Enhanced S-Cone Syndrome Masquerading as TORCH in an Infant and a Toddler.

PURPOSE: To report two cases masquerading as TORCH but eventually diagnosed with Enhanced S-cone Syndrome (ESCS). METHODS: Descriptive case report. RESULTS: Case 1: A ten-month-old boy presented with high hypermetropia, strabismus and bilateral chorioretinal pigmented scars with a history of cat scratch of his mother during pregnancy. He was treated for suspected toxoplasma retinitis. Choroidal neovascular membranes (CNV) were diagnosed bilaterally and treated with intravitreal bevacizumab. Genetic testing showed homozygote mutation in NR2E3 gene. Case 2: A two-year old girl presented with bilateral high hypermetropia and strabismus. Funduscopy revealed extrafoveal chorioretinal lesions and surrounding subretinal fibrosis. An elevated titer of anti-toxocara IgG antibodies was detected and managed accordingly. LE CNV was diagnosed and treated with intravitreal bevacizumab. Genetic testing disclosed homozygote mutation in NR2E3. CONCLUSION: Ocular manifestations in ESCS can be reminiscent to TORCH. CNV may develop with an incidence of 15%. We report the youngest patient with ESCS-associated CNV.

Hyperopic LASIK Enhanced by Pharmacological Treatment of Presbyopia.

Purpose: Regression of the refractive outcome is a major concern of LASIK procedures mainly in presbyopic patients. The purpose of this study was to evaluate the long-term efficacy of the pharmacological treatment of presbyopia performed with Benozzi's method, in combination with hyperopic LASIK surgery in presbyopic population. Methods: A nonrandomized case series was developed, including presbyopic patients who underwent bilateral "Hyperopic LASIK surgery" and were pharmacologically treated with Benozzi's Method from January 2011 to August 2018, with at least 2 years of follow-up, at two private ophthalmological clinics of Argentina. Main outcomes were spherical equivalent (SE), uncorrected distance visual acuity (UDVA), and uncorrected near visual acuity (UNVA). Measurements were evaluated at baseline and postoperative at 1 month (without Benozzi's treatment), 2 months (starting with Benozzi's treatment), and 2 years. The SE stability across the time was statistically compared. Results: A total of 84 eyes of 42 patients, with a mean age at the time of the surgery of 51.07 ± 4.5 (42-59), were found following 2 years of follow-up. Patients have improved and maintained UDVA, achieving Jaeger 1 in the second postoperative month, which was maintained up to the last year of follow-up. Refractive stability across the time is observed comparing first month after surgery with the last year of follow-up, without statistical significant difference (p: 0.11). Conclusion: Hyperopic presbyopic patients that underwent LASIK surgery and 1 month after surgery started with the pharmacological treatment of presbyopia (Benozzi's method) results in excellent UNVA and UDVA that is stable over time without refractive regression.

Refractive Changes After Corneal Stromal Filler Injection for the Correction of Hyperopia.

PURPOSE: To evaluate a new non-ablative and adjustable procedure for laser ablative refractive corneal surgery in hyperopia using the injection of a biocompatible liquid filler material into a stromal pocket. METHODS: A total of 120 stromal pockets were created using a clinical femtosecond laser system in 96 rabbit corneoscleral discs and 24 whole globes. Pockets were cut at a depth of 120 or 250 µm below the epithelial surface. Hyaluronic acid was injected manually into the pocket. To determine the refractive changes, three-dimensional optical coherence tomography images and a specifically developed picture recognition Matlab (The Mathworks) routine were used. RESULTS: After injection, a steepening of the anterior and flattening of the posterior corneal surface was observed, which led to hyperopic correction. The two main factors determining the amount of correction were the pocket depth and the injected volume. After the pocket was homogeneously filled, an initial refractive increase was observed, followed by a linear relation between the injected volume and the refraction increase. CONCLUSIONS: This possible clinical protocol for controlled refraction correction of hyperopia suggests a potential readjustable clinical application. [J Refract Surg. 2020;36(6):406-414.].

Topical Estrogen Therapy for Hyperopia Correction in Vivo.

