follow-up period, the patient developed epileptic seizures, mainly manifested as generalized and absent seizures. She took the medicine of levetiracetam and lamotrigine, and the seizures were under control. Patient 2, a 6-months old boy, visited our department due to developmental regression and seizures. He showed poor reactions to light and sound, and was not able to raise head without aid. Hypotonia was also noticed. The electroencephalogram showed intermittent hyperarrhythmia, and spasms were monitored. He was given topiramate and adrenocorticotrophic hormone (ACTH). Whole exome sequencing detected a de novo c.592C>T (Q198X) mutation in NEXMIF gene. During the follow-up period, the seizures were reduced with vigabatrin. He had no obvious progress in the psychomotor development, and presented strabismus. There were 91 cases reported abroad, 1 case reported in China, and 2 patients were included in this study. A total of 85 variants in NEXMIF gene were found, involving 83 variants reported in PubMed and HGMD, and the 2 new variants presented in our patients. The patients with variants in NEXMIF gene all had mild to severe intellectual disability. Behavioral abnormalities, epilepsy, hypotonia, and other neurological symptoms are frequently presented. The phenotype of male partially overlaps with that of female. Male patients often have more severe intellectual disability, impaired language, and autistic features, while female patients often have refractory epilepsy. Most of the variants reported so far were loss-of-function resulted in the reduced protein expression of NEXMIF. The degree of NEXMIF loss appears to correlate with the severity of the phenotype.

Resultados de la cirugía mínimamente invasiva en pacientes con estrabismos horizontales / Results of the minimally invasive surgery in patient with horizontal strabismus

Objetivo: Determinar los resultados de la cirugía mínimamente invasiva en pacientes con estrabismos horizontales. Métodos: Se realizó un estudio descriptivo, longitudinal y prospectivo de una serie de 19 pacientes (16 ojos derechos, 17 izquierdos) atendidos en el Servicio de Oftalmología Pediátrica del Instituto Cubano de Oftalmología "Ramón Pando Ferrer", en el periodo comprendido entre junio de 2018 y julio de 2019, a quienes se les realizó cirugía mínimamente invasiva de estrabismo y fueron seguidos por 6 meses. Se evaluaron las variables: tipo de desviación, agudeza visual, signos posoperatorios, ángulo de desviación pre- y posquirúrgico, éxito quirúrgico, fusión, estereopsis y complicaciones. Resultados: El 73,7 por ciento de los pacientes presentaba esotropías; la media de agudeza visual pre- y posoperatoria entre los dos ojos fue muy similar; el signo posoperatorio más frecuente fue la hiperemia conjuntival ligera (75 por ciento ojos derechos y 64,5 por ciento ojos izquierdos) a las 24 horas. Se encontraron diferencias estadísticas (p < 0,001) en el ángulo de desviación horizontal en dioptrías prismáticas, pre- y poscirugía, con el 84,2 por ciento de éxito quirúrgico. El 78,6 y el 80 por ciento de los pacientes con esotropía y exotropías alcanzaron fusión, pero solo lograron estereopsis el 28,5 y el 60 por ciento de ellos respectivamente. La frecuencia de complicaciones ocurrió en el 15,8 por ciento de los pacientes. Conclusiones: Se alcanzaron buenos resultados motores, así como aceptables en los sensoriales en pacientes operados de estrabismos horizontales por cirugía mínimamente invasiva(AU)

Objective: Determine the results of minimally invasive surgery in patients with horizontal strabismus. Methods: A prospective longitudinal descriptive study was conducted of a series of 19 patients (16 right eyes, 17 left eyes) attending the Pediatric Ophthalmology Service at Ramón Pando Ferrer Cuban Institute of Ophthalmology from June 2018 to July 2019. These patients underwent minimally invasive strabismus surgery and were followed up for six months. The variables considered were deviation type, visual acuity, postoperative signs, pre- and postoperative angle of deviation, surgical success, fusion, stereopsis and complications. Results: Of the patients studied, 73.7 percent presented esotropias; mean pre- and postoperative visual acuity was very similar in the two eyes; the most common postoperative sign was slight conjunctival hyperemia (75 percent right eyes and 64.5 percent left eyes) at 24 hours. Statistical differences (p < 0.001) were found in the horizontal angle of deviation in pre- and postoperative prism diopters, with 84.2 percent surgical success. 78.6 percent and 80 percent of the patients with esotropia and exotropia achieved fusion, but only 28.5 and 60 percent of them, respectively, achieved stereopsis. Complications occurred in 15.8 percent of the patients. Conclusions: Good motor results were obtained, as well as acceptable sensory results in patients undergoing minimally invasive horizontal strabismus surgery(AU)

