Purpose: We report endothelial cell (EC) characteristics and central corneal thickness (CCT) from Infant Aphakia treatment Study (IATS) patients at the 5-year exam.
Design: Randomized, controlled trial of the treatment of unilateral cataract with aphakic contact lens (CL) versus primary intraocular lens implant (IOL).
Subjects: 114 infants with unilateral cataract.
Methods: EC density, coefficient of variation (CV), and percent hexagonal cells were measured by non-contact specular microscopy. Central corneal thickness (CCT) was measured using contact pachymetry. Fellow eyes served as controls.
Main outcome measures: Mean differences between treated and fellow eyes of CL and IOL groups were compared with a paired t test. A one-way analysis of variance model and the Tukey-Kramer multiple comparison procedure were used to assess the effect of a diagnosis of glaucoma or glaucoma suspect.
Results: 105 (52 CL, 53 IOL) had either specular microscopy or corneal thickness data recorded. Mean EC densities were higher in the aphakic eyes compared to fellow eyes (3921 and 3495 cells/mm2, p < 0.0001). Mean CV was higher in aphakic eyes (27 vs 24, p=0.0002), and mean percent hexagonal cells was lower (72% vs 76%, p=0.002). Mean CCT of aphakic eyes was higher than controls (637 vs 563 μm, p < 0.0001). There was no difference in EC densisty in eyes treated with IOL compared to fellow eyes (3445 and 3487 cells/mm2, p=0.68). Means for CV (25 vs 24, p=0.07) and percent hexagonal cells (74 vs 76%, p=0.27) were also not significantly different. Mean CCT was higher in eyes with IOL (605 vs 571 μm, p < 0.0001) compared to fellow eyes. Compared to treated eyes without glaucoma or glaucoma suspect, treated eyes with glaucoma had lower EC density (3289 vs 3783 cells/mm2, p = 0.03) and treated eyes with glaucoma suspect had greater mean corneal thickness (660 vs 612 μm, p = 0.0036).
Conclusion: Cataract extraction during infancy with IOL implantation was not associated with a reduced EC count in treated compared to fellow eyes, although CCT was increased. Extended wear aphakic CL may cause corneal polymegathism with increased EC density and CCT. Glaucoma diagnosis was associated with reduced EC counts and increased CCT.
Objective: To evaluate the characteristics of strabismus in infants who underwent cataract surgery with and without intraocular lens (IOL) implantation.
Design: Secondary outcome analysis in a prospective, randomized clinical trial. Participants: The Infant Aphakia Treatment Study is a randomized, multicenter (n = 12), clinical trial comparing treatment of aphakia with a primary IOL or contact lens in 114 infants with a unilateral congenital cataract.
Intervention: Infants underwent cataract surgery with or without placement of an IOL.
Main Outcome Measures: The proportion of patients in whom strabismus developed during the first 12 months of follow-up was calculated using the life-table method and was compared across treatment groups and age strata using a log-rank test.
Results: Strabismus developed within the first 12 months of follow-up in 38 pseudophakic infants (life-table estimate, 66.7%) and 42 infants (life-table estimate, 74.5%) treated with contact lenses (P = 0.59). The younger cohort (<49 days) at the time of surgery demonstrated less strabismus (29 of 50; life-table estimate, 58.0%) than the older cohort (≥49 days; 51 of 64; life-table estimate, 80.0%; P<0.01).
Conclusions: Intraocular lens placement does not prevent the early development of strabismus after congenital cataract surgery. However, strabismus was less likely to develop in infants whose cataract was removed at an earlier age. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
Objective: Non-mydriatic fundus photography by non-ophthalmic trained personnel has recently been shown to be a potential alternative to direct ophthalmoscopy in the emergency department (ED). We evaluated the reliability of a novel quality rating scale and applied this scale to non-mydriatic fundus photographs taken during routine ED patient encounters to determine factors associated with diminished photograph quality.
Design: Prospective, cross-sectional
Participants: 350 patients enrolled in the Fundus photography vs. Ophthalmoscopy Trials Outcomes in the Emergency Department (FOTO-ED) study were photographed by nurse practitioners after <30 minutes of training followed by supervision.
