A rare optineurin mutation in an Indian family with coexistence of JOAG and PCG

Purpose: This study focused on the genetic screening of Myocilin (MYOC), Cytochrome P450 family 1 subfamily B member 1 (CYP1B1), Optineurin (OPTN), and SIX homeobox 6 (SIX6) genes in a family with coexistence of primary congenital glaucoma (PCG) and juvenile open?angle glaucoma (JOAG). Methods: Sanger sequencing was used to examine the coding region of all four genes. Six different online available algorithms were used for the pathogenicity prediction of missense variant. Structural analysis was done using Garnier–Osguthorpe–Robson (GOR), PyMol, ChimeraX, and Molecular Dynamic (MD) Simulations (using Graphics Processing Unit (GPU)?enabled Desmond module of Schrödinger). Results: There were a total of three sequence variants within the family. All seven algorithms determined that a single mutation, G538E, in the OPTN gene is pathogenic. The loops connecting the strands became more flexible, as predicted structurally and functionally by pathogenic mutations. Mutations create perturbations and conformational rearrangements in proteins, hence impairing their functioning. Conclusion: In this study, we describe a North Indian family in which members were having JOAG and PCG due to a rare homozygous/ heterozygous mutation in OPTN. The coexistence of two types of glaucoma within a single pedigree suggests that certain OPTN mutations may be responsible for the onset of different glaucoma phenotypes.

Evaluation of a Protocol for Diagnosis and Treatment of Primary Congenital Glaucoma in Tanta University Eye Hospital

Background: Primary congenital glaucoma is the most common type of glaucoma in infancy. It manifests within the first few years of life and is not associated with any other systemic or ocular abnormalities. This study aimed to evaluate a protocol for diagnosis and treatment of primary congenital glaucoma at Tanta University Eye Hospital.Methods: The study was prospective interventional on 60 eyes of 36 infants and children who presented between December 2018 to March 2021 at Tanta University Eye Hospital in Egypt. All patients were presented with primary congenital glaucoma and were managed according to specific diagnostic, therapeutic and follow up regimen. Pre- and Post-operative clinical data were recorded, and results were studied.Results: The mean of age±SD (range) at first presentation was 11.33±21.76 (0.5-120) months and of follow-up period was 7.85±2.71 (4-13) months. The mean of pre-operative IOP, corneal diameter, C/D ratio and AxL was 24.1±5.9 (12–41.5) mmHg, 12.97±0.69 (11–14.5) mm, 0.57±0.18 (0.2–0.9) and 21.87±1.28 (19.1–26.8) mm respectively, and post-operatively was 14.4±3.09 (8.5–20.5) mmHg, 12.71±0.89 (11–14.5) mm, 0.48±0.22 (0.2–1) and 22.16±1.53 (19–25.9) mm respectively. Surgical interventions included 1ry surgeries (trabeculotomy in 21 eyes and CTT with MMC in 39 eyes), 2ry surgeries (CTT with MMC in four eyes) and 3ry surgery (Ahmed valve implantation in one eye). Complete success was achieved in 38 eyes (63.3%) and qualified success in 22 eyes (36.7%).Conclusions: The current protocol applied at Tanta University Eye Hospital for diagnosis and treatment of primary congenital glaucoma proved efficacy and safety. Primary surgical intervention in the form of trabeculotomy (for mild cases) and combined trabeculotomy-trabeculectomy with MMC (for moderate to advanced cases) is a successful regimen for management of these eyes.

Evaluation of visual field changes with retinal nerve fiber layer thickness in primary congenital glaucoma

Purpose: To evaluate visual field changes in primary congenital glaucoma (PCG) with retinal nerve fiber layer thickness on optical coherence tomography. Methods: In this cross?sectional, observational study, consecutive PCG children who underwent combined trabeculotomy with trabeculectomy and on regular follow?up were enrolled. All patients were aged over four years and co?operative for RNFL OCT and visual field examination. Perimetry was done on Humphrey visual field (HVF) analyzer using 30?2 and 10?2 SITA standard algorithms as appropriate. If a reliable automated perimetry was not feasible, kinetic perimetry was done. The following were noted at baseline and every follow?up: age, sex, visual acuity, intraocular pressure (IOP), cup–disc ratio (CDR), corneal diameters, refraction, any topical antiglaucoma medications, surgeries underwent, age at surgery and duration between surgery and final examination. Results: Forty?eight eyes of 34 children operated for PCG and 19 eyes of 17 controls were analyzed. A statistically significant thinner average RNFL thickness of 87.2 ± 28 ?m was noted in PCG eyes as compared to controls with 100.6 ± 7.2 ?m (P = 0.04). The mean cup–disc area ratio on OCT in PCG eyes was 0.43 ± 0.2 (0.02–0.93) and in control eyes was 0.23 ± 0.07 (0.1–0.4) (P < 0.001). On RNFL OCT, there was significant focal RNFL loss in temporal superior (P = 0.003), nasal inferior (P = 0.037) and temporal inferior (P < 0.001) quadrants compared to controls. Among PCG eyes, 20/48 eyes (41.7%), had definitive, reproducible glaucomatous VF defects. Mean baseline IOP in PCG eyes with VF defect was 28.7 ± 5.7 mmHg and in eyes with normal VF was 24.6 ± 5.9 mmHg (P = 0.03). On univariate regression analysis, higher baseline IOP was significantly associated with both RNFL loss (odds ratio (OR): ?2.17) and VF defects (OR: 3.35). Fluctuation in follow?up IOP (OR: 3.33) was also significantly associated with the presence of VF defects. On multivariable regression analysis maximum, IOP was significantly associated with RNFL loss and VF defects. Conclusion: Peripapillary RNFL thickness could be used to identify PCG eyes having visual field loss and possibly poor visual function from PCG eyes without visual field defects. Baseline and follow?up IOP, significantly correlated with RNFL thickness in PCG eyes

