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...

Surgical Results of Trabeculectomy and Ahmed Valve Implantation Following a Previous Failed Trabeculectomy in Primary Congenital Glaucoma Patients

PURPOSE: To compare the surgical results of trabeculectomy and Ahmed glaucoma valve implantation after a previous failed trabeculectomy. METHODS: A retrospective comparative case series review was performed on 31 eye surgeries in 20 patients with primary congenital glaucoma who underwent trabeculectomy or Ahmed glaucoma valve implantation after a previous failed trabeculectomy with mitomycin C. RESULTS: The preoperative mean intraocular pressure was 25.5 mmHg in the trabeculectomy group and 26.9 mmHg in the Ahmed glaucoma valve implantation group (p = 0.73). The 48-month postoperative mean intraocular pressure was 19.6 mmHg in the trabeculectomy group and 20.2 mmHg in the Ahmed glaucoma valve implantation group (p = 0.95). The 12-month trabeculectomy success rate was 69%, compared with 64% for Ahmed glaucoma valve implantation, and the 48-month success rates were 42% and 36% for trabeculectomy and valve implantation, respectively. The success rates following the entire follow-up period were not significantly different between the two groups (p > 0.05 by log rank test). Postoperative complications occurred in 25% of the trabeculectomy-operated eyes and 9% of the Ahmed-implanted eyes (p = 0.38). CONCLUSIONS: There was no significant difference in surgical outcome between the trabeculectomy and Ahmed glaucoma valve implantation groups, neither of which had favorable results. However, the trabeculectomy group demonstrated a higher prevalence of adverse complications such as post-operative endophthalmitis.

Correlations between different tonometries and ocular biometric parameters in patients with primary congenital glaucoma / Correlações entre diferentes tonometrias e parâmetros biométricos oculares em pacientes com glaucoma congênito primário

PURPOSE: To identify the correlation between the difference of intraocular pressure measurements (IOP) obtained using the Goldmann applanation tonometer (GAT) and three others tonometers (Handheld applanation tonometer - HAT, Dynamic contour tonometer - DCT and Tono-Pen®) with biometric characteristics (corneal diameter, pachymetry, keratometry and axial length) in patients with congenital glaucoma. METHODS: A cross-sectional study was performed on 46 eyes from 46 patients with congenital glaucoma. IOP measurements were obtained in all patients using GAT, HAT, DCT and Tono-Pen®. Keratometry, pachymetry, biometry and corneal diameter measurements were performed after the IOP measurement. The order of the tonometries was randomized. The correlations between the differences of IOP values of GAT and the other tonometers (Delta-IOP), and the different biometric parameters were studied. RESULTS: Tono-Pen® Delta IOP revealed moderate positive correlation to keratometry (r=0.41, p=0.004). The other Delta-IOPs showed no correlation with any of the biometric characteristics evaluated. CONCLUSIONS: IOP differences between GAT (gold standard) and GAT, HAT, DCT or Tono-Pen tonometers seem not to correlate with majority of ocular biometric characteristics. The only exception was the keratometry, which correlated in a positive and moderate way with Tono-Pen® Delta-IOP. This result suggests that the differences of IOP values of Tono-Pen® and GAT increase with the steepness of the cornea.

OBJETIVOS: Identificar correlações entre as diferenças de medições de pressão intraocular (IOP) obtidas usando o tonômetro de aplanação de Goldmann (GAT) e três outros tonômetros (Tonômetro portátil de aplanação - TPA, Tonômetro de contorno dinâmico - TCD e Tono-Pen®) com características biométricas (diâmetro corneano, paquimetria, ceratometria e comprimento axial) em pacientes com glaucoma congênito. MÉTODOS: Estudo transversal prospectivo foi realizado em 46 olhos de 46 pacientes com glaucoma congênito. As medidas de pressão intraocular foram obtidas em todos os pacientes utilizando TAG, TPA, TCD e Tono-Pen®. Ceratometria, paquimetria, biometria e diâmetro corneano foram realizadas após mensuração da pressão intraocular. A ordem da utilização tonômetros foi aleatória. Correlações entre as diferenças de valores de PIO entre cada um dos três tonômetros (PIOs Delta) e o tonômetro de Goldmann e as características biométricas foram analisadas. RESULTADOS: PIO Delta do Tono-Pen®revelou correlação positiva moderada com ceratometria (r=0,41, p=0,004). As outras PIOs Delta não se correlacionaram significativamente com nenhuma das características biométricas. CONCLUSÕES: As diferenças entre as PIOs obtidas pelo TAG (padrão ouro) e TPA, TCD e Tono-Pen®parece não se correlacionar com a maioria das características biométricas. A única exceção foi a ceratometria, a qual se correlacionou de forma positiva e moderada com a PIO Delta do Tono-Pen®. Estes resultados indicam que o aumento da diferença entre a PIO obtida com TAG e Tono-Pen®aumenta com o encurvamento da curvatura corneana.

