Treatment Outcomes of Primary Combined Trabeculotomy With Trabeculectomy in Early Onset Glaucoma With Sturge-Weber Syndrome.

PRCIS: In eyes with Sturge-Weber syndrome (SWS) with early onset glaucoma, primary combined trabeculotomy with trabeculectomy (CTT) had a good intermediate-term success rate and minimal postoperative complications. DESIGN: Retrospective cohort study. OBJECTIVE: To report the intermediate-term outcomes of primary CTT in early onset glaucoma with SWS. PATIENTS AND METHODS: This study included 49 eyes of 49 children (1997-2020) with SWS and early onset glaucoma who underwent primary CTT with at least 1-year postoperative follow-up. Success was defined as complete when intraocular pressure was >5 mm Hg and ≤16 mm Hg under general anesthesia or ≤21 mm Hg in the clinic without antiglaucoma medications (AGMs) and as qualified with AGM. RESULTS: The median age (interquartile range) at CTT was 0.58 (0.19, 8.3) years. The median postoperative follow-up was 4.7 years (2.5, 9). The majority were male children (31/49, 63%). Epilepsy was noted in 7 (14.3%) and diffuse choroidal hemangioma in 17 children (35%). At diagnosis, 37 eyes(75%), had corneal edema, and the mean (±SD) horizontal corneal diameter was 12.8 ± 0.7 mm. Postoperatively, the median intraocular pressure decreased from 26 (22, 30) mm Hg to 16 (12, 20) mm Hg ( P < 0.0001) and the median number of AGM reduced from 1 (0,1) to 0 (0,1; P < 0.01). Complete success probability of CTT was 86% (76, 96) at 1 year and 64% (49, 84) at 5 years. Qualified success was 98% (94, 100) at 1 year and 89% (78, 100) at 5 years. A larger cup-to-disk ratio ( P < 0.005) was associated with a higher risk of surgical failure. Postoperative complications were noted in 9 eyes (18%), all resolved with conservative management except one eye with a retinal detachment that ended in phthisis bulbi. CONCLUSION: CTT as a primary procedure showed good long-term efficacy and safety in SWS with early onset glaucoma.

Glaucoma Evaluation and Management in Eyes With Boston Type 1 and Aurolab Keratoprostheses in an Indian Cohort.

PURPOSE: The purpose of this study was to evaluate glaucoma and its management in eyes with Boston type 1 keratoprosthesis (Boston-Kpro) and Aurolab keratoprosthesis (Auro-Kpro) in an Indian cohort. METHODS: This retrospective comparative case series included 175 eyes of 175 patients who underwent type 1 keratoprosthesis from 2009 until 2017; type 1 Boston-Kpro was performed in 107 eyes (107 patients) and Auro-Kpro in 68 eyes (68 patients). The eyes with <1-month follow-up were excluded, and hence, for the analysis of glaucoma, 62 eyes with Auro-Kpro and 99 eyes with Boston-Kpro were included. Progression was defined as the worsening of optic disc damage/progression by visual field/uncontrolled IOP needing additional medications or glaucoma surgery. RESULTS: The post-Kpro mean follow-up was 1.8 ± 1.7 years. After Kpro, overall glaucoma was noted in 61% of eyes (98/161). Preexisting glaucoma was noted in 28% of eyes (45/161), postoperative glaucoma was noted in 33% of eyes (53/161), and 39% of eyes (63/161) did not have glaucoma. Among those with preexisting glaucoma, 49% of eyes (22/45) progressed after Kpro implantation. The proportion of eyes with glaucoma in Auro-Kpro (56.5% of eyes) and Boston-Kpro (63.6% of eyes) was similar (P = 0.46). The Kaplan-Meier curve for cumulative survival (Kpro-retention rate) was similar in eyes with and without glaucoma (83% vs. 76% at 1 year, 72% vs. 70% at 2 years; P = 0.65). A higher number of prior intraocular surgeries (P = 0.04) was significantly associated with risk for postoperative glaucoma. CONCLUSIONS: Glaucoma was seen in more than half of the eyes with type 1 keratoprosthesis. The presence of glaucoma did not seem to affect the retention of keratoprosthesis. A high rate of postoperative glaucoma and the progression of preexisting glaucoma need close monitoring and appropriate management.

Visual photosensitivity threshold and objective photosensitivity luminance in healthy human eyes assessed using an automated ocular photosensitivity analyser: a step towards translation of a clinical tool for assessing photophobia.