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.

Outcome evaluation of non-penetrating trabecular surgery combined with suture trabeculotomy for the treatment of primary congenital glaucoma / 中华实验眼科杂志

Objective:To investigate the efficacy and safety of non-penetrating trabecular surgery combined with nearly 360-degree suture trabeculotomy for the treatment of primary congenital glaucoma (PCG).Methods:An observational case series study was conducted.A total of 29 cases (50 eyes) with PCG, including 21 males (35 eyes) and 8 females (15 eyes), were enrolled in Jiangsu Province Hospital and Nanjing Children's Hospital from January to November, 2019.The age of subjects ranged from 1 month to 4 years, and the median age was 6 months.Non-penetrating trabecular surgery was first performed in order to open the Schlemm canal.The cannulation and nearly 360-degree suture trabeculotomy were then performed with the twisted 6-0 polypropylene suture.Intraocular pressure (IOP), corneal diameter, cup-to-disc ratio (c/d) and complications were recorded preoperatively and 1 week, 1 month, 3 months, 6 months, 9 months, 12 months, and 24 months postoperatively, and the proportion of sutures successfully passed through the Schlemm canal and the success rate of operation were recorded.This study followed the Declaration of Helsinki.The study protocol was approved by an Ethics Committee of The First Affiliated Hospital of Nanjing Medical University (No.2019-SR-198). Written informed consent was obtained from the guardian of each subject prior to entering the study cohort.Results:Circumferential cannulation by suture was successfully performed in 90% of the subjects.The Harms trabeculotomy probe was applied in failed cases.Mean IOP was significantly lowered from preoperative (35.0±9.5) mmHg (1 mmHg=0.133 kPa) to (9.9±4.4), (10.0±4.2), (9.7±4.4), (9.0±2.9), (9.4±4.2), (9.3±3.3) and (9.5±3.8) mmHg at postoperative 1 week, 1 month, 3 months, 6 months, 9 months, 12 months and 24 months, respectively ( F=141.56, P<0.01). Mean corneal diameter was significantly reduced from preoperative (13.7±1.4) mm to (13.3±1.4), (12.9±1.4), (12.8±1.3), (12.7±1.2), (12.6±1.1), (12.6±1.1) and (12.8±0.4) mm at postoperative 1 week, 1 month, 3 months, 6 months, 9 months, 12 months and 24 months, respectively ( F=4.55, P<0.01). Mean c/d was significantly reduced from preoperative 0.81±0.15 to 0.55±0.22, 0.48±0.23, 0.45±0.22, 0.43±0.21, 0.41±0.20, 0.40±0.21 and 0.31±0.19 at postoperative 1 week, 1 month, 3 months, 6 months, 9 months, 12 months and 24 months, respectively ( F=21.07, P<0.01). Forty-two eyes (93.3%) achieved complete success and 45 eyes (100%) achieved qualified success at postoperative 12 months.No severe complications were observed during or after surgery. Conclusions:Non-penetrating trabecular surgery combined with nearly 360-degree suture trabeculotomy can effectively treat patients with PCG without any severe complications.

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.

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)

Expression differences and clinical significance of miR-29b,miR-24 and miR-200c in plasma of infants with PCG and normal / 眼科新进展

Objective To identify the expression differences of mircoRNA-29 b (miR-29b),microRNA? 24 (miR-24) and microRNA-200 c (miR-200c) in plasma of infants with primary congenital glaucoma (PCG) and normal,and analyze its clinical significance.Methods The expression quantity of microRNAs (miR-29b,miR-24,miR-200c) in plasma of PCG group (16 cases) and normal control group (49 cases) were detected by RT-PCR,and the relationship between their expression differences and severity of disease were analyzed.The diagnostic value of miRNAs for PCG was evaluated by receiver-operating characteristic curve (ROC).Results The expression quantity of miR-29b,miR-24,miR-200c in PCG group (0.31 ±0.19,0.17 ±0.16,0.55 ±0.18,respectively) were significantly lower than those in normal control group (1.18-±0.52,2.86 ±2.65,1.62 ± 0.76,respectively) (all P ＜ 0.05);The expression quantity of miR-24 and miR-29b in plasma was related to the severity of PCG,the more severe the disease,the lower the expression;ROC curve indicated that miR-24 and miR-29b had a higher diagnostic value for PCG disease than miR-200c.Conclusion Free miRNAs in plasma may be used as a new plasma markers for auxiliary diagnosis of PCG.