Boston keratoprosthesis and Ahmed glaucoma valve for visual rehabilitation in congenital anterior staphyloma.

Congenital anterior staphyloma entails grave visual prognosis. The majority of reported patients have undergone enucleation. We report a promising result of staphylectomy with implantation of a keratoprosthesis and a glaucoma drainage device in a seven-month-old child with a large, congenital anterior staphyloma.

Congenital Glaucoma from Sturge-Weber Syndrome: A Modified Surgical Approach

Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder that causes congenital glaucoma. Previous experiences have shown that drainage procedures are often required to control associated glaucoma. The conventional surgical approach in trabeculectomy carries a significant risk of intraoperative expulsive hemorrhage. Here, we describe a modified approach of the conventional trabeculectomy technique, which may lower the risk of expulsive hemorrhage. A viscoelastic device was employed to maintain a steady intraocular pressure throughout the procedure. Details of the surgical technique and material used are described. One patient with congenital glaucoma associated with SWS underwent a successful trabeculectomy using the modified technique. Postoperative intraocular pressure was successfully reduced and no intraoperative complications occurred. We describe a successful case of trabeculectomy in a SWS case where a modified technique was applied.

Espessura corneana central e suas correlações com outros dados biométricos oculares em pacientes portadores de glaucoma congênito / Central corneal thickness and its correlations with other ocular biometric data in patients with congenital glaucoma

OBJETIVO: Estudar a distribuição da espessura corneana central e suas correlações com outros dados biométricos em pacientes com glaucoma congênito. MÉTODOS: Pacientes foram divididos em dois grupos, o A composto por portadores de glaucoma congênito, sendo este subdividido em subgrupos: com estrias de Haab (A1) e sem estrias de Haab (A2). O B representou o grupo controle. RESULTADOS: O grupo A apresentou diâmetro corneano entre 11 e 15,5 mm, com média de 14,13 mm e desvio padrão de 1,28, enquanto o grupo B apresentou valores entre 11,5 e 12,5 mm, com média de 12,01 mm com desvio padrão de 0,09 (t=-8,9723 e p=1,5083 em nível 0,05). Os glaucomatosos apresentaram maiores valores médios de diâmetro axial (t=-6,46315, p=9,2498 em nível de significância de 0,05), e menores valores médios ceratométricos em relação aos controles. O subgrupo A1 apresentou espessura corneana central de 539 ± 46 µm, o subgrupo A2 apresentou média de 571 ± 56 µm e o grupo B de 559 ± 28 µm (t=0,43746 e p=0,66291). As correlações entre diâmetro corneano e axial foram positivas nos dois grupos. Já entre diâmetro corneano e ceratometria média foram negativas nos dois grupos. CONCLUSÃO: Os glaucomatosos apresentaram maior média de diâmetro axial e menor média ceratométrica em relação aos controles. Não houve diferença estatisticamente significativa da espessura corneana central. O diâmetro corneano se correlacionou positivamente como diâmetro axial e negativamente com a ceratometria média. Não se pode estabelecer correlações entre espessura corneana central e os demais dados biométricos.

