Detección y caracterización de defectos del campo visual mediante perimetría Octopus en glaucoma congénito primario / Detection and characterisation of visual field defects using Octopus perimetry in congenital glaucoma

Detectar y caracterizar los defectos del campo visual (CV) mediante perimetría Octopus en pacientes con glaucoma congénito primario (GCP) y determinar la calidad y duración del CV. Material y métodos: Se incluyeron 88 ojos de 70 pacientes diagnosticados de GCP. Las evaluaciones se realizaron con un Octopus 900 y cada ojo se evaluó con el algoritmo de perimetría orientada por tendencias (G-TOP). Se recogieron datos cuantitativos de CV: datos de calidad (respuestas falsa positiva y negativa, y duración del tiempo) y resultados de desviación media (DM) y raíz cuadrada de la varianza de pérdida (sLV). También se recogieron datos cualitativos: presencia de defectos difusos y localizados, hemicampo afectado y grado de defectos utilizando la clasificación de Aulhorn y Karmeyer. Se analizaron las correlaciones entre los resultados perimétricos y las variables clínicas. Resultados: La mediana de edad fue de 11 (8-17) años. El 65,9% (58/88) de los ojos con GCP presentaban defectos de CV. Se observaron defectos difusos en 10/58 ojos (16,94%) (DM media=23,92 [DE: 2,52]) dB) y defectos localizados en 48/58 ojos (82,75%). El defecto más frecuente fue el escotoma incipiente paracentral (n=15), el escalón nasal (n=8), el defecto arciforme añadido (n=2), el semianular (n=13) y el defecto concéntrico con isla central (n=9). El hemicampo visual afectado con mayor frecuencia fue el inferior. La duración media de la prueba fue de 2min 12s (DE: 21,6s). Los valores MD y sLV se correlacionaron con la agudeza visual mejor corregida, la relación excavación/disco y el número de cirugías de glaucoma (todas p<0,001). Conclusión: Se identificó un alto número de defectos difusos y localizados utilizando la perimetría Octopus en pacientes con GCP. El defecto más frecuente fue el escotoma paracentral, y el hemicampo inferior fue el más afectado.(AU)

Purpose: To detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. Material and methods: Eighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. ResultsMedian age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD=23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n=15), nasal step (n=8), adding arcuate defect (n=2), half ring-shaped (n=13) and concentric defect with a central island (n=9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2min 12s (SD: 21.6s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P<.001). Conclusion: A high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.(AU)

Detection and characterisation of visual field defects using Octopus perimetry in congenital glaucoma.

PURPOSE: To detect and characterise visual field (VF) defects using static Octopus perimetry in patients with primary congenital glaucoma (PCG) and to determine VF quality and time duration. MATERIAL AND METHODS: Eighty-eight eyes of 70 patients diagnosed with PCG were included. Assessments were performed using an Octopus 900 and each eye was assessed with the tendency-oriented perimetry (G-TOP) algorithm. Quantitative VF data were collected: quality data (false positive and negative response, and time duration) and results of mean deviation (MD) and square root of loss variance (sLV). Qualitative data were collected: the presence of diffuse and localized defects, the affected hemifield and grade of defects using the Aulhorn and Karmeyer classification. Correlations between perimetric results and clinical variables were analysed. RESULTS: Median age was 11 (8-17) years. 65.9% (58/88) of PCG eyes showed VF defects. Diffuse defects were observed in 10/58 eyes (16.94%) (mean MD = 23.92 [SD: 2.52]) dB) and localized defects in 48/58 eyes (82.75%). The most frequent defect was spot-like/stroke-like/incipient paracentral scotoma (n = 15), nasal step (n = 8), adding arcuate defect (n = 2), half ring-shaped (n = 13) and concentric defect with a central island (n = 9). And the most frequent affected visual hemifield was inferior hemifield. Mean test duration was 2 min 12 s (SD: 21.6 s). MD and sLV values were correlated with best corrected visual acuity (BCVA), cup to disc ratio and number of antiglaucoma surgeries (all P < .001). CONCLUSION: A high number of diffuse and localized defects were identified using Octopus perimetry in PCG patients. The most frequent defect was paracentral scotoma and inferior hemifield was the most affected.

Referral reasons for evaluating childhood glaucoma in a tertiary service / Motivos de encaminhamento para avaliação de glaucoma infantil em serviço terciário

ABSTRACT Purpose: To report the distribution of referral reasons for children from a pediatric glaucoma outpatient clinic in a tertiary eye care service. Methods: The medical records of patients aged <18 years who were referred to a pediatric glaucoma center in the city of São Paulo, Brazil, between 2012 and 2018 were retrospectively reviewed. The data collected included the referral reasons, age, hospital of origin, and who detected the ocular alteration. For defining the diagnosis, the Childhood Glaucoma Research Network classification was used. Results: Five hundred sixty-three eyes of 328 patients were included in the study. Glaucoma diagnosis was confirmed in 162 children (49%). In 83 patients (25%), the glaucoma diagnosis was ruled out, and 83 (25%) continued outpatient follow-up for suspected glaucoma. The main referral reasons were a cup-to-disc ratio >0.5 or an asymmetry ≥0.2 (24%), intraocular pressure >21 mmHg (21%), and corneal opacity (15%). In the neonatal period, the referral reasons were corneal opacity, buphthalmos, tearing, and photophobia. After the neonatal period, besides these external changes, other signs were also reasons for referral, such as cup-to-disc ratio >0.5 or asymmetry ≥0.2, intraocular pressure >21 mmHg, and myopic shift. The referrals were made by health professionals in 69% and parental concern in 30% of the cases. In 97% of the primary congenital glaucoma cases, the parents were the first to identify the change and to seek for health care. Conclusions: The referral reasons of the children to a tertiary glaucoma clinic were differed between the age groups and diagnoses. We suggest that awareness with these findings is important to avoid and postpone diagnosis, identify their impacts on prognosis, and avoid spending important resources for the management of diseases with inaccurate referrals.

