Profile of Newly Diagnosed Childhood Glaucoma in India: Indian Childhood Glaucoma Study (ICGS) Group 1.

OBJECTIVE: To report the profile of newly diagnosed childhood glaucoma using the Childhood Glaucoma Research Network (CGRN) classification, presenting over 1 year from across centers in India. DESIGN: Prospective observational multicentric study. SUBJECTS: Newly diagnosed children aged < 18 years diagnosed with childhood glaucoma according to CGRN criteria presenting between January and December 2019 to 13 centers across India. METHODS: All children underwent a comprehensive ocular examination, including examination under anesthesia for younger children, and were diagnosed with childhood glaucoma as per CGRN. Data were entered in a standard Excel chart. Refraction and visual acuity assessments were done when feasible. MAIN OUTCOME MEASURES: The profile of newly diagnosed childhood glaucoma in different parts of India and the severity of glaucoma at presentation. RESULTS: A total of 1743 eyes of 1155 children fulfilled the definition of glaucoma and were analyzed. Primary congenital glaucoma (PCG) comprised the single largest group (34.4%), most of which were infantile onset (19%). Neonatal-onset PCG comprised 6.2% of all glaucoma. Secondary glaucoma constituted 53.4% of all glaucoma, one-half of which were acquired conditions (28%), followed by isolated ocular anomalies (14.7%), glaucoma after cataract surgery (6.7%), and glaucoma with nonacquired systemic diseases (4.5%). Of the 1743 eyes with glaucoma, all 3 parameters for severity grading were available in 842 eyes, of which 501 (59.5%) eyes presented with mild, 320 (38%) with moderate, and 21 (2.5%) with severe glaucoma. Nearly one-third of the children (28.5%) were not brought back for follow-up after the initial treatment given. CONCLUSIONS: Our study has one of the largest numbers of consecutive children with glaucoma classified according to the CGRN classification. Despite a widely diverse population, the profile of childhood glaucoma was relatively uniform across India. Childhood glaucoma is a significant problem in India, primarily treated in tertiary care hospitals. The data presented may be the tip of the iceberg because we have only reported the children who reached the hospitals offering treatment for this challenging disease. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Central retinal vessel trunk exit location: An important factor while evaluating the neuroretinal rim.

PURPOSE: Optic nerve head (ONH) evaluation plays a key role in differentiating normal from glaucomatous disk. Thinning of the inferior neuroretinal rim (NRR) has been noted in early glaucoma. However, NRR thickness in different quadrants appears to depend on various factors including central retinal vessel trunk exit (CRVT) position. We evaluated ocular parameters that determined the NRR thickness in the different quadrants of normal eyes. METHODS: Retrospective review of demographic and ocular data from 773 eyes of 388 subjects with normal ONH over one year was undertaken. RESULTS: Nearly 54% were males, and the mean age was 43.2 years. The CRVT exit was central in 50% (773). The common site for noncentral CRVT was superotemporal (ST) [37%, 141/384] followed by inferotemporal (IT) [35%, 135/384]. With noncentral CRVT, the probability that the inferior, superior, nasal and temporal (ISNT) rule was not followed was 1.42 times ( P < 0.001). The thinnest rim quadrant (TRQ) was mostly ST (69%) irrespective of CRVT location. The TRQ was IT in 40% when CRVT was noncentral and 82% with IT CRVT exit. With noncentral CRVT, round disks favored noncompliance [132 (54.1%), odds ratio (OR) 2.56] with the ISNT rule. The OR of noncompliance with the ISNT rule increases 1.89 times with inferonasal CRVT and 1.22 times with a unit increase in the axial length. CONCLUSION: TRQ was IT in IT CRVT, and noncompliance with the ISNT rule was observed with large disks, longer axial length, and noncentral CRVT. This implies that despite the ISNT rule being violated these eyes do not have optic nerve pathology and should not be subjected to unnecessary diagnostic tests.

Intermediate-term outcome of Aurolab aqueous drainage implant in refractory glaucoma.