PURPOSE:To study the distribution of the central corneal thickness and its correlations with other biometric data in patients with congenital glaucoma. METHODS: Patients had been divided into two groups: group "A", composed of patients with congenital glaucoma, being subdivided in two sub-groups: with Haab striae (A1) and without Haab striae (A2), and group"B" that represented the controls. RESULTS: The group A presented corneal diameter between 11 and 15.5 mm, with mean of 14.13 mm and standard deviation (SD) of 1.28, while group B presented values between 11.5 and 12.5 mm, with average of 12.01 mm SD of 0.09 (t=-8.9723 and p=1.5083 in level 0.05). Glaucomatous patients presented greater mean values of axial diameter (t=-6.46315, p=9.2498 with level of significance of 0.05), and smaller mean keratometry in relation to the controls. The A1 sub-group presented mean central corneal thickness of 539 ± 46 µm, the A2 presented 571 ± 56 µm, and Group B 559 ± 28 µm (t=0.43746 and p=0.66291). The correlation between corneal and axial diameters was positive in both groups. The correlation between corneal diameter and mean keratometric values was negative in both groups. CONCLUSIONS: Patients with congenital glaucoma presented greater mean of axial dia meter and smaller mean keratometric values compared to the controls. No statistical significant difference of the central corneal thickness was demonstrated. Corneal and axial diameters were correlated positively. Corneal diameter was correlated negatively with the mean keratometry. It was not possible to establish correlations between the central corneal thickness and other biometric data.

Update on congenital glaucoma.

Congenital glaucoma is a global problem and poses a diagnostic and therapeutic challenge to the ophthalmologist. A detailed evaluation under general anesthesia is advisable to establish the diagnosis and plan for management. Medical therapy has a limited role and surgery remains the primary therapeutic modality. While goniotomy or trabeculotomy ab externo is valuable in the management of congenital glaucoma, primary combined trabeculotomy–trabeculectomy offers the best hope of success in advanced cases. Trabeculectomy with antifibrotic agent and glaucoma drainage devices has a role in the management of refractory cases, and cyclodestructive procedures should be reserved for patients where these procedures have failed. Early diagnosis, prompt therapeutic intervention and proper refractive correction are keys to success. Management of residual vision and visual rehabilitation should be an integral part of the management of children with low vision and lifelong follow-up is a must.

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.

Glaucoma congénito / Congenital glaucoma

El glaucoma congénito es una enfermedad rara cuya incidencia exacta es difícil de determinar, la mayoría de los pacientes no se manifiestan al nacimiento a causa de la pequeña producción de humor acuoso en los primeros meses. La aparición precoz tienen un mal pronóstico, estos pacientes tiene un drenaje tan alterado que ni siquiera pueden manejar la escasa cantidad de humor acuoso producida por el ojo del neonato. Se describe un paciente con glaucoma congénito diagnosticado en la etapa de lactante cuya alteración más significativa resultó ser en agrandamiento antero posterior del globo ocular.

The congenital glaucoma is a strange disease whose exact incidence is difficult to determine, most of the patients are not declareed at birth because of the small production of aqueous humor in the first months. The precocious apparition has a bad prognosis, these patients have such an altered drainage that they cannot even manage the scarce quantity of aqueous humor produced by the eye of the neonate. A patient is described with congenital glaucoma diagnosed in the lactation stage whose most significant alteration turned out to be in the anteroposterior enlargement of the eyeball.

Glaucoma congénito: presentación de un caso / Congenital glaucoma: presentation of a case

Por no conocer ningún caso presentado en la provincia de Matanzas desde que comenzó a brindar sus servicios la especialidad de Genética Clínica, se realizó una revisión bibliográfica sobre Glaucoma Congénito, a punto de partida de un niño nacido a término en el año 2006 en el área de salud de Varadero, con diagnóstico al nacimiento, tratamiento quirúrgico y seguimiento por equipo multidisciplinario. Se brindó asesoramiento genético a la familia y tratamiento psicológico, apoyándonos en los principios básicos de este proceso y mostrando la interrelación dinámica entre sus elementos.

Finding no case in the province of Matanzas since the service of the specialty of Genetic Clinic was open, we carried out a bibliographic review on congenital glaucoma after a child was born in term in 2006 in the health area of Varadero, with diagnosis at born, surgical treatment and follow up by a multidisciplinary team. We offered the family genetic assessment and psychological treatment, on the bases of the main principles of this process and showing the dynamic interrelation between these elements.