RESUMO Objetivos: Relatar a distribuição dos motivos de encaminhamento de crianças para ambulatório de glaucoma infantil em um serviço oftalmológico terciário. Métodos: Dados médicos de pacientes menores que 18 anos encaminhados para ambulatório de glaucoma infantil na cidade de São Paulo, Brasil, de 2012 a 2018 foram retrospectivamente analisados. Os dados incluíram os motivos de encaminhamento, a idade, a origem e quem detectou a alteração ocular. Para definição diagnóstica, a classificação do Childhood Glaucoma Research Network foi usada. Resultados: 563 olhos de 328 pacientes foram incluídos no estudo. O diagnóstico de glaucoma foi confirmado em 162 crianças (49%). 83 (25%) pacientes tiveram diagnóstico de glaucoma descartado, e 83 (25%) continuaram em acompanhamento como glaucoma suspeito. Os principais motivos de encaminhamento foram relação escavação-disco >0,5 ou assimetria ≥0,2 (24%), pressão intraocular >21 mmHg (21%) e opacidade corneana (15%). No período neonatal, os motivos de encaminhamento foram opacidade corneana, buftalmo, lacrimejamento e fotofobia. Após o período neonatal, além desses sinais externos, outros sinais foram também motivos de encaminhamento, como escavação-disco >0,5 ou assimetria ≥0,2, pressão intraocular >21 mmHg e miopização. Os encaminhamentos ocorreram por profissionais de saúde em 69% e preocupação dos pais em 30%. Os pais foram os primeiros a identificar as alterações e procurar cuidado médico em 97% dos casos de glaucoma congênito primário. Conclusões: Os motivos de encaminhamento de crianças para um serviço de glaucoma de glaucoma terciário foram determinados e diferem em diferentes faixas etárias e grupos. Os autores reforçam a necessidade de alertar diferentes grupos para os sinais mais comuns, a fim de evitar, não só o adiamento do diagnóstico, o que impacta no prognóstico, mas também despender recursos importantes direcionados ao enfrentamento dessas doenças, com encaminhamentos imprecisos.

Síndrome de Axenfeld-Rieger con glaucoma asociado / Axenfeld-Rieger syndrome associated with glaucoma

El síndrome de Axenfeld-Rieger es una enfermedad congénita que puede estar asociada a glaucoma en el 50 por ciento de los casos. Por esta razón se describen un caso clínico con síndrome de Axenfeld-Rieger y glaucoma bilateral. Se trata de un paciente masculino de 18 años con escleras azules, microcórnea, embriotoxon posterior, aniridia parcial, atrofia sectorial del iris y alteraciones sistémicas asociado a glaucoma, al que se decide realizar trabéculo-trabeculectomía en ambos ojos. Al mes de la cirugía se constata un aumento de las presiones intraoculares del ojo izquierdo y se realiza trabéculo-trabeculectomía con mitomicina C, posteriormente se hace necesario implantar válvula de Ahmed con mitomicina C en dicho ojo e implantar dispositivo de molteno en ojo derecho, el cual necesitó revisión con aguja y administración de mitomicina C para mantener presiones intraoculares dentro de límites normales con tratamiento médico asociado en ojo izquierdo(AU)

Axenfeld-Rieger syndrome is a congenital disease that can be associated with glaucoma in 50 percent of cases. For this reason, a clinical case with Axenfeld-Rieger syndrome and bilateral glaucoma is described. This is an 18-year-old male patient with blue sclera, microcornea, posterior embryotoxon, partial aniridia, sectorial atrophy of the iris, and systemic alterations associated with glaucoma, who decided to perform trabeculo-trabeculectomy in both eyes. One month after surgery, an increase in intraocular pressures in the left eye was observed and a trabeculo-trabeculectomy was performed with mitomycin C, subsequently it was necessary to implant Ahmed's valve with mitomycin C in said eye and implant a molteno device in the right eye, the which required revision with a needle and administration of mitomycin C to maintain intraocular pressures within normal limits with associated medical treatment in the left eye(AU)

Aniridic glaucoma: An update.

Aniridia is a congenital bilateral ocular disorder with dominant autosomal inheritance. More than 50% of patients will develop aniridic glaucoma (AG) during their lives. Open angle glaucoma is more common in aniridia, but a closed angle mechanism has been described in relation with anterior rotation of the rudimentary iris, occluding trabecular meshwork. Diagnosis and follow-up of AG is difficult in relation with the presence of keratopathy, nystagmus and foveal hypoplasia. Central corneal thickness usually measures more than 600 microns, which prevents achieving a reliable value of intraocular pressure. Medical treatment of AG is no different from the rest of glaucoma. It is recommended to use preservative free formulations, and combined therapy is often required. Surgical treatment is needed in many cases. There is no consensus on the first line surgery for AG, but in open angle AG, angle surgery is usually first choice, and glaucoma drainage devices are the next preferred surgical technique. In closed angle AG glaucoma drainage devices are usually the first choice, with trabeculectomy as the second preferred surgical technique.

Short-term results of ab-interno trabeculotomy using Kahook Dual Blade in patients with primary congenital glaucoma / Resultados de curto prazo da trabeculotomia ab-interno usando o Kahook Dual Blade em pacientes com glaucoma congênito primário

ABSTRACT The aim of this study is to present the results of ab-interno trabeculotomy using Kahook Dual Blade in patients with primary congenital glaucoma. An ab-interno trabeculotomy using a dual blade device was performed in three eyes of two patients with the diagnosis of primary congenital glaucoma. One of them in the left eye and the other patient in both eyes. In the first patient, an adequate response was achieved after the intraocular pressure reduced from 36 mmHg to 14 mmHg. The second patient did not respond adequately to the procedure, and high intraocular pressure levels persisted in both eyes after the procedure. The indication of Kahook Dual Blade ab-interno trabeculotomy in primary congenital glaucoma must be cautious and more studies are needed to establish its efficacy and the best indications. Seems that this procedure should not be indicated for primary congenital glaucoma treatment.