PURPOSE: The purpose of the study was to evaluate the intermediate-term outcomes of Aurolab aqueous drainage implant (AADI) in terms of intraocular pressure (IOP) lowering from baseline levels, the number antiglaucoma medications (AGMs) in the postoperative phase, and the rate of complications. METHODS: It was a retrospective interventional case series. All patients who underwent AADI surgeries with sulcus fixation from March 2018 to September 2018 at a tertiary eye care hospital in North India with a minimum follow-up of 1 year were recruited for the study. A standard AADI technique was employed. The primary outcome measures were the postoperative IOP, the requirement of AGMs, and early and late postoperative complications. RESULTS: A total of 20 patients were recruited in the study. The mean follow-up period was 25.25 ± 3.76 months. The mean IOP reduced from 33.20 ± 7.95 mmHg to 19.45 ± 9.19 mmHg at day 1, 13.62 ± 3.92 mmHg at 6 months, 12.78 ± 3.36 mmHg at 1 year, and 13.0 ± 2.53 mmHg at 2 years postoperatively (P < 0.001). The mean number of AGMs also reduced from 3.7 ± 0.97 to 0.35 ± 0.81 at 6 months, 0.42 ± 0.83 at 1 year, and 0.26 ± 0.73 at 2 years postoperatively (P < 0.001). Early postoperative complications, such as hypotony and hyphema, were seen in 5 (25%) patients, although none of them was sight-threatening. Late postoperative complications, such as hypertensive phase and persistent fibrinous membrane, were also seen in five eyes. CONCLUSION: The study assessed the clinical outcomes, safety profile, and long-term AGM requirement with AADI and found it to be a good viable surgical option in refractory glaucoma.

A unique case of bilateral nanophthalmos and pigmentary retinal abnormality with unilateral angle closure glaucoma and optic disc pit.

BACKGROUND: Microophthalmos or 'dwarf eye' is characterized by an axial length 2 standard deviation less than age-matched controls. It is classified into nanophthalmos, relative anterior microphthalmos, and posterior microphthalmos based on the anterior segment: posterior segment ratio. Nanophthalmos can occur in association with optic disc drusen, foveoschisis, and retinitis pigmentosa, as an autosomal recessive syndrome linked to mutations in the MFRP gene. We report a case of bilateral nanophthalmos and pigmentary retinopathy with angle closure glaucoma and optic disc pit in one eye. We believe this to be the first case presenting with optic disc pit in association with nanophthalmos. CASE PRESENTATION: A 56-year-old female presented with bilateral small eyes, high hypermetropia, shallow anterior chamber depth, increased lens thickness, mid-peripheral retinal flecks, and macular edema. She also had high intraocular pressure in the right eye, with a disc cupping of 0.9 with an Optic disc pit. The macular edema in the right eye was found to occur in association with the Optic disc pit, whereas, in the left eye, it was associated with intra-retinal hemorrhages and diagnosed as macular branch retinal vein occlusion secondary to hypertension. She was started on anti-glaucoma medications in both eyes and planned for Anti-VEGF injection in the left eye. CONCLUSION: This case report is unique as it reports an association of Nanophthalmos with Optic Disc pit, with an associated angle closure glaucoma in the same eye, an association which has never been previously reported in the literature.

Profile of Childhood Glaucoma Attending a Tertiary Eye Care Center in Northern India.

Purpose: To ascertain the prevalence and clinical features of the various types of childhood glaucoma at a tertiary eye care hospital in Northern India. Materials and methods: Retrospective chart review of all children less than 16 years of age with childhood glaucoma who presented from 1st April 2014 to 31st March 2019, who was diagnosed to have any subtype of childhood glaucoma as per Childhood Glaucoma Research Network (CGRN) classification and advised appropriate management. Results: Out of 405 children with childhood glaucoma, 36% had primary glaucoma, whereas the rest had secondary glaucoma. Primary congenital glaucoma (PCG) was the most common form of primary glaucoma. Glaucoma associated with acquired conditions was the most common cause of secondary glaucoma. Primary glaucoma was mostly bilateral in contrast to secondary glaucoma. The most common age of presentation with primary glaucoma was <1 year of age, and in children with secondary glaucoma was 11-16 years. On presentation, 80% of eyes had intraocular pressure (IOP) of >20 mm Hg and 70% had cupping of >0.7. Eyes with PCG were primarily managed surgically. Conclusion: In our cohort, PCG was the most common primary childhood glaucoma. Traumatic glaucoma was the most common secondary glaucoma. Since childhood glaucoma is an important cause of visual morbidity in children, its timely diagnosis and prompt management are essential to prevent irreversible visual loss. Clinical significance: Understanding the disease pattern, their presenting features, and the proportion of different types of childhood glaucoma can help in planning appropriate eye care services, create awareness and better allocate resources to plan appropriate management strategies. Screening programs and counseling of parents should also be strengthened. How to cite this article: Dubey S, Jain K, Pegu J, et al. Profile of Childhood Glaucoma Attending a Tertiary Eye Care Center in Northern India. J Curr Glaucoma Pract 2023;17(2):68-74.