Visual impairment secondary to congenital glaucoma in children: visual responses, optical correction and use of low vision AIDS

INTRODUCTION: Congenital glaucoma is frequently associated with visual impairment due to optic nerve damage, corneal opacities, cataracts and amblyopia. Poor vision in childhood is related to global developmental problems, and referral to vision habilitation/rehabilitation services should be without delay to promote efficient management of the impaired vision. OBJECTIVE: To analyze data concerning visual response, the use of optical correction and prescribed low vision aids in a population of children with congenital glaucoma. METHOD: The authors analyzed data from 100 children with congenital glaucoma to assess best corrected visual acuity, prescribed optical correction and low vision aids. RESULTS: Fifty-five percent of the sample were male, 43 percent female. The mean age was 6.3 years. Two percent presented normal visual acuity levels, 29 percent mild visual impairment, 28 percent moderate visual impairment, 15 percent severe visual impairment, 11 percent profound visual impairment, and 15 percent near blindness. Sixty-eight percent received optical correction for refractive errors. Optical low vision aids were adopted for distance vision in 34 percent of the patients and for near vision in 6 percent. A manual monocular telescopic system with 2.8 × magnification was the most frequently prescribed low vision aid for distance, and for near vision a +38 diopter illuminated stand magnifier was most frequently prescribed. DISCUSSION AND CONCLUSION: Careful low vision assessment and the appropriate prescription of optical corrections and low vision aids are mandatory in children with congenital glaucoma, since this will assist their global development, improving efficiency in daily life activities and promoting social and educational inclusion.

Comparative docking studies of CYP1b1 and its PCG-associated mutant forms.

Molecular docking has been used to compare and contrast the binding modes of oestradiol with the wild-type and some disease-associated mutant forms of the human CYP1b1 protein.The receptor structures used for docking were derived from molecular dynamics simulations of homology-modelled structures. Earlier studies involving molecular dynamics and principal component analysis indicated that mutations could have a disruptive effect on function,by destabilizing the native properties of the functionally important regions, especially those of the haem-binding and substrate-binding regions,which constitute the site of catalytic activity of the enzyme.In order to gain more insights into the possible differences in substrate-binding and catalysis between the wild-type and mutant proteins,molecular docking studies were carried out. Mutants showed altered protein -ligand interactions compared with the wild-type as a consequence of changes in the geometry of the substrate-binding region and in the position of haem relative to the active site. An important difference in ligand -protein interactions between the wild-type and mutants is the presence of stacking interaction with phenyl residues in the wild-type,which is either completely absent or considerably weaker in mutants.The present study revealed essential differences in the interactions between ligand and protein in wild-type and disease mutants,and helped in understanding the deleterious nature of disease mutations at the level of molecular function.

Efficacy and safety of trabeculectomy with mitomycin C for childhood glaucoma: a study of results with long-term follow-up

OBJECTIVE: To evaluate the safety and effectiveness of trabeculectomy with mitomycin C in the management of childhood glaucoma. INTRODUCTION: The use of antifibrotic agents enhances the success of trabeculectomy performed in both adults and children. METHODS: A retrospective chart review (1991-2001) of 114 patients (114 eyes) from 0-14 years of age with congenital or developmental glaucoma. These patients underwent trabeculectomy with mitomycin but had not been previously treated with any antifibrotic agent. RESULTS: The mean patient age was 57.36 51.14 months (range: 0.5-168 months). Treatment was considered successful in 63 eyes (55.26 percent), with a mean intraocular pressure of 12.11 3.98 mmHg. For patients categorized as successfully treated, the mean follow-up time was 61.16 26.13 months (range 12-113 months). A post-surgical intraocular pressure of < 16 was observed in 47 eyes. The life-table success rates for intraocular pressure control at 24, 36, 48, and 60 months were 90.2 percent, 78.7 percent, 60.7 percent and 50.8 percent, respectively. The cumulative probability of failure was 40.8 percent at 12 months. Following surgery, endophthalmitis appeared in eight eyes (4.88 percent) after an average 36.96 months (range: 1.7-106 months). Other complications included expulsive hemorrhage, flat anterior chamber and bleb leak. DISCUSSION: It has been reported in pediatric patients that trabeculectomy without adjunctive antimetabolites achieves a successful outcome in 30 percent to 50 percent of cases. In our study, treatment was considered successful in 63 eyes (55.26 percent) within 61.16 26.13 months of follow-up. CONCLUSIONS: Trabeculectomy with mitomycin is safe and effective for short-term or long-term treatment of congenital or developmental glaucoma. The frequency of bleb-related endophthalmitis was no higher in these patients than that described in adults.