Purpose: In vitro studies found that 17ß-estradiol (estrogen) modulates corneal biomechanical properties and reduces tissue stiffness. Therefore we hypothesized that topical estrogen might affect the refractive properties of the cornea, inducing a myopic shift. Methods: Twelve female New Zealand white rabbits 16 weeks old were used. The rabbits were randomly divided to either the treatment group receiving 1.5% (w/v) estrogen eye drops or a control group receiving vehicle only (n = 6 each group). Both groups were given drops (50 µL) to the right eye every 12 hours for 35 days. Ocular examination, pachymetry, intraocular pressure (IOP), keratometry ,and refraction were evaluated at baseline and on a weekly basis. Results: No significant differences were observed between the two groups at baseline in all outcome measures. Both groups displayed corneal flattening and a hyperopic shift. However, the change rate was slower in the treatment group. Repeated measurements analysis revealed a statistically significant difference in keratometry readings between groups (P = 0.034) with steeper keratometry by up to 0.6 diopters in the treatment group. The difference between the two groups diminished and became statistically insignificant after treatment cessation. No significant changes were observed in IOP and pachymetry throughout the study period. No side effects were observed in either group. Conclusions: Estrogen eye drops induced a myopic shift in keratometry readings. These results suggest that corneal refractive power might be manipulated pharmacologically. Further studies on the physiology behind this change are warranted to facilitate a pathway for development of novel pharmacologic treatments to correct refractive errors.

Photorefractive intrastromal crosslinking for correction of hyperopia: 12-month results.

PURPOSE: To evaluate the efficacy of epithelium-on photorefractive intrastromal crosslinking (PiXL), a noninvasive procedure to reduce refractive error in patients with low hyperopia. SETTING: Gemini Eye Clinic, Zlin, Czech Republic. DESIGN: Prospective single-center study. METHODS: Twenty-two low hyperopic eyes were enrolled and underwent PiXL treatment according to a standardized treatment protocol. Visual acuity, subjective distance refraction, keratometry, topography, pachymetry, subjective discomfort, and endothelial cell density (ECD) were recorded during 12-month follow-up. RESULTS: In 22 eyes, the median manifest refraction decreased significantly (P < .0001) from +0.75 diopters (D) (interquartile range [IQR], +0.63 to +1.06 D), median and IQR) diopters (D) preoperatively to +0.25 D (IQR, 0.0 to +0.50 D) at 12-month follow-up and remained stable. Seventy-seven percent of eyes achieved refraction within ± 0.50 D of emmetropia by 1 month postoperatively and was stable through 12-month follow-up. ECD was stable and did not show significant changes. There was low incidence of postoperative pain and dry eye. CONCLUSIONS: PiXL is a promising alternative to conventional laser refractive surgeries for low hyperopia. Further studies are warranted to optimize treatment parameters for a wider range of refractive errors and to evaluate the potential to improve precision.

Effects of intravitreal insulin and insulin signaling cascade inhibitors on emmetropization in the chick.

PURPOSE: Intravitreal insulin has been shown to be a powerful stimulator of myopia in chickens, in particular if the retinal image is degraded or defocused. In most tissues, the insulin receptor activates two main signaling pathways: a) the mitogen-activated protein kinase (MAPK) cascade (e.g., mitogen-activated protein kinasem kinase [MEK] and extracellular regulated kinase [ERK]) and b) the phosphatidylinositol 3-kinase (PI3K)/protein kinase B (Akt) pathway. In the current study, insulin was injected, and these pathways were separately inhibited to determine which is activated when the retinal image is defocused by spectacle lenses. METHODS: Chicks were treated with either +7 D, -7 D, or no lenses. They were intravitreally injected with insulin, the MEK inhibitor U0126, the PI3K inhibitor Ly294002, or a combination of insulin and one of the inhibitors. Refractions and ocular dimension were measured at the beginning and after four days of treatment. The retinal proteins of the chicks were measured with western blots after 2 h and four days of treatment. Incubation occurred with anti-Akt1, anti-Erk1/2, anti-phospho-Akt(Thr308), and anti-phospho-Erk1/2((Thr202/Tyr204)) antibodies, and the ratio between the relative intensity of the phospho-form and the total-form was calculated. RESULTS: Chicks wearing positive lenses and injected with saline and with PI3K inhibitor compensated for the imposed defocus and became hyperopic. Insulin injections and insulin plus PI3K inhibitor injections prevented lens-induced hyperopia, whereas the MEK inhibitor alone and insulin plus MEK inhibitor had no effect. Obviously, the MEK inhibitor suppressed the effect of insulin on eye growth in the plus lens-treated animals. Chicks treated with negative lenses and injected with insulin, or with insulin plus MEK inhibitor, overcompensated for the imposed defocus. This effect of insulin was not detected in eyes injected with PI3K inhibitor plus insulin, suggesting that the PI3K inhibitor suppressed the effects of insulin in minus lens-treated animals. Insulin increased the ratio of phospho-Akt/total-Akt in animals with normal visual exposure but even more so in chicks wearing plus or minus lenses. The increase was blocked by simultaneous PI3K inhibitor injections in control eyes but not in lens-treated eyes. Insulin also increased the ratio of phospho-ERK/total-ERK in animals with normal visual exposure and in animals wearing positive lenses, compared to U0126- and Ly294002-injected eyes. In contrast, no significant activation of the MEK/ERK pathway was observed in the negative lens-treated animals. CONCLUSIONS: Intravitreal insulin promoted axial eye growth and stimulated both signaling pathways. The PI3K/Akt pathway was activated in control and plus and minus lens-treated eyes, but the MEK/ERK pathway was activated only with positive lenses or no lenses. With negative lenses, insulin did not stimulate the MEK/ERK signaling cascade. Independent of the pathway stimulated after insulin binding, the effect on insulin was always the same: an increase in eye growth.