RESUMO O objetivo deste estudo é apresentar os resultados da trabeculotomia ab-interno com Kahook Dual Blade em pacientes com glaucoma congênito primário. Foi realizada trabeculotomia ab-interno com dispositivo de lâmina dupla em 3 olhos de 2 pacientes com diagnóstico de glaucoma congênito primário. Um deles no olho esquerdo e o outro paciente nos dois olhos. No primeiro paciente, houve resposta adequada uma vez que a pressão intraocular diminuiu de 36 mmHg para 14mmHg. O segundo paciente não respondeu adequadamente ao procedimento, mantendo altos níveis de pressão intraocular em ambos os olhos após o procedimento. A indicação da trabeculotomia ab-interno com o Kahook Dual Blade no glaucoma congênito primário deve ser cautelosa e são necessários mais estudos para estabelecer a eficácia e as melhores indicações. Parece que esse procedimento não deve ser indicado no tratamento do glaucoma congênito primário.

Comparación entre el tonómetro de rebote IC200 y el tonómetro de aplanación Perkins en sujetos sanos y pacientes con glaucoma congénito / Comparision of intraocular pressure measured using the new icare 200™ rebound tonometer and the Perkins™ applanation tonometer in healthy subjects and in patients with primary congenital glaucoma

Objetivo Comparar las medidas de presión intraocular (PIO) obtenidas con el tonómetro de rebote iCare 200 (IC200) con las obtenidas mediante la versión portátil del tonómetro de aplanación Goldmann, Perkins (GAT) en pacientes con glaucoma congénito primario (GCP) y en sujetos sanos. Material y métodos Se incluyeron 42 sujetos sanos (G1) y 40 pacientes con GCP (G2). Se incluyó un ojo por paciente. Se recogieron las variables clínicas de interés: sexo, edad, grosor corneal central (GCC) y se midió la PIO mediante los tonómetros IC200 y GAT en el mismo orden, en consulta. Se estudió la concordancia entre tonómetros mediante el coeficiente de correlación intraclase y el gráfico de Bland Altman. La influencia de las variables se analizó mediante test de regresión lineal. Resultado Las medias de PIO obtenidas mediante IC200 y GAT fueron: G1=15,91 (2,57) vs. 15,06 (2,12) mmHg (diferencia de medias, DM=0,84 (0,50) mmHg; p=0,101) y en el G2=20,10 (6,37) vs.19,12 (5,62) (DM=0,98 [1,36]; p=0,474). Se observó excelente concordancia entre IC200/GAT en ambos los grupos (coeficiente de correlación intraclase=G1: 0,875 [IC 95%: 0,768-0,933; p<0,001]; G2: 0,924 [IC 95% 0,852-0,961; p<0,001]), así como la influencia del GCC en la diferencia entre tonómetros en el G1 (B=0,021; IC 95%: 0,005-0,037; p=0,008), sin significación estadística en el G2. Conclusión Se ha encontrado una excelente concordancia entre ambos tonómetros, IC200 y GAT tanto en sujetos sanos como en pacientes con GCP, con una tendencia a la sobreestimación de la PIO de IC200 sobre Perkins. No se ha demostrado la influencia del GCC en los pacientes con GCP (AU)

Objective To compare intraocular pressure (IOP) measurements obtained using the Icare 200™ (IC200) rebound tonometer and the hand-held version of the Goldmann Applanation Tonometer (Perkins™ tonometer, GAT) in patients with primary congenital glaucoma (PCG) and in healthy subjects Material and method a total of 42 eyes of healthy subjects (G1) and 40 patients with PCG (G2) were analysed. The following clinical data were collected: gender, age, Cup/Disc ratio, central corneal thickness (CCT). IOP was determined in the examination room using the IC200 and GAT tonometers, in the same order Agreement between both tonometers was determined using the intraclass correlation coefficient (ICC) and Bland-Altman plot. A linear regression analysis was used to establish the IOP was affected by the studied variables. Results Mean IOP between both tonometers (IC200 minus GAT) was: G1=15.91 (2.57) mmHg vs. 15.06 (2.12) mmHg (mean difference, MD=0.84 (0.50) mmHg; P<.101) and G2=20.10 (6.37) vs.19.12 (5.62) (MD=0.98 (1.36); P=.474). Excellent agreement was found between IC200 and GAT in both groups (ICC=G1: 0.875 (95% CI; 0.768-0.933; P<.001); G2: 0.924 (95% CI; 0.852-0.961; P<.001), and there was a statistically significant correlation between the IOP difference measured with IC200 and GAT and CCT in G1 (B=0.021; 95% CI; 0.005–0.037; P=.008), but was not statistically significant in G2. Conclusion There was excellent agreement between the IC200 and GAT tonometers, both in healthy subjects and PCG, with a trend to overestimate IOP when measured with IC200. There was no influence by CCT on IOP measurements in patients with PGC (AU)

Aniridic glaucoma: An update. / Actualización en glaucoma anirídico.