Síndrome de pseudo-Duane secundario a Schwannoma de nervio abducens: Reporte de caso y revisión / Pseudo-Duane syndrome secondary to abducens nerve schwannoma: Case report and review

Reporte de caso de síndrome de pseudo-Duane asociado a schwannoma de nervio abducens y neurofibromatosis tipo 2 (NF2). Este síndrome es raro, caracterizado por limitación en abducción, retracción ocular, disminución de hendidura palpebral en abducción y restricción del recto medial ipsilateral en test de ducción forzada. La NF2 es también una enfermedad infrecuente caracterizada por tumores de sistema nervioso central y periférico. Los schwannomas de nervio abducens son también poco frecuentes, y se presentan comúnmente con diplopia. Se revisan brevemente estas enfermedades. Es el primer caso reportado de pseudo-Duane secundario a schwannoma de nervio abducens y NF2 según nuestro conocimiento.

A pseudo-Duane syndrome case associated with abducens nerve schwannoma and neurofibromatosis type 2 (NF2) is presented. This syndrome is a rare disease characterised by abduction limitation, ocular retraction, narrowing of the palpebral fissure in abduction, and ipsilateral medial rectus restriction on forced duction test. NF2 is also an uncommon disease which is characterised by peripheral and central nervous system tumours. Abducens nerve schwannomas are also uncommon and presents usually as diplopia. A short review of these diseases is given. This is the first case of pseudo-Duane secondary to abducens nerve schwannoma, to our knowledge.

Clinical profile of amblyopic children at a Philippine tertiary hospital

Objective@#To describe the clinical profile of patients with amblyopia seen at a Philippine tertiary hospital. @*Methodology@#This was a cross-sectional study utilizing a chart review of children ages 6 months to 18 years old diagnosed with amblyopia at a Philippine tertiary hospital. Records with complete entry of history and ocular examinations were included. Verbal children with best-corrected visual acuity in both eyes of less than 6/15 or a 2 line difference between eyes were considered amblyopic. Response to alternate occlusion or refixation patterns was used as a basis for diagnosing amblyopia in pre-verbal children. Patients with visual deprivation amblyopia such as from cataract who had undergone lens extraction and optical correction were included. @*Results@#There were 334 patients included in the study. The mean age was 5±3 years with 52% being male. Deprivation type of amblyopia was present in 192 (58%) patients, strabismic type in 81 (24%), and refractive type in 61 (18%). No sex predilection was observed. Majority of the patients were 3-6 years old on initial consult. Cataract was the most common cause of deprivation amblyopia affecting 127 (66%) patients. Strabismic amblyopia was present in 58 patients with 74% being esotropic. Anisometropic refractive amblyopia was more common at 37 (58%) than isometropic refractive amblyopia. @*Conclusion@#Visual deprivation amblyopia was the most common cause of amblyopia in a Philippine tertiary hospital.

Transplante autólogo de músculo ocular extrínseco para tratamento cirúrgico de estrabismos sensoriais grandes / Autologous graft of extraocular muscle to treat large angle sensory strabismus

RESUMO Apresenta-se uma série de 13 casos de pacientes com estrabismo sensorial de grande ângulo submetidos à técnica cirúrgica de autotransplante da musculatura ocular extrínseca. Foi realizada a técnica de recuo-ressecção dos músculos retos horizontais, e o retalho retirado do músculo ressecado foi suturado ao músculo enfraquecido como expansor autólogo. Foram avaliadas seis exotropias e sete esotropias, com desvios médios de 75 (70-90) dioptrias prismáticas (DP). Houve melhora significativa dos desvios no pós-operatório, sendo a média pós-operatória de 10,07 dioptrias prismáticas (ortotropia a 35DP). Somente um dos casos evoluiu com inversão do desvio após procedimento cirúrgico.