Acquired ectropion uveae: A case series.

Acquired ectropion uveae (AEU) is a common sequela to various inflammatory and ischemic conditions, though it is not well recognized. There is a paucity of literature describing AEU. So here, we present five cases where we documented ectropion uveae following chronic inflammation. Patients with ectropion uveae following chronic inflammation and ischemia were retrospectively reviewed. Their medical records and clinical findings were analyzed. Five patients of various ages were found to have AEU, of which 1 was post trabeculectomy with phacoemulsification and posterior chamber intraocular lens, 1 following neovascular glaucoma (NVG), 1 following uveitic glaucoma, and 2 following iridocorneal endothelial syndrome. Patients with NVG and uveitic glaucoma also had undergone glaucoma filtration surgeries. AEU may be seen secondary to inflammatory and ischemic processes and should be looked for carefully as they may cause progressive glaucoma.

Consensual eye intra-ocular pressure rise following unilateral glaucoma surgery.

Purpose: Consensual eye intra-ocular pressure (IOP) increase has been reported following ipsilateral glaucoma surgery. The study evaluated the need for increase in anti-glaucoma medications (AGM) ± glaucoma surgery to control the IOP in consensual eye following unilateral glaucoma surgery. Methods: Data of 187 consecutive patients who underwent either trabeculectomy or AGV implant were collected. Index (IE) and fellow eye (FE) IOP (baseline, follow-up day 1, week 1, months 1 and 3), acetazolamide and AGM use, FE surgery, glaucoma status, and relevant ophthalmological data were collected. Results: A significant increase from a baseline IOP of 14.4 mmHg was noted at week 1 (15.8 mmHg, p-0.005) and month 1 (15.62 mmHg, p-0.007) in FE (n-187). Among the 61 patients (33%, n-187) who needed additional intervention to reduce FE IOP, 27 (14.4%) underwent FE trabeculectomy. In the IE trabeculectomy group (n-164), significant increase in FE IOP was noted in week 1 (15.87 mmHg, p-0.014) and month 1 (15.61 mmHg, p-0.02), and in the IE AGV group (n-23) at day 1 (15.91 mmHg, p-0.06). Pre-operative acetazolamide resulted in significant increase in FE IOP at week 1 and month 1. Maximum increase in FE IOP of nearly 3.5 mm Hg was noted when IE IOP was persistently <5 mmHg at one month following surgery. Mean FE IOP remained elevated at all visits. Conclusion: An increase in fellow eye IOP needing any additional intervention in a third and surgical intervention in nearly a sixth meant that FE IOP be strictly monitored and addressed following unilateral glaucoma surgery.

Outcomes of ab-interno irrigating goniectomy with trabectome in primary and secondary glaucoma from a single center in India.