Aniridia is a congenital bilateral ocular disorder with dominant autosomal inheritance. More than 50% of patients will develop aniridic glaucoma (AG) during their lives. Open angle glaucoma is more common in aniridia, but a closed angle mechanism has been described in relation with anterior rotation of the rudimentary iris, occluding trabecular meshwork. Diagnosis and follow-up of AG is difficult in relation with the presence of keratopathy, nystagmus and foveal hypoplasia. Central corneal thickness usually measures more than 600 microns, which prevents achieving a reliable value of intraocular pressure. Medical treatment of AG is not different from the rest of glaucoma. It is recommended to use preservative free formulations, and combined therapy is often required. Surgical treatment is needed in many cases. There is no consensus on the first line surgery for AG, but in open angle AG, angle surgery is usually first choice, and glaucoma drainage devices are the next preferred surgical technique. In closed angle AG glaucoma drainage devices are usually the first choice, with trabeculectomy as the second preferred surgical technique.

Comparision of intraocular pressure measured using the new icare 200™ rebound tonometer and the Perkins™ applanation tonometer in healthy subjects and in patients with primary congenital glaucoma. / Comparación entre el tonómetro de rebote IC200 y el tonómetro de aplanación Perkins en sujetos sanos y pacientes con glaucoma congénito.

OBJECTIVE: To compare intraocular pressure (IOP) measurements obtained using the Icare 200™ (IC200) rebound tonometer and the hand-held version of the Goldmann Applanation Tonometer (Perkins™ tonometer, GAT) in patients with primary congenital glaucoma (PCG) and in healthy subjects. MATERIAL AND METHODS: A total of 42 eyes of healthy subjects (G1) and 40 patients with PCG (G2) were analysed. The following clinical data were collected: gender, age, Cup/Disc ratio, central corneal thickness (CCT). IOP was determined in the examination room using the IC200 and GAT tonometers, in the same order. Agreement between both tonometers was determined using the intraclass correlation coefficient (ICC) and Bland-Altman plot. A linear regression analysis was used to establish the IOP was affected by the studied variables. RESULTS: Mean IOP between both tonometers (IC200 minus GAT) was: G1=15.91 (2.57) mmHg vs. 15.06 (2.12) mmHg (mean difference, MD=0.84 (0.50) mmHg; P<.101) and G2=20.10 (6.37) vs.19.12 (5.62) (MD=0.98 (1.36); P=.474). Excellent agreement was found between IC200 and GAT in both groups (ICC=G1: 0.875 (95% CI; 0.768-0.933; P<.001); G2: 0.924 (95% CI; 0.852-0.961; P<.001), and there was a statistically significant correlation between the IOP difference measured with IC200 and GAT and CCT in G1 (B=0.021; 95% CI; 0.005-0.037; P=.008), but was not statistically significant in G2. CONCLUSION: There was excellent agreement between the IC200 and GAT tonometers, both in healthy subjects and PCG, with a trend to overestimate IOP when measured with IC200. There was no influence by CCT on IOP measurements in patients with PGC.

Trabéculo-trabeculectomía como tratamiento quirúrgico inicial del glaucoma congénito primario / Trabeculotomy-trabeculectomy as initial surgical treatment for primary congenital glaucoma

El glaucoma congénito primario constituye la forma más común de glaucoma infantil, el cual exige un diagnóstico precoz y un tratamiento quirúrgico relativamente urgente, ya que puede ocasionar graves secuelas morfológicas y funcionales. Se presenta la realización, por primera ocasión, de la trabéculo-trabeculectomía combinada en la República Democrática y Popular de Argelia. Se ejecutó en un paciente árabe, de un año de edad, a quien se le diagnosticó el glaucoma congénito primario II. Se evaluó el seguimiento posoperatorio con resultados satisfactorios, por lo que se consideró la trabéculo-trabeculectomía como el proceder quirúrgico efectivo y suficientemente seguro, el cual debe ser realizado por un personal calificado para poder admitirse como primera opción en el tratamiento quirúrgico del glaucoma congénito primario(AU)

Primary congenital glaucoma is the most common form of childhood glaucoma. This condition requires early diagnosis and relatively urgent surgical treatment, since it may leave serious morphological and functional sequelae. A description is provided of the first combined trabeculotomy-trabeculectomy performed in the People's Democratic Republic of Algeria. The operation was performed on a male one-year-old Arab patient diagnosed with primary congenital glaucoma II. Post-operative follow-up found satisfactory results, and trabeculotomy-trabeculectomy was thus considered to be an effective, sufficiently safe surgical procedure, which should be performed by qualified personnel to be accepted as the first surgical treatment option for primary congenital glaucoma(AU)

Baerveldt implant versus trabeculectomy as the first filtering surgery for uncontrolled primary congenital glaucoma: a randomized clinical trial / Implante de Baerveldt versus trabeculectomia como primeira cirurgia fistulizante no glaucoma congênito primário não controlado: ensaio clínico randomizado

ABSTRACT Purpose: Our initial goal was to compare the efficacy and safety of a glaucoma drainage device and trabeculectomy for children with primary congenital glaucoma after angular surgery failure. However, we discontinued the study due to the rate of complications and wrote this report to describe the results obtained with the two techniques in this particular group. Methods: This was a parallel, non-masked, controlled trial that included patients aged 0-13 years who had undergone previous trabeculotomy or goniotomy and presented inadequately controlled glaucoma with an intraocular pressure ≥21 mmHg on maximum tolerated medical therapy. We randomized the patients to undergo either placement of a 250-mm2 Baerveldt glaucoma implant or mitomycin-augmented trabeculectomy. The main outcome measure was intraocular pressure control. We calculated complete success (without hypotensive ocular medication) and qualified success (with medication) rates. We defined failure as uncontrolled intraocular pressure, presence of serious complications, abnormal increase in ocular dimensions, or confirmed visual acuity decrease. Results: We studied 13 eyes of 13 children (five in the glaucoma drainage device group; eight in the trabeculectomy group). Both surgical procedures produced a significant intraocular pressure reduction 12 months after intervention from the baseline (tube group, 22.8 ± 5.9 mmHg to 12.20 ± 4.14 mmHg, p=0.0113; trabeculectomy group, 23.7 ± 7.3 mmHg to 15.6 ± 5.9 mmHg, p=0.0297). None of the patients in the tube group and 37.5% of those in the trabeculectomy group achieved complete success in intraocular pressure control after 12 months of follow-up (p=0.928, Chi-square test). Two patients (40%) had serious complications at the time of tube aperture (implant extrusion, retinal detachment). Conclusions: Both the tube and trabeculectomy groups presented similar intraocular pressure controls, but complete success was more frequent in the trabeculectomy group. Non-valved glaucoma drainage devices caused potentially blinding complications during tube opening. Because of the small sample size, we could not draw conclusions as to the safety data of the studied technique.