ABSTRACT We report 13 cases of large angle sensory strabismus treated with autologous graft of extraocular muscle. Recession-resection procedure of the horizontal rectus muscles was performed, and the flap from the resected muscle was sutured to the weakened muscle as an autologous expander. Six cases of exotropia and seven of esotropia, with mean prism diopter deviation of 75 (range of 70-90). There was significant improvement in the postoperative deviation, and mean prism diopter of 10.07 (range of no deviation to 35). Only one patient progressed with inverted misalignment after the surgical procedure.

Strabismic amblyopia: compliance with occlusion treatment in a tertiary hospital in Midwestern Brazil / Ambliopia estrabísmica: adesão ao tratamento oclusivo em hospital terciário no Centro-Oeste Brasileiro

Abstract Purpose: Study aimed to determine compliance of patients with strabismic amblyopia undergoing occlusion treatment, followed from January 1 st, 2011 to January 1 st, 2017 at an Ophthalmology Reference Center, and identify risk factors for poor compliance. Methods: Retrospective, consecutive cohort study. Compliance reported at each visit was related to visual acuity, family history, changes in occlusion schedules and in patients' care team during treatment. The patients were divided into two groups according to the compliance: poor compliance group and compliance group (subdivided in full compliance subgroup and partial compliance subgroup). Results: Age at treatment beginning vary from 3.7 to 13.7 years, esotropia was the most frequent deviation and the occlusion was realized from 5 to 7 hours a day. Of 220 patients, compliance was achieved by 193 (87.7%), 114 (51.8%) in full compliance subgroup and 79 (35.9%) in partial compliance subgroup, and 27 do not achieved compliance (12.3%). Poor compliance was significantly related to a history of epilepsy, higher rate of suspension of treatment due to inefficacy, higher evasion rate, lower recurrence, and lower rate of maintenance of prophylactic occlusion after treatment. Good compliance was related to family history of strabismus, higher recurrence rate, and higher maintenance of prophylactic occlusion after treatment. No relations were found between poor compliance and changes in occlusion schedules or in patients' care team during treatment. Conclusion: Compliance with occlusion treatment of strabismic ambliopia was similar to other studies that included refractive and strabismic amblyopia and not related to changes in occlusion schedules or in patients' care team during treatment. Family history of strabismus was a protective factor.

Resumo Objetivo: Determinar a adesão de pacientes com ambliopia estrabísmica submetidos a tratamento oclusivo, acompanhados em Centro de Referência em Oftalmologia do Centro-Oeste do Brasil, e identificar fatores de risco para baixa adesão. Métodos: Estudo de coorte retrospectivo e consecutivo. A adesão relatada em cada visita foi relacionada à acuidade visual, história familiar, mudanças no tempo de oclusão e na equipe de atendimento ao paciente durante o tratamento. Os pacientes foram divididos em dois grupos de acordo com a adesão: grupo de não-adesão e grupo adesão (subdividido em subgrupo adesão total e subgrupo adesão parcial). Resultados: A idade ao início do tratamento variou de 3,7 a 13,7 anos, o desvio mais frequente foi a esotropia, o tempo médio de oclusão foi de 5 a 7 horas por dia. Dos 220 pacientes, 193 (87,7%) obtiveram adesão, 114 (51,8%) no subgrupo de adesão total e 79 (35,9%) no subgrupo de adesão parcial, e 27 não aderiram (12,3%). A não-adesão foi relacionada à história de epilepsia, maior taxa de suspensão do tratamento devido à ineficácia, maior taxa de evasão, menor recorrência e menor taxa de manutenção da oclusão profilática pós tratamento. A boa adesão foi relacionada à história familiar de estrabismo, maior taxa de recorrência e maior manutenção da oclusão profilática pós tratamento. Não houve relação entre não-adesão e mudanças nos horários de oclusão ou na equipe de atendimento durante o tratamento. Conclusão: A adesão ao tratamento oclusivo da ambliopia estrabísmica foi semelhante aos estudos que incluíram ambliopia refracional e estrabísmica e não se correlacionou a mudanças no tempo de oclusão ou na equipe de atendimento. A história familiar de estrabismo foi um fator de proteção.