Perimetria automatizada em pacientes com glaucoma congênito primário / Automated perimetry in patients with primary congenital glaucoma

OBJETIVO: Identificar e caracterizar os defeitos nos exames de perimetria automatizada encontrados nos pacientes com diagnóstico de glaucoma congênito primário. MÉTODOS: Foram avaliados retrospectivamente os campos visuais automatizados de 81 olhos (48 pacientes), sendo 15 olhos sem glaucoma (grupo N) e 66 olhos com glaucoma (grupo G). Os olhos com glaucoma foram divididos em olhos com alterações campimétricas incipientes, caracterizadas pelo MD> -6 dB (grupo G I= 41 olhos) e olhos com alterações campimétricas moderadas/avançadas, caracterizadas pelo MD < -6 dB (grupo G II= 25 olhos). RESULTADOS: No grupo G I observaram-se 68 por cento de campos visuais normais, 22 por cento de alterações localizadas e 10 por cento com baixa difusa da sensibilidade. No grupo G II observaram-se 56 por cento de baixa difusa da sensibilidade e 44 por cento de defeitos localizados. O defeito campimétrico localizado mais comum foi o escotoma paracentral inferior. Os campos visuais normais do grupo G I revelaram limiar foveal e MD mais baixos que o grupo N. O "glaucoma hemifield test" foi normal em 68 por cento dos olhos no grupo G I, e foi anormal em 100 por cento dos olhos no grupo G II. CONCLUSÃO: O exame de perimetria automatizada mostrou-se eficaz em revelar as alterações campimétricas típicas do glaucoma, sendo importante para avaliação imediata e fornecendo parâmetros para seguimento dos pacientes com glaucoma congênito.

PURPOSE: To identify and characterize defects using the automated perimetry test in patients with primary congenital glaucoma. METHODS: Automated visual fields and charts of 81 eyes (48 patients) with congenital glaucoma were retrospectively analyzed, 15 being normal eyes (group N) and 66 eyes with primary congenital glaucoma (group G). Eyes of group G were grouped in eyes without perimetric changes and with early defects, characterized by MD> -6 dB (group G I= 41 eyes), and eyes with mild/advanced perimetric defects characterized by MD < -6 dB (group G II= 25 eyes). Main outcome measures: reliability indices, global indices, GHT report, foveal threshold index and descriptive visual field defects. RESULTS: The majority of patients showed good reliability. In group G I, 68 percent had normal visual fields; 22 percent showed localized defects and 10 percent showed general reduction of sensitivity. In group G II, 56 percent of the visual fields showed general reduction of sensitivity and 44 percent showed localized defects. The most common localized change was inferior paracentral scotoma. Eyes with normal visual fields in group G I had foveal threshold and MD values lower than normal eyes. The hemifield test was normal in 68 percent of eyes in group G I, and was abnormal in 100 percent of eyes in group G II. CONCLUSIONS: Automated perimetry test was able to show typical defects of glaucoma contributing to initial evaluation and giving reliable parameters for the follow-up of eyes in patients with congenital glaucoma.

Resultado em longo prazo da trabeculectomia no tratamento do glaucoma congênito primário / Long-term results of trabeculectomy in the treatment of primary congenital glaucoma