RESUMO Objetivo: O objetivo inicial era comparar a eficácia e a segurança do implante de drenagem e a trabeculectomia em crianças com glaucoma congênito primário após falência de cirurgia angular. Como o estudo foi descontinuado devido à taxa de complicações, o objetivo deste artigo foi descrever os resultados das duas técnicas neste grupo específico. Métodos: Ensaio clínico randomizado, não mascarado, incluindo pacientes com idade de 0 a 13 anos previamente submetidos à goniotomia ou trabeculotomia. Os pacientes, que apresentavam glaucoma não controlado com pressão intraocular ≥21 mmHg em terapia medicamentosa máxima, foram randomizados para o implante de drenagem de Baerveldt 250 mm2 (Grupo Tubo) ou trabeculectomia com mitomicina (grupo TREC). O principal desfecho avaliado foi o controle da pressão intraocular. Sucesso completo (sem medicação ocular hipotensora) e sucesso qualificado (com medicação) foram descritos. A falência foi baseada na pressão intraocular não controlada, presença de complicações sérias, aumento anormal das dimensões oculares e diminuição confirmada da acuidade visual. Resultados: Treze olhos de 13 crianças foram estudados (cinco no grupo Tubo e oito no grupo TREC). Ambos os procedimentos reduziram a pressão intraocular em relação às medidas iniciais após 12 meses da intervenção (grupo Tubo 22.8 ± 5.9 mmHg para 12.20 ± 4.14 mmHg, p=0.0113; grupo TREC, 23.7 ± 7.3 mmHg para 15.6 ± 5.9 mmHg, p=0.0297). Nenhum paciente no grupo Tubo e 37.5% do grupo TREC alcançaram o sucesso completo após 12 meses de acompanhamento (p=0.928, teste qui-quadrado). Dois pacientes (40%) apresentaram sérias complicações no momento da abertura do tubo (extrusão do implante e descolamento de retina). Conclusão: Os dois grupos estudados apresentaram resultados semelhantes quanto ao controle da pressão intraocular, mas o sucesso completo foi mais frequente no grupo da trabeculectomia. Implantes de drenagem não valvulados podem cursar com potenciais complicações visuais no momento da abertura do tubo. Devido ao pequeno tamanho da amostra, não foi possível determinar quaisquer dados de segurança conclusivos em relação à técnica estudada.

Primary megalocornea: Case report. / Megalocórnea esencial: relato de caso.

The neonate has a horizontal diameter of the cornea, usually up to 10mm with growth up to 2mm in the first 2 years of life. We report a case of megalocornea, a rare, recessive, X-linked disorder in a 3-month-old child, seeking to review what the medical literature brings information about the condition, as well as diagnostic and follow-up parameters, of its main differential diagnoses.

Facomatosis tipo Cesioflammea: Reporte de caso de neonato con Manchas Mongólicas y Vino oporto. Una presentación simple de una condición dermatológica compleja.

La facomatosis pigmentovascular es un conjunto de signos y síntomas caracterizado por la coexistencia de malformaciones vasculares capilares con una lesión pigmentaria cutánea congénita (nevo epidérmico, nevo spilus o melanosis dérmica) asociada o no, a manifestaciones sistémicas. Existen varios grupos de facomatosis pigmentovasculares según el tipo de nevo asociado a la lesión capilar vascular. Puede existir solo la afectación cutánea o tener manifestaciones sistémicas, entre ellas, traumatológicas, neurológicas, oftalmológicas, inmunológicas, renales, vasculares, linfáticas, entre otras. Exponemos el caso de un neonato masculino, quien presenta manchas vino oporto facial bilateral, cuello y tórax anterior y posterior, junto a melanocitosis dérmicas en espalda y ambos miembros inferiores, asociado a glaucoma congénito del ojo derecho. Debido a las manifestaciones clínicas cutáneas y sistémicas, se diagnostica facomatosis pigmentovascular, realizándose evaluación multidisciplinaria por radiología, dermatología, oftalmología, genética y neurología, para descartar afectación extracutánea. Los estudios complementarios son de vital importancia para detectar alteraciones extracutáneas, como se presentaron en el paciente.

Phakomatosis pigmentovascularis is set of signs and symptoms characterized by the association of a vascular nevus with a congenital pigmented lesion (epidermal nevus, nevus spilus, and dermal melanocytosis) associated or not to systemic malformations. There are different types of phakomatosis pigmentovascularis according to the pigmentary nevus associated with the vascular malformation. There may be only the cutaneous condition or have systemic manifestations such as, trauma, neurological, ophthalmological, inmunological, renal, vascular, lymphatic, among others disorders. We present the case of a male newborn who had a port wine stain located bilaterally on the face, anterior and posterior thorax and neck, together with dermal melanocitosis in the back and lower extremities, associated with congenital glaucoma of the right eye. Due the clinical manifestations, the diagnosis of phakomatosis pigmentovascularis was made and multidisciplinary evaluation is requested by radiology, dermatology, ophthalmology, genetics and neurology to rule out extracutaneous involvement. Complementary studies are essential to detect extracutaneous alterations as presented in the patient.