Topography-guided hyperopic LASIK with and without high irradiance collagen cross-linking: initial comparative clinical findings in a contralateral eye study of 34 consecutive patients.

PURPOSE: To evaluate the safety and efficacy of intrastromally applied collagen cross-linking (CXL) in a comparative contralateral eye study of topography-guided femtosecond laser-assisted hyperopic LASIK. METHODS: Thirty-four consecutive patients with hyperopia and hyperopic astigmatism elected to have bilateral topography-guided LASIK and were randomized to receive a single drop of 0.1% sodium phosphate riboflavin solution under the flap followed by 3-minute exposure of 10 mW/cm2 ultraviolet A (UVA) light with the flap realigned in one eye (CXL group) and no intrastromal CXL in the contralateral eye (no CXL group). All eyes were treated with the WaveLight FS200 femtosecond laser and WaveLight EX500 excimer laser (Alcon Laboratories Inc). Refractive error and keratometric, topographic, and tomographic measurements were evaluated over mean follow-up of 23 months. RESULTS: Preoperatively, mean spherical equivalent refraction was +3.15 +/- 1.46 diopters (D) and +3.40 +/- 1.78 D with a mean cylinder of 1.20 +/- 1.18 D and 1.40 +/- 1.80 D and mean uncorrected distance visual acuity (UDVA) (decimal) of 0.1 +/- 0.26 and 0.1 +/-0.25 in the CXL and no CXL groups, respectively. At 2 years postoperatively, mean spherical equivalent refraction was -0.20 +/- 0.56 D and +0.20 +/- 0.40 D with mean cylinder of 0.65 +/- 0.56 D and 0.76 +/- 0.72 D and mean UDVA of 0.95 +/- 0.15 and 0.85 +/- 0.23 in the CXL and no CXL groups, respectively. Eyes with CXL demonstrated a mean regression from treatment of +0.22 +/- 0.31 D, whereas eyes without CXL showed a statistically significant greater regression of +0.72 +/- 0.19 D (P = .0001). CONCLUSIONS: Topography-guided hyperopic LASIK with or without intrastromal CXL is safe and effective, with greater long-term efficacy (less regression) in eyes with CXL. Our data suggest that the regression seen with hyperopic LASIK may be related to biomechanical changes in corneal shape over time.

Acute hyperopic shift in refraction associated with posterior choroidal detachment following phacoemulsification surgery.

PURPOSE: To describe a patient who presented with hyperopic shift as an initial manifestation of choroidal detachment in the posterior pole following an uneventful phacoemulsification cataract surgery. METHODS: An 82-year-old woman with preexisting diabetes mellitus and hypertension had bilateral primary angle closure glaucoma on maximal tolerated hypotensive medication. An uneventful phacoemulsification surgery using topical anesthesia was performed in her left eye. RESULTS: On the next day, refraction was markedly increased to +7.25 -1.00 × 65 and axial length was reduced from 23.24 mm to 20.13 mm. Funduscopic examination revealed choroidal detachment in the posterior pole without involvement of the peripheral fundus. Axial length increased to 22.19 mm following corticosteroid treatment 1 month later. Six months postoperatively, axial length improved to 22.87 mm with a residual hyperopia of +1.00 -1.00 × 63. CONCLUSIONS: To our knowledge, there have been no reports of choroidal detachment in the posterior pole after phacoemulsification. Acute hyperopic shift following phacoemulsification surgery should lead one to suspect a posterior choroidal detachment. Considering axial length and refractive errors along with fundus examination may contribute to a more accurate follow-up.

