Long-term outcomes in Primary congenital glaucoma, aniridia and anterior segment dysgenesis

Aim To determine the long-term outcomes of a cohort of complex patients with primary congenital glaucoma, aniridia and anterior segment dysgenesis. Methods Retrospective consecutive series between 1990–2021 in two UK tertiary centres: Guy's and St Thomas’ NHS Foundation Trust and King's College Hospital NHS Foundation Trust. We recorded the number and types of surgical and laser treatments along with preoperative and postoperative data, including intraocular pressures (IOP) and anti-glaucoma medications. Results A total of 41 eyes of 21 patients were included. Primary diagnoses were primary congenital glaucoma in 16 eyes (39.0%), aniridia in 14 eyes (34.2%), and anterior segment dysgenesis in 8 eyes (19.5%). Sixteen eyes (39.0%) had one or more glaucoma surgery or laser procedures for advanced glaucoma, and the long-term follow-up was 12.8 ± 3.6 years. There was a significant decrease in postoperative IOP (mmHg) at 3 months (16.5 ± 1.6; p = 0.0067), 6 months (18.7 ± 2.1; p = 0.0386), 12 months (18.6 ± 1.7; p = 0.0229), 3 years (14.7 ± 1.2; p = 0.0126), 5 years (15.5 ± 1.8; p = 0.0330) and 10 years (15.4 ± 2.3; p = 0.7780), compared to preoperatively (24.1 ± 2.6). Surgical success (complete and qualified) was 62.5%, 50.0%, 43.8%, 46.2%, 45.5% and 28.6% at 3 months, 6 months, 12 months, 3 years, 5 years and 10 years, respectively. There was no significant change in the number of anti-glaucoma drugs postoperatively ( p > 0.05). Four eyes (25.0%) had postoperative complications (hyphaema, hypotony) that resolved after conservative management. Conclusions Surgical management of these complex eyes with advanced glaucoma is challenging. Overall, the cohort had good surgical outcomes with a significant decrease in IOP by 36.1% after long-term follow-up.

Choroidal Thickness in Advanced Glaucoma Patients

Purpose: We investigated choroidal thickness according to the classification of glaucoma and related factors in patients with advanced glaucoma.Methods: The present study included 133 eyes of 133 advanced glaucoma patients with a mean deviation of <-12 dB. Patients were classified into primary open angle glaucoma (POAG) and normal-tension glaucoma (NTG) groups. Factors related to the subfoveal and peripapillary choroidal thickness were analyzed using linear regression analysis.Results: The mean peripapillary choroid thickness was 99.20 ± 46.85 µm in the NTG group, which was significantly thinner than in the POAG group (121.85 ± 45.39 µm, p = 0.006). Additionally, in the sectoral comparison, the NTG group had thinner choroids than the POAG group in all areas (p < 0.05 for all). In the linear regression analysis, glaucoma class (p = 0.007), age (p = 0.005), and intraocular pressure (IOP) (p = 0.024) significantly affected the peripapillary choroid thickness. Moreover, age (p = 0.029) and macular thickness (p = 0.002) were significantly associated with subfoveal choroid thickness.Conclusions: In advanced glaucoma, low baseline IOP, NTG, and old age were significantly associated with a thin peripapillary choroid, suggesting an association between thin peripapillary choroid and the etiology of NTG. Further studies are needed to clarify the significance of a thin choroid in the pathogenesis of glaucoma.

Path to Diagnosis and Clinical Characteristics of Advanced Glaucoma at Initial Diagnosis: a Tertiary Single Center Experience

Purpose: As routine health examinations become more common, many patients first diagnosed with glaucoma have advanced glaucoma. We analyzed the routes to diagnosis and the characteristics of patients initially diagnosed with advanced glaucoma.Methods: We retrospectively retrieved the medical records of patients first diagnosed with advanced glaucoma in our tertiary care center. The inclusion criteria were a mean deviation (MD) less than -12 dB on the visual field test, accompanied by structural damage. All patients were classified in terms of unilateral/bilateral disease, the intraocular pressure before medication, and lens status. We divided patients into those with monocular or binocular advanced glaucoma, high- or normal-pressure glaucoma, and those who were pseudophakic or phakic.Results: We included 73 patients of mean age 69.3 years. The visual field test MD was -19.6 dB. In those with binocular advanced glaucoma, incidental ophthalmic examination was the most common means of diagnosis (52.2%). Central-island visual field defects were the most common defects (54.2%). In those with monocular advanced glaucoma, glaucoma-associated symptoms most commonly triggered diagnosis (46.9%). Both superior and inferiorvisual field defects were the most common defects (42.8%). Glaucoma-associated symptoms were present in 68.2 and 22.8% of patients with high- and normal-pressure glaucoma, respectively. Central-island visual field defects were present in 43.6 and 29.4% of those with high- and normal-pressure glaucoma, respectively.Conclusions: We analyzed the routes to diagnosis and the clinical characteristics of patients with advanced glaucoma. In those with binocular disease, glaucoma was most commonly diagnosed on incidental ophthalmic examination. Central-island visual field defects were the most common defects in patients with binocular and high-pressure glaucoma, and the pseudophakic group. A multi-center longitudinal study on risk factors for delayed glaucoma diagnosis is needed.