Glaucoma congénito primario: ¿cuándo es preciso sospechar? / Primary congenital glaucoma: when should it be suspected?

RESUMEN Se realiza una actualización sobre el diagnóstico y tratamiento del glaucoma infantil primario, considerando la importancia que tiene un diagnóstico precoz y un tratamiento oportuno para obtener los mejores resultados posibles. Actualmente, a pesar del surgimiento de nuevos fármacos y de técnicas quirúrgicas para tratar esta afección, aún existen discrepancias sobre cómo debemos realizar el diagnóstico positivo; qué tratamiento o técnica quirúrgica emplear; cuándo y cuál sería la mejor. Cuando se trata de glaucoma pediátrico (primario o secundario), presente en etapas tan tempranas de la vida -incluso al nacimiento, donde la cirugía está indicada lo más precozmente posible, con la disyuntiva de cuáles medicamentos podemos emplear o no, por desconocimiento de qué efectos indeseables pudieran presentarse en estas edades y además, teniendo en cuenta que estos niños serán nuestros pacientes para toda la vida- es necesario conocer y repasar una vez más este tema. Se recomienda instruir a pediatras y oftalmólogos generales para lograr la remisión adecuada y precoz del niño a un centro especializado para su tratamiento quirúrgico, y mejorar así su pronóstico visual(AU)

ABSTRACT Updated information is provided about the diagnosis and treatment of primary childhood glaucoma, given the importance of early diagnosis and timely treatment to obtain the best possible results. Despite the emergence of new drugs and surgical techniques to treat this disorder, there is still controversy about how we should perform the positive diagnosis, what treatment or surgical technique should be used and when, and which would be the best. When childhood glaucoma (whether primary or secondary) presents at early stages of life - even at birth -, in which case surgery should be indicated as soon as possible, we are faced with the dilemma of what drugs we may or may not use, due to lack of knowledge about undesirable effects which could appear at these ages, and bearing in mind that these children will be our patients for a lifetime. It is thus necessary to be informed about this topic and go over it once again. It is recommended to instruct pediatricians and ophthalmologists to ensure timely, appropriate referral of the child patient to a specialized center where they will receive surgical treatment, thus improving their visual prognosis(AU)

The correlation between the thickness of the inner macular layers and the mean deviation of the visual field in children with primary congenital glaucoma. / Correlación entre el grosor de las capas internas de la retina y el defecto medio del campo visual en pacientes con glaucoma congénito primario.

PURPOSE: To analyse the association between the thickness of the circumpapillary retinal nerve fibre layer (cpRNFL) and the thickness of the inner macular layers with the mean deviation of the visual field (MD) in children with primary congenital glaucoma (PCG). MATERIAL AND METHODS: A total of 41 children with PGC were included in the study. They all had a complete ophthalmological examination, including visual acuity, intraocular pressure, funduscopy, Octopus™ visual field, as well as circumpapillar and macular spectral domain optical coherence tomography (SD-OCT). SD-OCT with automated segmentation was used to measure the thicknesses and volumes of the macular retinal nerve fibre layer (mRNFL), ganglion cell layer (GCL), and inner plexiform layer. RESULTS: The mean age was 11.2±3.86 years, and the mean MD was 8.85±6.76dB. The visual field was classified as normal in 46% of the patients, and 20% of the patients had a concentrical restriction of the visual field. A positive correlation was found between between the cup-to-disc ratio and the MD, r=0.51 (P=.004). The correlation between the MD and the cpRNFL was r=-0.63 (P<.001), and r=-0.69 (P<.001) with the GCL. CONCLUSIONS: Inner macular layers thickness and cpRNFL thickness show a good correlation with the mean deviation of the visual field in children with primary congenital glaucoma.

Creation of a childhood glaucoma registry database / Criação de um banco de dados para glaucoma infantil

ABSTRACT Purpose: The aim is creation of a database to register patients diagnosed with childhood glaucoma. Information collection and storage will utilize a digital platform that permits multiple centers to register patients. This registry will be an important tool to evaluate and group patient profiles, thus allowing identification of risk factors, estimating prevalence, and recruiting participants in future studies. Methods: An online form was designed at the Department of Ophthalmology and Visual Sciences of the Federal University of São Paulo to create a registry of patients diagnosed with congenital glaucoma. In addition, a pilot study was conducted in the same institution to validate the Google Forms platform that comprises the registry questionnaire. Results: Data can be entered online and stored in the cloud. The database allows the acquisition and storage of epidemiological and clinical data, detection of patterns, and evaluation of the current treatment of children with childhood glaucoma. The data from multiple centers can be combined as collection in online and storage is in the cloud. The form is comprehensive and includes social data, gestational history, family history, clinical data on entering the center, and the treatment received. Conclusions: Google Forms is a useful tool for collecting and analyzing statistical data, facilitating the process of registering patients, and analyzing information. Using the online questionnaire, it will be possible to create a multicenter online database that allows identification of risk factors and evaluation of the outcome of interventions and treatment.