Hyperopic LASEK treatments with mitomycin C using the SCHWIND AMARIS.

PURPOSE: To investigate the outcomes of hyperopic laser epithelial keratomileusis (LASEK) with mitomycin C (MMC) using the SCHWIND AMARIS platform. METHODS: Prospective study of 28 eyes of 14 patients. Mean preoperative manifest spherical equivalent refraction was +2.71±0.72 diopters (D) (range: +1.50 to +4.50 D). All eyes underwent LASEK with an Aberration-Free™ algorithm with the SCHWIND AMARIS followed by a 35-second application of MMC with a 6.7-mm optical zone size. RESULTS: At 1 year, mean manifest spherical equivalent refraction was 0.03±0.30 D (range: -0.53 to +0.50 D), with 13 eyes within -0.13 to +0.13 D and all 28 eyes within ±0.50 D. Mean uncorrected distance visual acuity was -0.03±0.09 logMAR, and the efficacy and safety indices were 1, respectively, at 1 year. The postoperative manifest spherical equivalent refraction was stable between 1 and 3 months and 1 year. CONCLUSIONS: Hyperopic LASEK with MMC using the SCHWIND AMARIS provided good outcomes in terms of accuracy, efficacy, safety, and stability.

Photorefractive keratectomy with mitomycin-C for consecutive hyperopia after radial keratotomy.

PURPOSE: To evaluate the safety, efficacy, and stability of excimer laser photorefractive keratectomy (PRK) and mitomycin-C (MMC) 0.02% for consecutive hyperopia after radial keratotomy (RK). METHODS: A prospective, nonrandomized, noncomparative interventional case series of 35 eyes (22 patients) with consecutive hyperopia after RK. All eyes were treated with PRK, using a single intraoperative topical application of MMC 0.02% for 60 seconds. Uncorrected visual acuity, best spectacle-corrected visual acuity, refraction, slit-lamp evidence of corneal haze, and endothelial cell counts were evaluated for up to 18 months after surgery. RESULTS: Postoperative follow-up was 9.6 +/- 5.5 months (ranged from 3 to 18 months). The mean spherical equivalent was +3.36 +/- 1.94 diopters preoperatively and +0.27 +/- 1.38 diopters 12 months after surgery. The uncorrected visual acuity was > or =20/30 in 37.1% of the eyes at 1 month and 78.6% of the eyes at 12 months. At 12 months, 14% of the eyes lost up to 1 line of Snellen acuity in the best spectacle-corrected visual acuity. No corneal haze was observed and the endothelial cell counts remained unchanged postoperatively (P > 0.05). CONCLUSION: PRK with MMC 0.02% for consecutive hyperopia after RK seems to be a safe and effective procedure at least in the short-term period of 6 months.

Hyperopic photorefractive keratectomy with adjunctive topical mitomycin C for refractive error after penetrating keratoplasty for keratoconus.

OBJECTIVE: To present a case of photorefractive keratectomy (PRK) with adjunctive topical mitomycin C (MMC) in an anisometropic hyperopic patient after penetrating keratoplasty (PKP) for keratoconus. METHODS: Interventional case report, chart review, and literature review. RESULTS: A 43-year-old man with a refraction of +7.00 -4.75 x 125 in the right eye underwent PRK 10 months after PKP for keratoconus. The patient had sutures removed for 3 months and was intolerant of contact lenses. After photoablation, 0.02% MMC was applied to the corneal stromal bed. The patient was followed up daily until the epithelium closed and at 1 week, 1 month, 3 months, and 6 months postoperatively. CONCLUSIONS: To our knowledge, this represents the first reported case of the use of MMC to prevent postoperative haze after PRK for PKP in an eye with keratoconus. MMC (0.02%) applied topically to the cornea immediately after PRK is safe and effective to treat a hyperopic refractive error after PKP and prevent postoperative corneal haze formation without the risks of performing a lamellar flap into an ectatic corneal bed.

Ketorolac for the regression of myopic LASIK overcorrection.

