Demographics
A total of 105 patients (181 eyes), were seen in Ophthalmology Department of JMC during the 3-year study period. Majority, 72.4%, of patients had bilateral ocular involvement. There were 62.9% (n = 66) males and 37.1% (n = 39) girls, with a mean age of 8.3 years at the time of diagnosis (SD, 6.6 years; median, 7 years).
Gender and Age
Males presented with PCG at a higher rate (73.8%) than females (Table 1). The mean age at presentation for PCG patients was 2.7 years, with a SD of 3.1 years (median 1.2). While, the mean age at presentation for patients with secondary glaucoma was 8.8 years, with a SD of 4.7 years (median 9.1) (Table 2).
Family History of Childhood-onset Glaucoma and Consanguinity
A family history of childhood-onset glaucoma was found in approximately 3% of all patients. However, for most patients’ the history of consanguinity was unknown (Table 1).
Diagnosis
PCG was the most common (n = 76 eyes, 42.0%) type childhood glaucoma, followed by glaucoma suspect (n = 40, 22.1%) and JOAG (n = 28, 15.5%) (Table 4). Glaucoma suspect was diagnosed in 26 (65%) eyes by suspicious optic nerve cupping and in 14 (35%) eyes by IOP > 21 mmHg. Steroid-induced glaucoma was the most common cause of secondary childhood glaucoma, accounting for 9 (5.0%) of all cases, followed by glaucoma following cataract surgery in 8 (4.4%) eyes.
Baseline Visit Clinical Assessment
Visual Acuity
Baseline visit quantitative VA was recorded in 63.0% (n = 114 eyes) of all childhood glaucoma eyes (n=181) and in only 22.4% of PCG eyes (considering age at diagnosis). The overall mean VA of the 114 eyes that had a quantitative VA at the baseline visit was 0.92 logMAR (SD, 1.06) (Table 3).
Intraocular Pressure
Of 181 eyes with childhood glaucoma or glaucoma suspect IOP was recorded for 157 eyes. Of 76 eyes with PCG, 57 (75%) of them have baseline IOP measurements. For 9 (15.8%) of them the measurement was taken during an EUA under ketamine anesthesia. The mean IOP for all eyes was 34.6±13.4 mmHg, and 41.3±9.8 mmHg for PCG (Table 3).
Management
Primary Congenital Glaucoma
In this study PCG was the most common type of childhood glaucoma (76 of 181 eyes [42.0%]), its management analyzed in detail. Majority of PCG eyes (71/76, 93.4%) were treated surgically, and 6.6% received medical treatment only (n = 5 eyes) (Table 5). Among the eyes that underwent surgery, 94.4% (67/71) had anti-glaucoma medication prior to surgery, and on average, 2.04 classes of medication were prescribed (range, 1-5). The most commonly prescribed medication was a beta-blocker, followed by oral carbonic anhydrase inhibitors (CAIs), topical CAIs, and prostaglandin analogues.
Surgery
Surgery was carried out within 7 days of the baseline visit in 52.1% (n = 37 eyes), 14 days in 81.7% (n = 58 eyes), and 28 days in 90.1% (n = 64 eyes). Overall, it took 18.0 days on average from the baseline visit to surgery (SD, 30.5 days; median, 7 days) (Table 5).
Of the PCG eyes that had surgery, 67.6% had only one glaucoma surgery. Of the 32.4% of eyes that had more than one glaucoma surgery, 29.6% had two operations, 1.4% had three, and 1.4% had four (Table 5).
The initial surgical procedure of choice for PCG eyes that underwent surgery was combined trabeculotomy-trabeculectomy (CTT) in 43.7% (31/71 eyes), followed by trabeculectomy 32.4% (23/71), goniotomy 22.5% (16/71), and trans-scleral cyclophotocoagulation (CPC). Primary glaucoma drainage device (GDD) surgery and trabeculotomy were not the first-line surgery for any PCG eyes (Fig 1).
Of 71 PCG eyes treated surgically, 23 eyes underwent more than 1 surgery (26 procedures), most underwent CTT (n = 12, 52.1%), followed by trabeculectomy (n = 6, 26.1%), GDD (n = 4, 17.4%), CPC (n = 3, 13.0%), and goniotomy (n = 1, 4.3%) (Fig 2).
Glaucoma Suspect
There were 39 (22.1%) eyes with glaucoma suspect, majority of them 28 (70.0% of total childhood glaucoma eyes) had no treatment. Medication was given in 30.0% (n = 12), and goniotomy was performed for one eye (Table 6).
Secondary Childhood Glaucoma
Thirty-seven (20.4%) eyes had secondary childhood glaucoma, of which who had treatment record, all of them were treated medically (Table 5).
Juvenile Open-Angle Glaucoma
Twenty-eight (15.5%) eyes had JOAG, and 19 (67.8%) of those were treated medically. Surgery was performed in 20.0% (n = 7) (Table 5).
Final Visit Clinical Assessment
Of the 181 eyes with childhood glaucoma, 111 (61.3%) eyes had final visit quantitative VA and 130 (71.8%) eyes IOP records (Table 3).
Visual Acuity
Final visit quantitative VA was recorded in 61.3% (n = 111 eyes) of all childhood glaucoma eyes (n=181) and in only 23.7% of PCG eyes (considering age at diagnosis). The overall mean VA of the 111 eyes that had a quantitative VA at the final visit was 0.94 logMAR (SD, 1.11) (Table 3).
Intraocular Pressure
IOP was successfully controlled in 85.4% (111/130) of the eyes at the final visit, with 43.8% of complete and 41.5% of qualified success (Table 6). In contrast, 14.6% of failed cases had IOPs greater than 21 mmHg.
Of the 57 PCG eyes who had final visit IOP record, 78.9% of them had successful IOP control (complete 64.9% and qualified 14.0%). While IOP >21 mmHg was present in 21.1% of the failed eyes.
For JOAG, 91.3% (21/23 eyes) had successful control (complete 21.7% and qualified 69.6%). For eyes with secondary glaucoma, 83.9% (25/29) had successful IOP control (complete 3.2% and qualified 80.6%). While all of glaucoma suspect eyes had a successful IOP control (complete 73.7% and qualified 26.3%) (Table 6).