RESUMO Objetivo: Este estudo visa a criação de um programa para a formação de uma base de dados dos pacientes com diagnóstico de glaucoma pediátrico do Departamento de oftalmologia e ciências visuais da Universidade Federal de São Paulo. O armazenamento das informações se dará por meio de uma plataforma digital, possibilitando que outros centros também possam cadastrar pacientes. Este registro será uma ferramenta importante para avaliar o perfil dos pacientes e agrupá-los, permitindo identificar fatores de risco, estimar prevalência e recrutar indivíduos para futuras pesquisas. Métodos: Um formulário online foi projetado para criar um registro de pacientes com diagnóstico de glaucoma congênito do Departamento de Oftalmologia e Ciências Visuais da Universidade Federal de São Paulo. Além disso, um estudo piloto foi conduzido para validar a base de dados criada. Resultados: Foi criado um questionário usando a plataforma Google Forms, de preenchimento online e armazenamento na nuvem, para registro dos pacientes com diagnóstico de glaucoma infantil do Departamento de Oftalmologia e Ciências Visuais da Universidade Federal de São Paulo. Tal registro permite armazenar dados epidemiológicos e clínicos, detectar padrões e avaliar o tratamento atual de crianças com glaucoma da infância, com a possibilidade de intercâmbio e uso de informações com outros centros, já que a coleta e o armazenamento dos dados se dá de forma online. O formulário é extenso e engloba dados sociais, histórico gestacional, antecedentes familiares, dados clínicos de entrada no nosso centro e o tratamento recebido. Conclusão: Este estudo demonstrou que o Google Forms é uma ferramenta útil para coleta e análise de dados estatísticos, facilitando o processo de registro de pa­cientes e análise de informações. A criação do questionário online e a consequente análise das informações permitirá o maior conhecimento do perfil dos pacientes acompanhados em nosso centro e, com a centralização dos dados num ambiente único - nuvem digital - tornar-se-á possível criar um banco de dados online multicêntrico que permitirá a identificação de fatores de risco e avaliar o resultado das intervenções e tratamentos, assim como recrutar as crianças para pesquisas futuras.

Macular hemorrhage after roller coaster riding in a single-eyed patient with congenital glaucoma / Hemorragia macular após passeio em montanha russa em paciente monocular com glaucoma congênito

ABSTRACT A 21-year-old female presented with a 4-day history of decreased vision in her only functional eye (right eye, OD). She had a history of multiple ocular surgeries in both eyes because of congenital glaucoma and had lost light perception in her left eye several years prior. Ophthalmological examination revealed 0.15 Snellen visual acuity, and fundoscopy revealed nearly total cupping and pallor of the optic disc and multiple retinal hemorrhagic foci in the macula in OD. Lesions spontaneously resolved over a few months. Gravitational forces during a roller coaster ride may have caused this macular hemorrhage.

RESUMO Uma paciente de 21 anos de idade se apresentou com perda de visão há quatro dias em seu único olho com visão útil. Ela tinha uma história de cirurgias oculares múltiplas nos dois olhos devido a um glaucoma congênito e perda de percepção luminosa em olho esquerdo há muitos anos. O exame oftalmológico revelou acuidade visual de Snellen de 0,15 e na fundoscopia foi observada escavação do nervo óptico quase total e palidez de papila, assim como focos hemorrágicos múltiplos na região macular. As lesões se resolveram espontaneamente em alguns meses. Acreditamos que essas hemorragias maculares tenham sido causadas pelas forças gravitacionais geradas durante o passeio na montanha russa.

Panophthalmitis with orbital cellulitis following glaucoma drainage implant surgery in a pediatric patient / Panoftalmite com celulite orbitária após implante de drenagem em glaucoma congênito

ABSTRACT Here we report a case of childhood glaucoma refractory to angle and trabeculectomy surgery. The patient was treated with an Ahmed™ drainage implant that was subsequently complicated by rapid-onset panophthalmitis and orbital cellulitis. Intravenous and intravitreal antibiotic therapy was initiated and the drainage tube was removed. The infectious process resolved within 3 weeks; however, phthisis bulbi developed subsequently.

RESUMO Relato de um caso de uma criança portadora de glaucoma congênito primário, refratário a cirurgias angulares e trabeculectomias prévias, submetido à implante de drenagem do tipo Ahmed®. O paciente evoluiu com panoftalmite e celulite orbitária de aparecimento súbito, sendo submetido à remoção do tubo e antibioticoterapia endovenosa e intravítrea. O processo infeccioso foi resolvido em três semanas, porém o olho evoluiu para phthisis bulbi.

Perfil epidemiológico dos pacientes com glaucoma congênito atendidos no Hospital Regional de São José / Epidemiological profile of patients with congenital glaucoma treated in the Hospital Regional de São José

Objetivo Delinear um perfil epidemiológico de pacientes com glaucoma congênito atendidos no serviço de Oftalmologia do Hospital Regional de São José, bem como a evolução destes pacientes com os tratamentos empregados. Métodos Foi realizado um estudo longitudinal, retrospectivo, descritivo que avaliou 32 pacientes com glaucoma congênito atendidos em ambulatório, desde a primeira consulta quando ingressaram no serviço e consultaram pelo menos duas vezes no período de 1º de março de 2009 até 1º de fevereiro de 2011. Resultados Houve predominância do sexo feminino (59,37%). Com relação à lateralidade, ambos os olhos foram acometidos em 91% dos casos. A maioria dos pacientes (78,12%) apresentava glaucoma congênito primário. Oitenta e cinco cirurgias foram realizadas para o glaucoma congênito, destes 63,52% foram submetidos à trabeculotomia. A pressão intraocular aferida na primeira e na última consulta diminuiu em 85,93% dos 64 olhos, já em relação à pressão intraocular média houve uma diminuição de 82,81% em relação ao valor na primeira consulta. Ao realizar a comparação do diâmetro corneano horizontal da primeira consulta em relação à última, bem como da primeira consulta em relação à média das aferições de todas as consultas, em 25,42% ocorreu aumento. Conclusão Nos pacientes com glaucoma congênito, houve predomínio do acometimento bilateral e do sexo feminino. A maioria dos pacientes apresentou diminuição da pressão intraocular na última consulta em relação à primeira e em poucos pacientes houve um aumento do diâmetro corneano horizontal...