The Impact of Superficial Vessel Density on Glaucoma Progression according to the Stage of Glaucoma

Purpose: To investigate the clinical significance of vessel density (VD) on visual field (VF) progression regarding the severity of glaucoma. Methods: A total of 130 eyes were recruited in this retrospective and longitudinal study. Superficial and deep VDs in circumpapillary and macular regions were measured via ImageJ. The rate of VF progression was defined as the mean deviation (MD) slope (dB/year). Linear regression was used to verify factors affecting deterioration of VF. The eyes with lower superficial VD were further analyzed. Results: Fifty patients with early glaucoma (EG) (MD > −6 dB) and 52 patients with moderate-to-advanced glaucoma (MAG) (MD ≤ −6 dB) were included. A faster progression rate was found in MAG (p = 0.049). Superficial VD was noticeably related to the VF progression rate in total eyes and in MAG (Both Ps ≤ 0.007, respectively). With patients in the lower half of the superficial VD, the VD was significantly associated with the rate of progression (B, 0.049, p = 0.021). This association was independent of the baseline MD and OCT parameters. Conclusion: Decreased superficial VD might conversely affect the progression of glaucoma even in MAG, which suggests superficial VD could be used as a potential marker to foresee the disease progression even in progressed eyes.

Role of minimally invasive glaucoma surgery in the management of chronic open-angle glaucoma

To compare the safety and efficacy of phacoemulsification combined with ab-interno trabeculectomy (Trabectome) and phacoemulsification combined with I-Stent inject in patients with medically uncontrolled primary open-angle glaucoma (POAG). A retrospective comparative case series. 70 eyes of 66 patients completed 2 years follow up after these treatments performed in 2017–2018. 35 eyes of 33 patients underwent combined Phaco-Trabectome (PT); and 35 eyes of 33 patients underwent combined Phaco-I-Stent inject (Pi). Patient demographics and preoperative characteristics are comparable. A 20% drop in IOP was achieved in 27 eyes (77.14%) in PT group and 28 eyes (80%) in Pi group (p = 0.77). Success rate (target IOP achieved and maintained for 2 years) in advance glaucoma was 25% in PT group and 30.7% in Pi group (p = 0.90). In mild to moderate glaucoma, success rate was 85.71% in PT group and 90% in Pi group (p = 0.67). There was no significant difference between two groups with regards to mean reduction in glaucoma medications and complication rates. Trabectome and I-Stent combined with phacoemulsification are equally efficacious and safe for treating patients with medically uncontrolled mild and moderate primary open-angle glaucoma (POAG). However, they are not an effective treatment for patients with advanced glaucoma.

Atypical presentations of non-familial anterior megalophthalmos: a rare disease

Anterior megalophthalmos usually presents early in life with megalocornea, deep anterior chamber, raised intraocular pressure, glaucomatous optic nerve damage and iridodonesis/stromal thinning with positive family history. We report atypical features and presentations in two patients (four eyes) with non-familial megalophthalmos. While the first patient, a male, presented at 51 years of age with megalocornea, cataract, phacodonesis, normal pupillary dilatation/normal iris and advanced glaucoma, the second patient presented with iridodonesis with stromal thinning, aphakia and advanced glaucoma. The family history was negative in both patients. The vitreous index was unusually high, >70% in all four eyes, owing to aphakia in the second patient and possible late presentation/variant phenotype in the first patient. Thus, atypical features such as greater vitreous length, absent iris involvement and late-onset adult presentation are common in non-familial anterior megalophthalmos. Clinical surprises due to varied phenotypes should be kept in mind in such cases.

Slowed Saccadic Reaction Times in Seemingly Normal Parts of Glaucomatous Visual Fields

Purpose: In eye movement perimetry, peripheral stimuli are confirmed by goal-directed eye movements toward the stimulus. The saccadic reaction time (SRT) is regarded as an index of visual field responsiveness, whereas in standard automated perimetry (SAP), the visual field sensitivity is tested. We investigated the relation between visual field sensitivity and responsiveness in corresponding locations of the visual field in healthy controls and in patients with mild, moderate and advanced glaucoma.Materials and Methods: Thirty-four healthy control subjects and 42 glaucoma patients underwent a 54-point protocol in eye movement perimetry (EMP) and a 24-2 SITA standard protocol in a Humphrey Field Analyzer. The visual field points were stratified by total deviation sensitivity loss in SAP into 6 strata. A generalized linear mixed model was applied to determine the influence of the various factors.Results: The generalized linear mixed model showed that the mean SRT increased with increasing glaucoma severity, from 479 ms in the control eyes to 678 ms in the eyes of patients with advanced glaucoma (p &lt; 0.001). Mean SRTs significantly increased with increasing SAP sensitivity loss. Even at the locations where no sensitivity loss was detected by SAP (total deviation values greater or equal than 0 dB), we found lengthened SRTs in mild, moderate and advanced glaucoma compared to healthy controls (p &lt; 0.05) and in moderate and advanced glaucoma compared to mild glaucoma (p &lt; 0.05). At locations with total deviation values between 0 and −3 dB, −3 and −6 dB and −6 and −12 dB, we found similar differences.Conclusions: The lengthened SRT in areas with normal retinal sensitivities in glaucomatous eyes, i.e., planning and execution of saccades to specific locations, precede altered sensory perception as assessed with SAP. Better understanding of altered sensory processing in glaucoma might allow earlier diagnosis of emerging glaucoma.