PURPOSE: To evaluate the visual photosensitivity threshold and objective photosensitivity luminance in healthy eyes, thereby providing a normative dataset that will lead to a better understanding of diseases causing photophobia. METHODS: This was a prospective cross-sectional study. Emmetropes whose visual acuity was better than 0.18 logMAR (6/9) with no other ocular abnormality were included. Headache Impact Test-6 and visual light sensitivity questionnaires were administered. Visual photosensitivity threshold was measured subjectively using the ocular photosensitivity analyser. Objective photosensitivity luminance was assessed manually by evaluating videos recorded using an infrared camera and noting the intensity of light at the first squeezing reflex. RESULTS: Seventy five normal subjects (age range, 7-71 years) were included. Median age was 32.7 years (inter-quartile range, 20.3-47.9 years). Forty (53.3%) were males. Median Headache Impact Test score was 38 (inter-quartile range, 36-42) and visual light sensitivity questionnaire score was 11 (inter-quartile range, 8-15). Mean (standard deviation) right eye, left eye and binocular visual photosensitivity threshold was 3.34 (0.78), 3.33 (0.81) and 3.37 (0.78) loglux, respectively. There was a significant negative correlation of visual light sensitivity questionnaire scores with right eye, left eye and binocular visual photosensitivity thresholds, and positive correlation of age with binocular visual photosensitivity thresholds. Mean (standard deviation) right eye, left eye and binocular objective photosensitivity luminance was 3.25 (0.55), 3.35 (0.47) and 3.15 (0.52) loglux, respectively. Age was only positively correlated with binocular objective photosensitivity luminance, and there was no correlation between age and right eye or left eye objective photosensitivity luminance. CONCLUSIONS: The study characterised, for the first time, objective photosensitivity luminance and established normative data for both visual photosensitivity threshold and objective photosensitivity luminance. The data will help in understanding the pathophysiology of diseases causing photophobia, monitoring the disease progression and evaluating treatment modalities.

Outcomes of keratoplasty in a cohort of Indian patients with xeroderma pigmentosum.

Purpose: To evaluate the outcomes of keratoplasty for xeroderma pigmentosum (XP) performed at a tertiary eye care center. Methods: A retrospective review of medical records of those patients who were clinically diagnosed to have XP (54 eyes of 36 patients) and underwent keratoplasty; either deep anterior lamellar keratoplasty (DALK, four eyes), endothelial keratoplasty (EK, eight eyes), or penetrating keratoplasty (PK, 42 eyes) from 1994 to 2018. Results: The median age at surgery was 20.6 years (interquartile range [IQR], 14.6-27.6 years) and 20 (55.6%) were males. Graft failure occurred in 15 eyes (35.7%) in the PK group and two eyes (50%) in the DALK group; none failed in the EK group. The probability of graft survival in the PK group was 97.2% ± 2.7% at 1 year, 74.0% ± 8.0% at 2 years, and 54.8% ± 11.7% at 5 years. In the PK group, 13 eyes needed antiglaucoma medications, 11 eyes developed graft infiltrate, and 13 eyes needed secondary interventions (cataract surgery, excision biopsy, and tarsorrhaphy). In the EK group, three eyes needed secondary interventions (excision biopsy). Median postoperative endothelial cell density at the last follow-up in the PK group was 1214 cells/mm2 (IQR, 623-2277 cells/mm2). Conclusion: Despite the complexities of the ocular surface and adnexal issues in XP, keratoplasty had reasonably good outcomes. More than half of the PK grafts survived 5 years with no failures in the EK group. Regular follow-up and timely management of suture-related infections raised intraocular pressure, and suspicious ocular surface lesions, in addition to solar protection, are important for the success of keratoplasty in these eyes.

Corneal endothelium in unilateral Fuchs heterochromic iridocyclitis.

Purpose: To assess the corneal endothelium in patients with Fuchs heterochromic iridocyclitis (FHI) and compare it with the normal fellow eye. Methods: Retrospective, observational, cross-sectional study of 31 patients seen between Jan 2016 to Dec 2018, with clinical diagnosis of Fuchs heterochromic iridocyclitis, was performed. Specular microscopic examination was documented in both eyes. The affected eyes formed the study group and the fellow healthy eyes served as controls. Results: The mean age of the patients was 29.9 ± 8.2 years. The endothelial cell density (P = 0.0001) was significantly lower, whereas average cell size (P = 0.0001), coefficient of variation (P = 0.004), and maximum cell area (P = 0.01) were significantly higher in the affected eye compared to the control eye. In three patients, the affected eye showed guttae, while the healthy fellow eye revealed a normal specular mosaic. Conclusion: Specular microscopic analysis shows endothelial alterations in the affected eyes in FHI.