Objective To outline an epidemiological profile of patients with congenital glaucoma treated in the ophthalmology service, as well as the evolution of these patients with the treatment adopted. Methods A longitudinal, retrospective, descriptive study was undertaken in order to evaluate 32 patients with congenital glaucoma treated in the clinic, from the first consultation in which they entered the service and who attended the clinic at least two times in the period from march 1st, 2009 to february 1st, 2011. Results There was a predominance of female patients (59.37%). In relation to the lateralization, both eyes were affected in 91% of the cases. Most patients (78.12%) presented primary congenital glaucoma. Eighty-five surgeries were performed for congenital glaucoma, 63.52% of them were submitted to trabeculotomy. The intraocular pressure measured in the first and in the last consultation diminished in 85.93% of the 64 eyes, and in relation to the mean intraocular pressure there was a decrease of 82.81% in relation to the value of the first consultation. When making the comparison of the horizontal corneal diameter of the first consultation in relation to the last one, as well as of the first consultation in relation to the average of the measurements of all consultations, in 25.42% there was an increase. Conclusion In the patients with congenital glaucoma, there was a predominance of bilateral involvement and of female patients. Most patients presented a decrease of intraocular pressure in the last consultation in relation to the first one, and in a few patients there was an increase of horizontal corneal diameter...

El uso de válvulas de Ahmed en el tratamiento del glaucoma refractario en niños / Use of Ahmed valves in the treatment of refractory glaucoma in children

Objetivo: evaluar la efectividad del implante de la válvula de Ahmed (VA) en niños desde los 2 años de vida hasta los 16 años de edad, que presentaron glaucoma refractario al tratamiento quirúrgico convencional, atendidos en el Hospital Garrahan durante el periodo comprendido entre el 1 de enero de 2006 hasta el 30 de Junio de 2014. Resultados: Se incluyeron 16 ojos de 13 pacientes con un total de 17 VA colocadas. La presión intraocular (PIO) promedio de las tres últimas tomas antes de la colocación de la VA fue de 25,38 mm Hg con un rango de 19,33 mm Hg a 39,33 mm Hg. Todos estos pacientes se encontraban con tratamiento farmacológico tópico y nueve pacientes (69,2%) estaban medicados con acetazolamida vía oral. Trece ojos de 16 incluidos en este trabajo (81,25%) recibieron más de una cirugía filtrante previa. Los 3 ojos (18,75%) no operados previamente eran glaucomas secundarios a uveítis; se decidió colocar el dispositivo de drenaje de primera elección. Un solo ojo (6,25%) incluido en la presente serie requirió la colocación de una segunda VA. La PIO promedio al año de cirugía de la válvula fue de 13,36 mm Hg. y en los pacientes a los que se logró seguir por cinco años la PIO se mantuvo por debajo de 20 mm Hg. Ninguno de los pacientes a los que se les colocó válvula necesitó inhibidores de la anhidrasa carbónica por vía oral. Un 64,72% de los ojos a los que se les colocó VA no necesitó medicación para regular la PIO. Del 35,28% de los 17 ojos posquirúrgicos que debieron usar medicación tópica encontramos que un ojo (5,88%) requirió una droga, cuatro ojos (23,52%) demandaron el uso de dos drogas y un solo ojo (5,88%) necesitó tres drogas tópicas para mantener su PIO menor a 20 mm Hg. Las complicaciones posquirúrgicas tardías de la colocación de VA fueron en cuatro casos (23,5%) quistes conjuntivales perivalvulares, en uno (5,88%) hubo exposición del tubo que requirió su reposicionamiento, un caso (5,88%) presentó alteraciones de la motilidad ocular y otro (5,88%) desprendimiento de retina. Conclusión: La colocación de la VA es efectiva para el tratamiento del glaucoma infantil refractario porque se consiguió una disminución del 100% de la presión intraocular, con y sin medicación coadyuvante en los diversos controles de nuestro grupo de pacientes estudiados. El 64,7% de los pacientes no necesitó medicación tópica posterior a la cirugía y ninguno de ellos necesitó tratamiento con medicación sistémica para regular la PIO (AU)

Objective: To assess the effectiveness of Ahmed valve (AV) implantation in children between 2 and 16 years of age with glaucoma refractory to conventional surgical treatment seen at the Garrahan Hospital from January 1, 2006 to June 30, 2014. Results: 16 eyes of 13 patients were included of a total of 17 AV implanted. Mean intraocular pressure (IOP) of the last three taken before AV placement was 25.38 mm Hg with a range of 19.33 mm Hg to 39.33 mm Hg. All the patients were receiving topical drug treatment and nine (69.2%) received oral acetazolamide. Thirteen of 16 eyes included in the study (81.25%) underwent more than one previous filtration surgery. The three eyes (18.75%) that did not undergo surgery had glaucoma secondary to uveitis; a first-choice drainage device was placed. Only one eye (6.25%) included in this series required placement of a second AV. Mean IOP one year after implantation of the valve was 13.36 mm Hg and in those patients followed-up for five years IOP remained below 20 mm Hg. None of patients in whom an AV was implanted needed oral carbonic anhydrase inhibitors. Overall, 64.72% of the eyes with an AV implant did not need medication to regulate IOP. Postsurgically, of 35.28% of 17 eyes that needed topical medication, we found that one eye (5,88%) required one drug, four eyes (23.52%) needed two drugs, and only one eye (5.88%) needed three drugs to maintain IOP below 20 mm Hg. Late surgical complications of AV placement were perivalvular conjunctival cysts in four cases (23.5%), exposition of the tube that needed repositioning in one case (5.88%), alterations of ocular movements in one (5.88%), and retinal detachment in another (5.88%). Conclusion: AV implantation is effective in the treatment of refractory childhood glaucoma as it decreases IOP by 100% with or without coadjuvant medication in the follow-up of our patients. Of all patients, 64.7% did not need topical medication after surgery and none of the patients needed systemic medication to regulate IOP (AU)