OBJECTIVE: To determine whether ketorolac (Acular) treatment and other factors influence regression after LASIK-induced consecutive hyperopia. METHODS: Seventy-two eyes of 51 patients who had undergone LASIK for myopia and compound myopic astigmatism and who experienced consecutive hyperopia of at least +0.50 diopters within the first postoperative week were analyzed. The consenting patients were treated with ketorolac (Acular). Data were collected over a period of 2 months. Primary preoperative variables included age, eye, preoperative manifest and cycloplegic refractions, and pachymetry. Postoperative variables included presence of microstriae and treatment with ketorolac. Treatment success was measured as reduction of consecutive hyperopia. RESULTS: Thirty-seven eyes were treated, and 35 eyes were in the control group. Mean start time for treatment with ketorolac was 9 days after surgery (range 3-35 days). Mean treatment time was 24.5 days (range 10-63 days). Both groups were matched for all preoperative variables except for age, including manifest and cycloplegic refraction, eye treated, and pachymetry. Treated patients were on average 9 years older than the control group. There was no significant difference in the overall rate of regression between the 2 groups at the 1-month and 2-month periods. Thicker preoperative corneas in all eyes had a sporadic association with reduction of consecutive hyperopia. CONCLUSIONS: Ketorolac does not improve consecutive hyperopia after LASIK for myopia and compound myopic astigmatism when compared with a matched control group. Pachymetry appears to be a determining factor in the degree of regression experienced after consecutive hyperopia. This finding warrants further investigation.

Atropine versus cyclopentolate plus tropicamide in esodeviations.

BACKGROUND AND OBJECTIVE: The correction of the total hypermetropic refractive error in the management of esodeviations is well accepted. The choice of the appropriate cycloplegic agent is still not settled. Despite evidence that atropine will more effectively uncover the total refractive error, cyclopentolate in combination with other cycloplegic agents continues to be recommended. This study evaluates the use of atropine compared with a combination of cyclopentolate and tropicamide and analyzes age, size of the refractive error, and size of the esodeviation as possible contributing variables in the response. PATIENTS AND METHODS: The records of 74 patients who underwent refraction with atropine 1% on one occasion and a combination of 1% cyclopentolate and 1% tropicamide on another occasion were reviewed. The mean and range of refractive errors for each eye for each group were determined. Comparisons were made for groups depending on age, size of the refractive error, and size of the esodeviation. RESULTS: The amount of hypermetropia determined with atropine was significantly higher than the amount determined with the combination of cyclopentolate and tropicamide. This difference was significant for three age groups of three groups with increasing amounts of hypermetropia, and two groups with differing amounts of esodeviations. In addition, 11% of right eyes and 19% of left eyes showed 1.25 D or more of hypermetropia with atropine. CONCLUSION: Refraction with 1% atropine ointment yields a significantly larger amount of hypermetropia than does refraction with a combination of cyclopentolate and tropicamide. This difference is statistically significant regardless of age, amount of hypermetropia, or size of the esotropia.

Pilocarpine in the management of overcorrection after radial keratotomy.

BACKGROUND: Induced hyperopia is a potential complication of radial keratotomy with few effective treatments. We report the results of a retrospective study to evaluate the effectiveness of pilocarpine in the treatment of eyes overcorrected by radial keratotomy. METHODS: Sixteen eyes of 14 patients, from a consecutive pool of 200 eyes who underwent radial keratotomy, had hyperopia. The patients were subsequently treated with topical pilocarpine. The patients were treated from 3 to 14 weeks (mean, 8.2 weeks). RESULTS: The mean time of diagnosis of hyperopia was 3 weeks after the surgery (range, 1 to 12 weeks). The mean spherical equivalent of the manifest (fogging) refraction was +1.92 diopters (D) (range, +0.75 D to +5.00 D) and the keratometric power ranged from 31.25 D to 41.00 D (mean, 36.05 D). Mean uncorrected visual acuity before the treatment with pilocarpine was 20/50. After the treatment with pilocarpine, the mean spherical equivalent refraction was -0.31 D (range, -1.75 D to +0.50 D). The mean keratometric power was 38.32 D (range, 34.87 D to 43.12 D), with a mean uncorrected visual acuity at 20/25. The patients were followed for 8 to 49 weeks after treatment without pilocarpine (mean, 21 weeks). The mean spherical equivalent refraction and keratometric readings after that period were -0.71 D (range, -2.25 D to +0.25 D) and 38.33 D (range, 36.12 D to 43.12 D), respectively. All eyes in this study had more than 1.00 D of reduction of hyperopia at the conclusion of the study. CONCLUSION: Pilocarpine effectively reduced overcorrections after radial keratotomy. After termination of treatment, the steepening of corneal curvature was maintained.