TEK gene-related primary congenital glaucoma: Phenotypic features and mutational spectrum in a Mexican cohort of 10 unrelated families.

Primary congenital glaucoma (PCG) is one of the leading causes of visual damage and blindness, severely affecting the quality of life of affected children. It is characterized by cupping of the optic disc and loss of ganglion cells due to elevated intraocular pressure. While most PCG patients exhibit epiphora, photophobia, and buphthalmos with corneal opacity, variability in phenotypic manifestations is not uncommon. Prompt diagnosis and treatment of PCG affected individuals becomes relevant to preserve visual function throughout their lives. Most PCG cases are sporadic or autosomal recessive; however, an incompletely dominant autosomal dominant form arising from mutations in the TEK gene has recently been demonstrated. Here, we describe the clinical and mutational features of a cohort of Mexican patients with TEK-related PCG. Our results support the involvement of the TEK gene as an important cause of the disease in our ethnic group and expand the mutational spectrum causing PCG by reporting 10 novel disease-causing variants.

Glaucoma drainage devices in children: an updated review / Dispositivos de drenagem para glaucoma em crianças: uma revisão atualizada

ABSTRACT Implantation of glaucoma drainage devices is a valuable therapeutic option, particularly in children with glaucoma refractory to primary surgical treatment. Glaucoma drainage devices are typically used when conjunctival scarring hampers filtration surgery or prior angle procedures are not effective in controlling intraocular pressure. Despite known complications, the use of glaucoma drainage devices in children has increased in recent years, even as the primary surgical option. In this review, we evaluate the results of recent studies involving the implantation of glaucoma drainage devices in children, discussing new advances, and comparing the success rates and complications of different devices.

RESUMO O implante de dispositivos de drenagem para glaucoma (DDGs) é uma opção terapêutica valiosa, principalmente em crianças com glaucoma refratário ao tratamento cirúrgico primário. Os dispositivos de drenagem para glaucoma têm sido utilizados principalmente quando a cicatrização conjuntival dificulta a cirurgia fistulizante ou procedimentos angulares prévios não foram eficazes no controle da pressão intraocular. Apesar das complicações conhecidas, o uso de dispositivos de drenagem para glaucoma em crianças tem aumentado nos últimos anos, inclusive como opção cirúrgica primária. Nesta revisão, atualizamos os resultados de estudos recentes envolvendo o implante de dispositivos de drenagem para glaucoma em crianças, discutindo novos avanços e comparando diferentes dispositivos, taxas de sucesso e complicações.

Outcomes of early versus delayed trabeculotomy for primary congenital glaucoma.

PURPOSE: In patients with primary congenital glaucoma (PCG), elevated intraocular pressure (IOP) causes abnormal eye growth. This study compared the outcomes of children with PCG who underwent ab externo trabeculotomy (TROC) at age ≤ 6 months (early TROC) and of those who underwent TROC at age > 6 months (delayed TROC). METHODS: Intraocular pressure, horizontal corneal diameter (HCD), central corneal thickness (CCT) and axial length (AL) were compared before TROC and at 1-, 3-, 6- and 12-month follow-up visits between the groups of children who underwent TROC until or after 6 months of age. The ALs of these groups were also compared with the ALs of healthy age-matched eyes examined under the same conditions. RESULTS: Trabeculotomy was performed in 43 children: 18 (33 eyes) aged 6 months (group 1) and 25 (37 eyes) aged >6 months (group 2); the mean ages were 86.56 ± 53.64 and 504.48 ± 448.14 days, respectively. The mean pre- and 12-month postoperative IOP values were 15.97 ± 4.78/16.62 ± 4.85 and 9.77 ± 2.88/10.93 ± 4.83 mmHg, respectively. Delayed TROC was associated with abnormal AL in 31 (88.6%) out of 37 eyes, while after early TROC, only 13 (41.9%) out of 33 eyes had abnormal AL (chi-square, 8.00; p = 0.03). In multivariable analysis, each 1-mmHg increase in preoperative IOP was associated with a 0.25-mmHg increase at 12 months (p = 0.04). On average, the mean IOP of the delayed TROC group was higher than that of the early TROC group by 3.72 mmHg at postoperative month 12 (95% CI = 0.44-6.99; p = 0.02). CONCLUSION: Compared with delayed TROC, early TROC is associated with reduced IOP and substantially reduced incidence of abnormal AL at postoperative month 12.