Methods: Photographs of both eyes were graded for quality on two occasions by two neuro-ophthalmologists. Four regions were independently evaluated for quality: optic disc, macula, superior and inferior vascular arcades. Quality as a function of the number of photographs taken was evaluated by Kaplan-Meier analysis. Mixed effects ordinal logistic regression was used to evaluate for predictors of image quality while accounting for the repeated measures design.
Main Outcome Measure: Overall photographic quality (1–5 scale, 5 best).
Results: We evaluated 1734 photographs. Inter- and intra-observer agreements between neuro-ophthalmologists were very good (weighted kappa:0.84–0.87). Quality of the optic disc area was better than those of other retinal areas (p<0.002). Kaplan-Meier analysis showed that if a high-quality photograph of an eye was not obtained by the third attempt it was unlikely that one would be obtained at all. A 10 second increase in the inter-photograph interval before a total of forty seconds increased the odds of a one unit higher quality rating by 1.81 times (95%CI: 1.68–1.98), and a ten year increase in age decreased the odds by 0.76 times (95%CI: 0.69–0.85). Black patients had 0.42 times (95%CI: 0.28–0.63) the odds of a one unit higher quality rating compared to whites.
Conclusions: Our 5-point scale is a reliable measure of non-mydriatic photograph quality. The region of interest, interphotograph interval, age, and race are significant predictors of image quality for non-mydriatic photographs taken by nurse practitioners in the ED. Addressing these factors may have a direct impact on the successful implementation of non-mydriatic fundus photography into the ED.
by
Jasmine H. Francis;
Duncan Berry;
David H. Abramson;
Christopher A. Barker;
Chris Bergstrom;
Hakan Demirci;
Michael Engelbert;
Hans Grossniklaus;
Baker Hubbard;
Codrin E. Iacob;
Korey Jaben;
Madhavi Kurli;
Michael A. Postow;
Jedd D. Wolchok;
Ivana K. Kim;
Jill Wells
Purpose:
Cutaneous melanoma metastatic to the vitreous is very rare. This study investigated the clinical findings, treatment, and outcome of patients with metastatic cutaneous melanoma to the vitreous. Most patients received checkpoint inhibition for the treatment of systemic disease, and the significance of this was explored. Design: Multicenter, retrospective cohort study.
Participants:
Fourteen eyes of 11 patients with metastatic cutaneous melanoma to the vitreous.
Methods:
Clinical records, including fundus photography and ultrasound results, were reviewed retrospectively, and relevant data were recorded for each patient eye.
Main Outcome Measures:
Clinical features at presentation, ophthalmic and systemic treatments, and outcomes.
Results:
The median age at presentation of ophthalmic disease was 66 years (range, 23–88 years), and the median follow-up from diagnosis of ophthalmic disease was 23 months. Ten of 11 patients were treated with immune checkpoint inhibition at some point in the treatment course. The median time from starting immunotherapy to ocular symptoms was 17 months (range, 4.5–38 months). Half of eyes demonstrated amelanotic vitreous debris. Five eyes demonstrated elevated intraocular pressure, and 4 eyes demonstrated a retinal detachment. Six patients showed metastatic disease in the central nervous system. Ophthalmic treatment included external beam radiation (30–40 Gy) in 6 eyes, intravitreous melphalan (10–20 μg) in 4 eyes, enucleation of 1 eye, and local observation while receiving systemic treatment in 2 eyes. Three eyes received intravitreous bevacizumab for neovascularization. The final Snellen visual acuity ranged from 20/20 to no light perception.
Conclusions:
The differential diagnosis of vitreous debris in the context of metastatic cutaneous melanoma includes intravitreal metastasis, and this seems to be particularly apparent during this era of treatment with checkpoint inhibition. External beam radiation, intravitreous melphalan, and systemic checkpoint inhibition can be used in the treatment of ophthalmic disease. Neovascular glaucoma and retinal detachments may occur, and most eyes show poor visual potential. Approximately one quarter of patients demonstrated ocular disease that preceded central nervous system metastasis. Patients with visual symptoms or vitreous debris in the context of metastatic cutaneous melanoma would benefit from evaluation by an ophthalmic oncologist.