Complex strabismus: a case report of hypoplasia of the third cranial nerve with an unusual clinical presentation / Estrabismo complexo: um caso relacionado à hipoplasia do terceiro nervo craniano com apresentação clínica incomum

ABSTRACT Congenital cranial dysinnervation disorders are a group of complex strabismus syndromes that present as congenital and non-progressive ophthalmoplegia. The genetic defects are associated with aberrant axonal targeting onto the motoneurons, development of motoneurons, and axonal targeting onto the extraocular muscles. We describe here the surgical management of a 16-year-old boy who presented with complex strabismus secondary to hypoplasia of the third cranial nerve and aberrant innervation of the upper ipsilateral eyelid.

RESUMO Os distúrbios de inervação craniana congênita en­globam um grupo de síndromes associadas a estrabismos complexos, que se apresentam como oftalmoplegia congênita e não progressiva e são frequentemente herdadas. Os defeitos dos genes estão associados a erros no desenvolvimento ou direcionamento axonal dos motoneurônios, e erros no direcionamento axonal para os músculos extraoculares. Este caso descreve o caso de um menino que apresenta estrabismo complexo secundário à hipoplasia do terceiro nervo craniano e inervação aberrante da pálpebra superior ipsilateral, bem como o resultado após a correção cirúrgica.

La tecnología en la estrabología / Technology in strabismus exploration

RESUMEN La exploración oculomotora incluye una extensa batería de exámenes que fundamentalmente en los niños y en los adultos poco cooperadores se hace compleja y difícil por su poca cooperación, el agotamiento y el entretenimiento frecuente. Esto se ha visto modificado con el surgimiento de la videoculografía, lo que permite el estudio de los movimientos oculares en tiempo real. Con esta revisión bibliográfica se pretende reflejar su utilidad, así como las ventajas y los inconvenientes de esta tecnología, para lo cual se realizó una búsqueda en diferentes publicaciones y textos de la especialidad desde hace 14 años. Concluimos que la videoculografía ha revolucionado la exploración del paciente estrábico y permite un examen preciso y rápido, lo que facilita el desarrollo de numerosas investigaciones y constituye una técnica con gran futuro(AU)

ABSTRACT Oculomotor exploration comprises a large set of tests, the performance of which may be difficult with children and unhelpful adults, because they are not cooperative, become tired and get distracted. But such a situation has changed with the emergence of videoculography, which makes it possible to observe eye movements in real time. The purpose of the present bibliographic review was to gain insight into the usefulness, advantages and drawbacks of this technology, to achieve which a search was conducted in various publications and texts about the specialty since 14 years ago. We conclude that videoculography has revolutionized strabismus exploration, enabling accurate and fast examination and facilitating the development of numerous studies, all of which makes it a very promising technique(AU)

Bupivacaine injection for comitant horizontal strabismus: clinical and radiological results / Injeção de bupivacaína para estrabismo comitante horizontal: resultados clínicos e radiológicos

ABSTRACT Purpose: To report the outcomes of bupivacaine injection for the treatment of comitant horizontal strabismus and evaluate clinical effectiveness and associated radiological changes. Methods: This prospective observational clinical study was conducted on 10 patients with comitant horizontal strabismus of up to 40 prism diopters. Ophthalmologic examinations and three-dimensional orbital magnetic resonance imaging were performed pre and post-injection (at first, third, and 12th months). A 4.5 ml of 0.5% bupivacaine was injected into the extraocular muscle under topical anesthesia using an electromyography in all patients. Results: The mean follow-up time at post bupivacaine injection and mean deviation at primary position were was 17 ± 2 months and 21.3 prism diopters, respectively. The mean changes in ocular alignment, enlargement of the cross-sectional area in the injected muscle, and volumetric enlargement were 7.7 PD, 12%, and 17% at the first year post-injection, respectively. No severe or persistent complication was observed. Ptosis and mydriasis were noted post-injection due to the anesthetic effects of bupivacaine but disappeared within 2 h post-injection. Conclusions: Bupivacaine injection improved eye alignment in small-angle horizontal comitant strabismus, effectively diagnosed with orbital magnetic resonance imaging to evaluate volumetric changes of the extraocular muscles. Further clinical studies with larger numbers of patients should be performed to define optimal dosages, concentration, and application method and dose-response relationship.