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)

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.

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)

Corrigendum: Optic neuropathy and congenital glaucoma associated with probable Zika virus infection in Venezuelan patients.

[This corrects the article DOI: 10.1099/jmmcr.0.005145.].

Optic neuropathy and congenital glaucoma associated with probable Zika virus infection in Venezuelan patients.

Introduction: Although the current Zika virus (ZIKV) epidemic is a major public health concern, most reports have focused on congenital ZIKV syndrome, its most devastating manifestation. Severe ocular complications associated with ZIKV infections and possible pathogenetic factors are rarely described. Here, we describe three Venezuelan patients who developed severe ocular manifestations following ZIKV infections. We also analyse their serological response to ZIKV and dengue virus (DENV). Case presentation: One adult with bilateral optic neuritis, a child of 4 years of age with retrobulbar neuritis [corrected]. and a newborn with bilateral congenital glaucoma had a recent history of an acute exanthematous infection consistent with ZIKV infection. The results of ELISA tests indicated that all patients were seropositive for ZIKV and four DENV serotypes. Conclusion: Patients with ZIKV infection can develop severe ocular complications. Anti-DENV antibodies from previous infections could play a role in the pathogenesis of these complications. Well-designed epidemiological studies are urgently needed to measure the risk of ZIKV ocular complications and confirm whether they are associated with the presence of anti-flaviviral antibodies.

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)

Actualización en el tratamiento médico del glaucoma pediátrico / Updating on the medical treatment of pediatric glaucoma

En la actualidad no existen suficientes estudios para valorar la seguridad y eficacia de los medicamentos hipotensivos oculares en el glaucoma pediátrico. El uso de medicamentos para reducir la presión intraocular en niños, se considera desacertado principalmente en glaucomas congénitos primarios y en los asociados a anomalías oculares. En otros tipos de glaucomas, como los juveniles no asociados a goniodisgenesias y secundarios, se suele comenzar con tratamiento medicamentoso, aunque por los efectos adversos de este y el no control adecuado de la tensión ocular, un porcentaje importante necesita tratamiento quirúrgico. Se realiza una revisión bibliográfica actualizada con el objetivo de describir el mecanismo de acción, dosificación y efectos indeseables de los medicamentos hipotensores oculares en niños con glaucoma

Nowadays, there are few studies that assess safety and effectiveness of hypotensive ocular medications for pediatric glaucomas. Use of drugs to reduce the intraocular pressure (IOP) in children is considered ineffective mainly in congenital primary glaucoma and in associated ocular anomalies. In other types of glaucomas such as juvenile glaucoma unrelated to goniodysgenesis and secondary ones, drug treatment is the initial therapy, but because of adverse reactions and inadequate control, a significant percentage of cases require surgical treatment. An updated literature review was made, with the objective of describing the mechanism of action, dosage and undesirable effects of hypotensive ocular drugs in glaucomatous children

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

Se describen dos casos clínicos con síndrome de Axenfeld-Rieger y glaucoma bilateral. El primer caso representa una paciente femenina con corectopia, embriotoxon posterior, atrofia del estroma anterior del iris, adherencias iridocorneales y glaucoma bilateral; por lo que se decide realizar trabéculo-trabeculectomía en ambos ojos y mantuvo cifras de tensión intraocular dentro de límites normales al año de evolución. El segundo caso se trata de un paciente masculino con escleras azules, microcórnea, embriotoxon posterior, aniridia parcial, atrofia sectorial del iris y glaucoma bilateral; por lo que se realizó trabéculo-trabeculectomía de ambos ojos con descompensación posterior del ojo izquierdo. Se inició el tratamiento médico sin buenos resultados y se reintervino el ojo izquierdo, se descompensó dos meses después de la cirugía. Actualmente se encuentra compensado con tratamiento médico

Two clinical cases of Syndrome of Axenfeld–Riegel and bilateral glaucoma were described. The first case was a female patient with corectopia, posterior embriotoxon, atrophy of the anterior stroma of the iris, iridocorneal adherences and bilateral glaucoma. To treat her, it was decided to perform trabeculo-traceculectomy in both eyes and as a result, the patient kept intraocular pressure values within the normal limits after one year. The second case was male patient who suffered with blue sclera, microcornea, posterior embrotoxon, partial aniridia, sector atrophies of the iris and bilateral glaucoma. Trabeculo-trabeculectomy of both eyes with posterior decompensation of the left eye was applied. The medical treatment did not yield good results, His left eye was then re-operated and two month afterwards, decompensation occurred again. At present, he is compensated with a new medical treatment

Long-term surgical outcomes of primary congenital glaucoma in China.