Objetivo: Avaliar o resultado da trabeculectomia a 180º superiores como primeiro procedimento em paciente com glaucoma congênito primário. Local: Serviço de Oftalmologia do Hospital Municipal da Piedade - Universidade Gama Filho - RJ. Métodos: Foram avaliados 35 olhos de 23 pacientes portadores de glaucoma congênito primário submetidas a trabeculotomia no Hospital Municipal da Piedade, avaliadas e operadas sempre pelo mesmo cirurgião. Todos os pacientes foram submetidos a exame sob narcose 1 mês após a cirurgia e de acordo com o resultado cirúrgico a intervalos trimestrais ou semestrais. Foram considerados controlados olhos com pressões oculares menores ou iguais a 17 mmHg, ou redução da pressão ocular de pelo menos 20 por cento após a cirurgia, sem sinais de evolução da doença e considerados como sucesso absoluto, controle do glaucoma com apenas um procedimento, sucesso relativo quando a doença estava estabilizada com um procedimento e medicação tópica complementar e insucesso quando foram necessários mais de um procedimento cirúrgico. Resultados: O período médio de acompanhamento foi de 44,48 meses variando de 12 a 126 meses. Do total dos pacientes, 88,5 por cento foram operados antes de 1 ano de idade. O índice de sucesso absoluto foi de 62,9 por cento e relativo de 22,8 por cento com sucesso total de 85,7 por cento. A pressão ocular pré-operatória média foi de 20,9 +/- 4,5 mmHg e pós-operatória de 12,6 +/- 4,1mmHg, havendo diferença estatisticamente significativa (p+0,0000). Nos pacientes operados até 3 meses de idade obtivemos índice de sucesso de 93,75 por cento, nos operados entre 3 e 6 meses 81,8 por cento, 75 por cento entre 6 meses e uma ano e 75 por cento após um ano de idade. Não houve diferença significativa na medida do diâmetro horizontal da córnea pré e pós-operatória (p+0,18). Conclusões: A trabeculectomia é uma técnica segura e com alto índice de sucesso quando realizada como primeiro procedimento cirúrgico no glaucoma congênito.

Congenital glaucoma associated with 22p+ variant in a dysmorphic child.

A case of congenital glaucoma with developmental delay and several dysmorphic features showing 22p+ chromosomal variant is reported.

Safety and efficacy of simultaneous bilateral primary combined trabeculotomy-trabeculectomy for developmental glaucoma.

PURPOSE: To establish the safety and efficacy of simultaneous bilateral primary combined trabeculotomy-trabeculectomy for developmental glaucoma. METHODS: We studied 109 consecutive patients who underwent planned simultaneous bilateral primary combined trabeculotomy-trabeculectomy for developmental glaucoma by a single surgeon from January 1990 through December 1999. The main outcome measures were postoperative intraocular pressure (IOP), corneal clarity and diameter, visual acuity, bleb characteristics, time of surgical failure and complications. Postoperative complications including endophthalmitis and anaesthetic morbidity and mortality were also analysed. RESULTS: The series consisted of 218 primary combined trabeculotomy-trabeculectomy surgeries during 109 anaesthesias. The mean follow-up period was 16.33 +/- 16.22 months. The IOP reduced from 26.4 +/- 5.9 mmHg to 13.5 +/- 4.5 mmHg, with a mean percentage reduction of 46.2 +/- 23.7 (P < 0.0001). The success (IOP < 16 mmHg) probabilities were 90.9%, 88.0% and 69.3% at first, second and third year respectively (Kaplan-Meier analysis). The success probability of 69.3% obtained at third year was maintained till 6 years of follow-up. One hundred and sixty six (76.1%) eyes had significant corneal oedema. Postoperatively, the cornea cleared in 93 (57.8%) eyes. Clinically, well functioning blebs were present in 114 of 171 eyes (66.6%). Postoperatively, 18 (8.3%) eyes developed shallow anterior chamber and 6 (33.3%) of them required surgical reformation. There was no incidence of endophthalmitis or any other sight-threatening complication. Of the anesthetic complications, apnea occurred in 17 (15.6%) patients and all were successfully resuscitated. The most serious post-anaesthetic complication was cardio-pulmonary arrest that occurred 5 hours postoperatively following aspiration during feeding in one child; this child could not be resuscitated. Two children had delayed recovery (2 and 4 hours respectively). The child who had delayed recovery by 2 hours survived and has completed 3 years of follow-up while the other child expired 48 hours later. CONCLUSION: Simultaneous bilateral primary combined trabeculotomy-trabeculectomy is safe and effective for developmental glaucoma. It obviates the need for long second anaesthesia with its attendant risks. It offers several other benefits to the patients and families.