Objective: To design a simple matching acuity test based on hand gestures that is minimally dependent on familiarity with symbols and letters. The visual acuity results obtained from children using the Handy Eye Chart™ were compared with results obtained with the Early Treatment Diabetic Retinopathy Study (ETDRS) chart.
Design: Evaluation of diagnostic test or technology.
Participants: 60 children between 6 and 16 years of age were recruited consecutively from the Pediatric Ophthalmology section of the Emory Eye Center.
Methods: Monocular visual acuity was tested using both the new eye chart and the ETDRS chart, alternating the order of administration between subjects. Testing was performed on the subject’s eye with the poorest acuity.
Main Outcome Measures: Outcome measures were monocular logarithm of the minimum angle of resolution (logMAR) visual acuity scores for each chart.
Results: The acuities were shown to have a strong linear correlation (r = 0.95) and a mean difference in acuity of −0.03 (95% confidence interval −0.05 to −0.01) logMAR, equivalent to approximately 1.5 letters, with the new eye chart underestimating the vision as determined by the ETDRS chart. The 95% limits of agreement were ±1.6 lines.
Conclusion: The present study supports the validity of the new eye chart as a measure of visual acuity in pediatric patients ages 6 to 18 with vision ranging from 20/16 to 20/200.
by
Nathaniel R. Moxon;
Anju Goyal;
JoAnn A. Giaconi;
Jamie B. Rosenberg;
Emily Graubart;
Evan L. Waxman;
Daniel Knoch;
Susan H. Forster;
Privthi S. Sankar;
Rukhsana G. Mirza
Eye disease and vision loss impact quality of life, independence, mental health, social function, and mortality.1, 2, 3 Visual impairment affects 7.5% of the United States population and requires frontline providers to diagnose and manage eye conditions.4 , 5 However, the time dedicated to teaching medical students about these conditions has been on the decline for half a century.6, 7, 8 After the 1910 Flexner report, ophthalmology preserved a small but regular role in United States medical school curricula.9 Unfortunately, as undergraduate medical curricula expanded, ophthalmology saw a decline in curricular time, which may be related to the fact that the Liaison Committee on Medical Education does not provide specific guidelines on ophthalmology training.
To adapt to the changing field of ophthalmology, graduate medical education (GME) must teach trainees to harness the power of information technology. The Accreditation Council for Graduate Medical Education (ACGME) Ophthalmology Milestones 2.0 includes the use of information technology, such as the integration of multimodal data into clinical diagnosis for patient-centered care, effective communication and documentation utilizing the electronic health records (EHR), and analysis of EHR data for quality improvement.
Purpose: Autosomal-dominant optic atrophy (DOA) is one of the most common inherited optic neuropathies, and it is genetically heterogeneous, with mutations in both OPA1 and OPA3 known to cause disease. About 60% of cases harbor OPA1 mutations, whereas OPA3 mutations have only been reported in two pedigrees with DOA and premature cataracts. The aim of this study was to determine the yield of OPA1 and OPA3 screening in a cohort of presumed DOA cases referred to a tertiary diagnostic laboratory.
Design: Retrospective case series.
Participants: One hundred and eighty-eight probands with bilateral optic atrophy referred for molecular genetic investigations at a tertiary diagnostic facility: 38 patients with an autosomal-dominant pattern of inheritance and 150 sporadic cases.
Methods: OPA1 and OPA3 genetic testing was initially performed using PCR-based sequencing methods. The presence of large-scale OPA1 and OPA3 genomic rearrangements was further assessed with a targeted comparative genomic hybridization (CGH) microarray platform. The three primary Leber hereditary optic neuropathy (LHON) mutations, m.3460G>A, m.11778G>A, and m.14484T>C, were also screened in all patients.
Main Outcome Measures: The proportion of patients with OPA1 and OPA3 pathogenic mutations. The clinical profile observed in molecularly confirmed DOA cases.