OBJECTIVE: To evaluate the long-term outcomes of three surgical procedures for the treatment of primary congenital glaucoma (PCG). INTRODUCTION: PCG is one of the main causes of blindness in children. There is a paucity of contemporary data on PCG in China. METHODS: A retrospective study of 48 patients (81 eyes) with PCG who underwent primary trabeculectomy, trabeculotomy, or combined trabeculotomy and trabeculectomy (CTT). RESULTS: All patients were less than 4 years (yrs) of age, with a mean age of 2.08 +/- 1.23 yrs. The mean duration of follow-up was 5.49 +/- 3.09 yrs. The difference in success rates among the three surgical procedures at 1, 3, 6 and 9 yrs was not statistically significant (p = 0.492). However, in patients with over 4 yrs of follow-up, Kaplan-Meier survival analysis revealed that the success rates of trabeculectomy and CTT declined more slowly than that of trabeculotomy. Among the patients, 66.22% acquired good vision (VA > 0.4), 17.57% acquired fair vision (VA = 0.1 - 0.3), and 16.22% acquired poor vision (VA < 0.1). The patients with good vision were mostly in the successful surgery group. Myopia was more prevalent postoperatively (p = 0.009). Reductions in the cup-disc ratio and corneal diameter were only seen in the successful surgery group (p = 0.000). In addition, the successful surgery group contained more patients that complied with a regular follow-up routine (p = 0.002). DISCUSSION: Our cases were all primary surgeries. Primary trabeculectomy was performed in many cases because no treatment was sought until an advanced stage of disease had been reached. CONCLUSIONS: In contrast to most reports, in the present study, trabeculectomy and CTT achieved higher long-term success rates than trabeculotomy. The patients with successful surgical results had better vision. Compliance with a routine of regular follow-up may increase the chances of a successful surgical outcome.

Long-term surgical outcomes of primary congenital glaucoma in China

OBJECTIVE: To evaluate the long-term outcomes of three surgical procedures for the treatment of primary congenital glaucoma (PCG). INTRODUCTION: PCG is one of the main causes of blindness in children. There is a paucity of contemporary data on PCG in China. METHODS: A retrospective study of 48 patients (81 eyes) with PCG who underwent primary trabeculectomy, trabeculotomy, or combined trabeculotomy and trabeculectomy (CTT). RESULTS: All patients were less than 4 years (yrs) of age, with a mean age of 2.08 ¡À 1.23 yrs. The mean duration of follow-up was 5.49 ¡À 3.09 yrs. The difference in success rates among the three surgical procedures at 1, 3, 6 and 9 yrs was not statistically significant (p = 0.492). However, in patients with over 4 yrs of follow-up, Kaplan-Meier survival analysis revealed that the success rates of trabeculectomy and CTT declined more slowly than that of trabeculotomy. Among the patients, 66.22% acquired good vision (VA ¡Ý 0.4), 17.57% acquired fair vision (VA = 0.1 - 0.3), and 16.22% acquired poor vision (VA < 0.1). The patients with good vision were mostly in the successful surgery group. Myopia was more prevalent postoperatively (p = 0.009). Reductions in the cup-disc ratio and corneal diameter were only seen in the successful surgery group (p = 0.000). In addition, the successful surgery group contained more patients that complied with a regular follow-up routine (p = 0.002). DISCUSSION: Our cases were all primary surgeries. Primary trabeculectomy was performed in many cases because no treatment was sought until an advanced stage of disease had been reached. CONCLUSIONS: In contrast to most reports, in the present study, trabeculectomy and CTT achieved higher long-term success rates than trabeculotomy. The patients with successful surgical results had better vision. Compliance with a routine of regular follow-up may increase the chances of a successful surgical outcome.