RESUMO Objetivo: Relatar resultados da injeção de bupivacaína para o tratamento do estrabismo comitante horizontal e avaliar sua eficácia clínica e as alterações radiológicas associadas. Métodos: Este estudo clínico observacional prospectivo foi realizado em 10 pacientes com estrabismo comitante horizontal de até 40 dioptrias de prisma. Exames oftalmológicos e ressonância magnética orbital tridimensional foram realizados pré e pós-injeção (no primeiro, terceiro e 12º mês). A 4,5 mL de bupivacaína a 0,5% foi injetado no músculo extraocular sob anestesia tópica usando eletromiografia em todos os pacientes. Resultados: O tempo médio de acompanhamento pós-injeção de bupivacaína e o desvio médio na posição primária foram de 17 ± 2 meses e 21,3 dioptrias de prisma, respectivamente. As alterações médias no alinhamento ocular, aumento da área da secção transversal no músculo injetado e aumento volumátrico foram de 7,7 PD, 12% e 17% no primeiro ano pós-injeção, respectivamente. Nenhuma complicação grave ou persistente foi observada. Ptose e midríase foram observadas após a injeção devido ao efeito anestésico da bupivacaína, mas desapareceram dentro de duas horas após a injeção. Conclusões: A injeção de bupivacaína melhorou o alinhamento dos olhos no estrabismo comitante horizontal de pequeno ângulo, efetivamente diagnosticado com ressonância magnética orbital para avaliar as alterações volumétricas dos músculos extraoculares. Outros estudos clínicos, com maior número de pacientes devem ser realizados para definir dosagens, concentração, método de aplicação e a relação dose-resposta.

Anterior chamber depth and axial length affect clinical performance of Spot Vision Screener / A profundidade da câmara anterior e o comprimento axial afetam o desempenho clínico do Spot Vision Screener

ABSTRACT Purpose: The aim of this study was to evaluate the effect of anterior chamber depth and axial length on clinical performance of the Spot Vision Screener in detecting amblyopia risk factors in children aged 3-10 years. Methods: A total of 300 eyes from 150 patients aged 3-10 years were prospectively tested with Spot Vision Screener (firmware version 3.0.02.32, software version 3.0.04.06) and a standard autorefractometer (Nidek ARK-1). The anterior chamber depth and axial length were measured with an optical biometer (Nidek AL-Scan). The sensitivity and specificity values for detecting significant refractive errors using the referral criteria of the American Association for Pediatric Ophthalmology and Strabismus were determined. Pearson's correlation analysis was employed to evaluate the relationship between the Spot Vision results and the anterior chamber depth and axial length. Results: Compared with the standard autorefractometer results, the Spot Vision Screener's sensitivity and specificity was 59% and 94%, respectively. The differences between the cycloplegic autorefractometer and the Spot Vision Screener spherical equivalents were negatively correlated with anterior chamber depth (r=-0.48; p<0.001) and axial length (r=-0.45; p<0.001). Conclusion: The Spot Vision Screener has moderate sensitivity and high specificity, using the criteria of the American Association for Pediatric Ophthalmology and Strabismus. The anterior chamber depth and axial length affect the Spot Vision results.