A case of spontaneously resolved primary congenital glaucoma.

Primary congenital glaucoma usually presents as enlarged and hazy cornea at birth or early childhood. The diagnosis is based on a thorough clinical examination under anesthesia. Most cases require surgical intervention as the definitive treatment. In very rare instances, primary congenital glaucoma may arrest and resolve spontaneously. We describe a case of spontaneously arrested and resolved primary congenital glaucoma in a 37‑year‑old male presenting with large cornea, Haab’s striae, and normal intraocular pressure in one eye. Such a case has not been previously described from the Indian subcontinent.

Clinical evaluation of Ahmed glaucoma valve implantation for uncontrolled primary congenital glaucoma / 国际眼科杂志(Guoji Yanke Zazhi)

?AlM: To investigate the outcome and safety of Ahmed glaucoma valve implantation treatment in uncontrolled primary congenital glaucoma ( PCG) . ? METHODS: Twenty - two eyes in 22 children with uncontrolled PCG were reviewed retrospectively and underwent Ahmed glaucoma valve implantation treatment from January 2011 to December 2014. Main checking index included intraocular pressure ( lOP ) before and after operation, corneal diameter and complications. ?RESULTS: Preoperative mean age was 3. 74±2. 24y, and 2. 59 ± 1. 78y apart from the last operation. Postoperative average lOP was 35. 22 ± 6. 36mmHg. Average corneal diameter was 12. 79 ± 0. 75mm. Mitomycin C ( 0. 3 - 0. 5mg/mL ) was used in all operations for 3-5min. Glaucoma valves were implanted in the temporal or nose above the equator sclera. Postoperative lOP was 11. 4±4. 45mmHg at 1wk, and 16. 73± 7. 23mmHg after 12mo. As lOP ?CONCLUSlON:Ahmed glaucoma valve implantation in uncontrolled PCG is a safe and viable 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 ¡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.

Visual Outcome in Children With Congenital Glaucoma

PURPOSE:To evaluate the prognosis of vision and the development of amblyopia in primary congenital glaucoma patients. METHODS: The author reviewed 38 eyes of 22 primary congenital glaucoma patients and evaluated variables such as age at time of surgery and at the last visit, preoperative IOP, Cup-to-disc(CD) ratio, corneal diameter, refractive error, axial lengths and IOP, CD ratio and visual acuity at the last visit. According to visual acuity, the patients were divided into 3 groups, good (>0.5), fair (0.1~0.5), and poor (<0.1). The amblyopia was defined when BCVA was below 0.8 and no evidence of progression of glaucoma. There were 4 types of amblyopia: deprivation, anisometropic, strabismic and organic. The author compared the 3 groups and evaluated factors affecting the vision as well as the prevalence of amblyopia. RESULTS: There were 17 eyes in the good group, 4 eyes in the fair group and 17 eyes in the poor group (p<0.05). Amblyopia developed in 17 eyes with 7 eyes showing deprivation amblyopia. Anisometropic and organic amblyopia were each found in 4 eyes, and strabismic amblyopia was found in 2 eyes. The postoperative IOP and CD ratio and preoperative CD ratio were significantly lower in the good group than the other groups (p<0.05). CONCLUSIONS: Final VA was poor in 45% of eyes with primary congenital glaucoma, and amblyopia developed in 45% of these eyes. The prognosis for vision may be related to the treatment of glaucoma and amblyopia.

A comparison study of the clinical features between Chinese and Indian primary congenital glaucoma patients / 国际眼科杂志(Guoji Yanke Zazhi)

· AIM: To summarize the clinical features of Chinese primary congenital glaucoma and to investigate the discrepancies of the clinical features between the Chinese and Indian patients.congenital glaucoma patients were reviewed. The clinical features were summarized as several quantifiable clinical parameters and the severity of the disease was evaluated. Both the quantified clinical features and severity were statistically compared with those of the Indian patients, which were cited from the previous published articles.included in the study. In Chinese patients, sex ratio (male to female )was about 2:1, family history was presented in 3 patients (7.5%) and consanguinity was found in one patient (2.5%). The main symptoms and signs observed in Chinese patients spanned a wide spectrum of manifestations. The most frequent signs noted on the initial examination were enlarged eyeball (42.5%) and decreased visual acuity (35.0%). Compared with Indian patients, Chinese patients had a later onset, a delayed diagnosis, more severe corneal changes and more severe optic nerve damages (P＜0.01). The combined tabeculectomy and trabeculotomy operation was preferred by both Chinese and Indian doctors whereas a higher proportion of Indian patients received the combined operation (P＜0.01). The proportions of the severity grade were different between Chinese and Indian patients. Most Chinese patients were in the severe grade while most Indian patients were in the very severe grade (P＜0.01).patients were sporadic and non-consanguineous.Compared with Indian patients, Chinese patients had a relative later onset, a delayed diagnosis and treatment.More attempts are needed in Chinese PCG prevention and treatment.