Results: We found 21 different OPA1 mutations in 27 of the 188 (14.4%) probands screened. The mutations included six novel pathogenic variants and the first reported OPA1 initiation codon mutation at c.1A>T. An OPA1 missense mutation, c.239A>G (p.Y80C), was identified in an 11-year-old African-American girl with optic atrophy and peripheral sensori-motor neuropathy in her lower limbs. The OPA1 detection rate was significantly higher among individuals with a positive family history of visual failure (50.0%) compared with sporadic cases (5.3%). The primary LHON screen was negative in our patient cohort, and additional molecular investigations did not reveal any large-scale OPA1 rearrangements or OPA3 genetic defects. The mean baseline visual acuity for our OPA1-positive group was 0.48 logarithm of the minimum angle of resolution (LogMAR) (Mean Snellen equivalent = 20/61, range = 20/20–20/400, 95% confidence interval = 20/52–20/71), and visual deterioration occurred in 54.2% of patients during follow-up.
Conclusions: OPA1 mutations are the most common genetic defects identified in patients with suspected DOA, whereas OPA3 mutations are very rare in isolated optic atrophy cases.
by
Oscar A. Cruz;
Michael X. Repka;
Amra Hercinovic;
Susan A. Cotter;
Scott R. Lambert;
Amy K Hutchinson;
Derek t. Sprunger;
Christie L. Morse;
David K. Wallace
As a service to its members and the public, the American Academy of Ophthalmology has developed a series of Preferred Practice Pattern guidelines that identify characteristics and components of quality eye care. Appendix 1 describes the core criteria of quality eye care.
The Preferred Practice Pattern guidelines are based on the best available scientific data as interpreted by panels of knowledgeable health professionals. In some instances, such as when results of carefully conducted clinical trials are available, the data are particularly persuasive and provide clear guidance. In other instances, the panels have to rely on their collective judgment and evaluation of available evidence.
Objective:
To describe presenting clinical features and surgical techniques that are associated with successful surgical repair of pediatric rhegmatogenous retinal detachment (RRD).
Design:
Retrospective interventional case series.
Subjects:
212 eyes of 191 patients, aged 0–18 years, undergoing surgical repair for RRD between 2001 and 2015 with a minimum follow up of 3 months.
Methods:
Patients were divided into three age groups (0–6 years, 7–12 years, 13–18 years) and comparisons were made using bivariate and multivariable generalized estimating equation models. A mixed means model was used to examine visual acuity in each age group over time.
Main Outcome Measures:
Complete reattachment of the retina at final follow up.
Results:
Of a total of 212 eyes, 166 (78%) achieved total reattachment at final follow up. Mean follow up was 36.3 months. RRD associated with Stickler syndrome was more likely to occur in the younger cohorts (odds ratio [OR] 0.45, 95 % confidence interval [CI] 0.22 – 0.91), while RRD associated with blunt trauma was more likely to occur in the oldest cohort (OR 2.3, 95% CI 1.2–4.4). Subtotal RRD was more likely to be successfully repaired than total RRD (OR 3.6, 95% CI 1.5 – 8.4, p = 0.0100), and eyes with previous vitreoretinal surgery were less likely to have successful repair (OR 0.30, 95% CI 0.12 – 0.78, p = 0.0258). There was no significant difference between age groups in the rate of surgical success (p = 0.55). There was a significantly higher success rate with primary scleral buckle (SB) (63%, OR 2.2, 95% CI 1.1–4.5) and combined scleral buckle/vitrectomy (SB/PPV) (68%, OR 2.3 95% CI 1.1–5.1) compared to vitrectomy (PPV) alone (51%).
Conclusions:
Most pediatric patients with RRD achieved complete reattachment with surgery. Success was more common in patients with a subtotal RRD at presentation. Previous vitreoretinal surgery was a risk factor for failure. Younger patients were more likely to present with RRD involving the macula but there was no difference between age groups in successful reattachment at final follow up. Primary PPV had a lower rate of success than SB or combined SB/PPV.