RESUMO Objetivo: O objetivo deste estudo foi avaliar o efeito da profundidade da câmara anterior e do comprimento axial sobre o desempenho clínico do Spot Vision Screener, na deteção de fatores de risco para a ambliopia em crianças de 3 a 10 anos de idade. Métodos: Um total de 300 olhos de 150 pacientes de 3-10 anos de idade foram prospectivamente testados com o Spot Vision Screener (firmware: 3.0.02.32, software: 3.0.04.06) e com autorefratómetro padrão (Nidek ARK-1). Todas as medições de profundidade e comprimento axial da câmara anterior dos pacientes foram realizadas através de Nidek AL Scan. A sensibilidade e especificidade para a deteção de erros refrativos significativos foram determinadas de acordo com os critérios de referência da Associação Americana de Oftalmologia e Estrabismo Pediátricos. A análise da Correlação de Pearson foi utilizada para avaliar a correlação entre os resultados do Spot Vision e a profundidade ou comprimento axial da câmara anterior dos pacientes. Resultados: Em comparação com os resultados do autorefratómetro padrão, a sensibilidade do Spot foi de 59% e a especificidade de 94%. As diferenças entre os equivalentes esféricos do autorefratómetro cicloplégico e o Spot Vision Screener foram correlacionados negativamente com a profundidade (r=-0,48; p<0,001) e o comprimento axial (r=-0,45; p<0,001) da câmara anterior dos casos. Conclusão: O Spot Vision Screener possui uma sensibilidade moderada e uma especificidade elevada utilizando os critérios da Associação Americana de Oftalmologia Pediátrica e Estrabismo; a profundidade da câmara anterior e o comprimento axial dos pacientes afetam os resultados do Spot Vision.

Serious adverse event: late neurotropic disease associated with yellow fever vaccine / Evento adverso grave: doença neurotrópica tardia associada à vacina da febre amarela

ABSTRACT The yellow fever is a systemic disease that was under control due to the effective campaigns against the vector and promotion of vaccines programs. However, since 1999, outbreaks appeared because of inefficient control of the vector, and led to the need of amplifying the immunization in large scale against the yellow fever virus, and consequently, raising the risk of adverse reactions to the vaccine. We report a case of previously healthy infant, who was referred to our care service, after 3 days with fever, chills, nausea and vomits, he received support therapy and was discharged from the hospital. After 24 hours of supportive measures, he was discharge. The patient returned to our service with general condition decline, strabismus, inability to control of cervical musculature and reduced force of the legs. The patient vaccine had received all vaccines from the calendar, and he was vaccinated for yellow fever 20 days before symptoms. During the hospitalization, liquor was collected, and ceftriaxone and aciclovir were administered. After negative cultures from the liquor, the antibiotics were suspended. The computed tomography of patient's brain showed no alterations. Research for antibodies against yellow fever was requested, being positive for IgM in the liquor, and confirming the neurotropic disease associated with the yellow fever vaccine. On the fifth day of hospitalization, the patient showed improvement on the strabismus, cervical tonus, and musculature force. On the tenth day of hospitalization, patient showed complete improvement, and his laboratory exams no alterations. Subsequently, patient was discharged. The vaccine against yellow fever is safe, efficient and highly recommended, however it is not completely free from serious adverse reactions, including death.

RESUMO A febre amarela é uma doença sistêmica que estava controlada graças às efetivas campanhas de combate ao vetor e aos programas de vacinação. Porém, desde 1999, os surtos reiniciaram-se, devido à ineficácia do controle do vetor, levando à necessidade da imunização em larga escala contra o vírus da febre amarela, gerando aumento do risco de ocorrência de reação adversa à vacina. O presente estudo se propôs a relatar o caso de um lactente previamente saudável, que procurou pronto atendimento, pois, há 3 dias, apresentava febre, calafrios, náusea e vômitos. Em 24 horas após medidas de suporte e alta, evoluiu com queda do estado geral, estrabismo, falta de controle da musculatura cervical e redução da força muscular de membros inferiores. O caderno vacinal encontrava-se completo, tendo recebido vacina contra febre amarela há 20 dias. Durante a internação, foi realizada coleta do liquor, e foram administrados ceftriaxona e aciclovir. Após cultura negativa do liquor, o antibiótico foi suspenso. A tomografia computadorizada de crânio não apresentou alterações. Solicitou-se pesquisa de anticorpos contra o vírus da febre amarela no liquor, sendo positiva para IgM e confirmando a doença neurotrópica associada à vacina da febre amarela. A partir do quinto dia de internação, o paciente evoluiu com melhora do estrabismo, do tônus cervical e da força muscular. No décimo dia de internação, apresentou melhora completa do quadro, sem alterações laboratoriais, recebendo alta. A vacina contra febre amarela é segura, eficaz e fortemente recomendada, porém não está completamente isenta de reações adversas graves, inclusive podendo levar a quadros fatais.