Purpose: This study was done to report intermediate-term outcomes of irrigating goniectomy with trabectome (trabectome) surgery among different types of glaucoma eyes from a single center in India using a cross-sectional, longitudinal, observational study design. Methods: Fifty-three patients (58 eyes) with glaucoma who underwent irrigating goniectomy with trabectome between January 2019 and February 2020 were included. Pre-operative data included age, gender, eye laterality, specific diagnosis, number of anti-glaucoma medications (AGMs), prior glaucoma surgeries, visual acuity, and intraocular pressure (IOP) on medical treatment. Post-operative data included IOP changes during the follow-up till 1-year, number of AGMs, any complications, or additional surgical intervention required. Success was defined as IOP ≤ 21 mmHg and ≥ 20% reduction of IOP from pre-operative IOP with no additional glaucoma surgery. Results: The cohort included 58 eyes (male 53.4% and female 46.6%) ranging from 0.6 to 81 years of age. The average baseline IOP was 23.4 ± 10.2 mmHg and reduced significantly with surgery to 14.1 ± 5.3 mmHg at 1-year follow-up. The AGMs reduced from 2.4 ± 1.4 pre-surgery to 1.6 ± 1.4 at 1-year follow-up. Four eyes required additional glaucoma surgeries for IOP control. The success rate of trabectome with phacoemulsification (88%) was discernibly higher than with trabectome alone (67%). Intra-operatively, significant blood reflux was noticed in 27 eyes, of which only one required tamponading with a viscoelastic agent. Conclusion: This study concludes that irrigating goniectomy with trabectome has good efficacy and safety in both pediatric and adult cases of glaucoma in terms of IOP control, reduction in AGMs, and low incidence of complications in the Indian population.

Association of primary angle-closure disease in patients with retinal vein occlusion in North Indian population.

Purpose: To determine the association of primary angle-closure disease (PACD) in patients with retinal vein occlusion (RVO) at a tertiary eye care center in North India. Methods: It is a cross-sectional, observational study. Sixty consecutive patients with retinal vein occlusion within a period of one year from a single tertiary eye care center were enrolled. Detailed history, slit-lamp examination of the anterior segment, intraocular pressure measurement by applanation tonometry, gonioscopy and fundus examination were done. Anterior chamber depth and axial length were also measured. Results: Among the 60 patients, 29 were males (48.3%) and 31 females (51.6%). Twenty-seven (45%) of them had central retinal vein occlusion (CRVO) and 33 (55%) had branch retinal vein occlusion (BRVO). Forty percent of patients with RVO had PACD. Relative risk of PACD was 1.71 times in patients with CRVO as compared to BRVO. Risk of glaucoma was 49% more in CRVO than BRVO. Probability of PACD was more in patients of RVO who had diabetes and CAD as comorbidity. Conclusion: The association between PACD and RVO is less known. PACD can be one of the risk factors for the development of RVO. A comprehensive examination and detailed angle evaluation of both of the eyes should be done in all cases of RVO, in addition to investigating for systemic risk factors. However, larger population-based studies would be required to prove it as an independent risk factor.

Clinical and molecular aspects of congenital aniridia - A review of current concepts.

Congenital aniridia is a pan ocular disorder characterized by partial or total loss of iris tissue as the defining feature. Classic aniridia, however, has a spectrum of ocular findings, including foveal hypoplasia, optic nerve hypoplasia, nystagmus, late-onset cataract, glaucoma, and keratopathy. The latter three are reasons for further visual compromise in such patients. This entity is often due to mutations in the PAX6 (Paired box protein Pax-6) gene. Recently, aniridia-like phenotypes have been reported due to non-PAX6 mutations as in PITX2, FOXC1, FOXD3, TRIM44, and CYP1B1 as well wherein there is an overlap of aniridia, such as iris defects with congenital glaucoma or anterior segment dysgenesis. In this review, we describe the various clinical features of classic aniridia, the comorbidities and their management, the mutation spectrum of the genes involved, genotype-phenotype correlation of PAX6 and non-PAX6 mutations, and the genetic testing plan. The various systemic associations and their implications in screening and genetic testing have been discussed. Finally, the future course of aniridia treatment in the form of drugs (such as ataluren) and targeted gene therapy has been discussed.

Anterior segment dysgenesis: Insights into the genetics and pathogenesis.

Childhood glaucoma is a treatable cause of blindness, provided it is recognized, diagnosed, and treated in time. WHO has estimated that it is responsible for Blind Years second only to cataracts. The fundamental pathophysiology of all childhood glaucoma is impaired outflow through the trabecular meshwork. Anterior segment Dysgeneses (ASD) are a group of non-acquired ocular anomalies associated with glaucoma, characterized by developmental abnormalities of the tissues of the anterior segment. The cause is multifactorial, and many genes are involved in the development of the anterior segment. Over the last decade, molecular and developmental genetic research has transformed our understanding of the molecular basis of ASD and the developmental mechanisms underlying these conditions. Identifying the genetic changes underlying ASD has gradually led to the recognition that some of these conditions may be parts of a disease spectrum. The characterization of genes responsible for glaucoma is the critical first step toward developing diagnostic and screening tests, which could identify individuals at risk for disease before irreversible optic nerve damage occurs. It is also crucial for genetic counseling and risk stratification of later pregnancies. It also aids pre-natal testing by various methods allowing for effective genetic counseling. This review will summarize the known genetic variants associated with phenotypes of ASD and the possible significance and utility of genetic testing in the clinic.

A deep dive into the latest European Glaucoma Society and Asia-Pacific Glaucoma Society guidelines and their relevance to India.

Glaucoma is the second leading cause of blindness in India. Despite advances in diagnosing and managing glaucoma, there is a lack of India-specific clinical guidelines on glaucoma. Ophthalmologists often refer to the European Glaucoma Society (EGS) and Asia-Pacific Glaucoma Society (APGS) guidelines. A group of glaucoma experts was convened to review the recently released EGS guideline (fifth edition) and the APGS guideline and explore their relevance to the Indian context. This review provides the salient features of EGS and APGS guidelines and their utility in Indian scenario. Glaucoma diagnosis should be based on visual acuity and refractive errors, slit-lamp examination, gonioscopy, tonometry, visual field (VF) testing, and clinical assessment of optic nerve head, retinal nerve fiber layer (RNFL), and macula. The intraocular pressure target must be individualized to the eye and revised at every visit. Prostaglandin analogues are the most effective medications and are recommended as the first choice in open-angle glaucoma (OAG). In patients with cataract and primary angle-closure glaucoma (PACG), phacoemulsification alone or combined phacoemulsification and glaucoma surgery are recommended. Trabeculectomy augmented with antifibrotic agents is recommended as the initial surgical treatment for OAG. Laser peripheral iridotomy and surgery in combination with medical treatment should be considered in high-risk individuals aged <50 years. In patients with phakic and PACG, phacoemulsification alone or combined phacoemulsification and glaucoma surgery are recommended. Visual acuity, VF testing, clinical assessment of the optic disc and RNFL, and tonometry are strongly recommended for monitoring glaucoma progression.

Correlation between choroidal thickness and intraocular pressure control in primary angle-closure glaucoma.

PURPOSE: To study the correlation between choroidal thickness (CT) and IOP control in primary angle-closure glaucoma (PACG). METHODS: In total, 61 patients (102 eyes) with PACG underwent subfoveal CT (SFCT) scanning using enhanced depth imaging-optical coherence tomography. The subjects with PACG were further grouped as controlled IOP (≤21 mm Hg on maximal medical therapy) and uncontrolled IOP (>21 mm Hg on maximal medical therapy). The average CT of the PACG eyes was calculated and compared between both groups. A correlation analysis was done between CT and intereye difference in CT with the disease parameters. RESULTS: The mean CT was 274.38 ± 42.10 µm in 102 PACG eyes. SFCT was significantly increased in the uncontrolled IOP group as compared with the controlled IOP group. The mean SFCT was 245.57 ± 62.10 µm in the controlled group and 294.46 ± 51.05 µm in the uncontrolled group (P < 0.01). Factors associated with a thicker choroid were younger age, high IOP, and higher optic nerve head cupping (P < 0.001). Neither the visual field-mean deviation (VF-MD) nor pattern standard deviation (PSD) was found to be associated with overall CT. The intereye asymmetry between CT was significantly associated with poor VF-MD and PSD. CONCLUSION: PACG eyes with thicker choroid may be a risk factor for poor IOP control on medical anti-glaucoma therapy. Thicker choroid as compared to the fellow eye is a poor prognostic sign and these eyes should be monitored closely.

Bilateral multiple iridociliary cysts causing secondary pigment dispersion.

Primary epithelial iris cysts are benign innocuous lesions, which are mostly bilateral and asymptomatic in nature. They can lead to creeping angle closure, when the angle progressively narrows due to age-associated changes. This photoessay, herewith, signifies the dual importance of identifying minute bumpy elevations in the iris by a thorough slit lamp examination as well as the use of ultrasound biomicroscopy in patients presenting with creeping angle closure, frequently misdiagnosed in the primary angle closure disease spectrum.