Elhusseiny AM, Saeed HN. Posterior Polymorphous Corneal Dystrophy in a Pediatric Population. Cornea 2022;41(6):734-739.Abstract
PURPOSE: The aim of this study was to evaluate the clinical and topographic features of posterior polymorphous corneal dystrophy (PPCD) in children aged 15 years or younger with a long-term follow-up. Retrospective case series. METHODS: A retrospective chart review of patients who were diagnosed with PPCD at Boston Children's Hospital from 1999 to 2020 was performed. Data collected included age at the time of diagnosis, slit lamp findings, cycloplegic refraction, best-corrected visual acuity, central corneal thickness, specular microscopy, and corneal topography findings whenever available. RESULTS: Twenty-seven eyes of 19 patients were included (11 unilateral and 8 bilateral cases). Ten patients were girls (52.6%). Left eye was affected in 14 eyes. The mean age at the time of diagnosis was 8.5 ± 3.3 years, with a mean follow-up of 5.3 years. In unilateral cases, there was a statistically significant difference in the endothelial cell density (P = 0.01), coefficient variation (P = 0.03), and hexagonality (P = 0.01) between the affected and the contralateral unaffected eyes. The mean best-corrected visual acuity at initial presentation was 0.8 ± 0.2 compared with 0.9 ± 0.08 in unaffected eyes (P = 0.04). The mean astigmatism was higher in the affected eye (+1.7 diopters) compared with (+1.00) the unaffected eye (P = 0.07). At initial presentation, 7 of 27 eyes had amblyopia, which resolved, either partially or completely, in 5 eyes after treatment. CONCLUSIONS: PPCD can present early in children with astigmatism and anisometropic amblyopia. A careful slit lamp examination for children presenting with anisoastigmatism is necessary to diagnose PPCD. Contrary to adults, presentation is often unilateral. Such patients should be followed up regularly with cycloplegic retinoscopy to prevent and treat refractive amblyopia if present.
Jiro MC, Sigua M, Ivey SL, Maus M, Hennein L, Dio M, Cocohoba J. Ang Ating Mata: Disparities in Eye Health Knowledge, Attitudes and Practices among Older Adult Filipino-Americans in the San Francisco Bay Area Counties. J Immigr Minor Health 2022;Abstract
Filipino-Americans are the third largest Asian-American population, with a median age of 44. However, there is limited literature focusing on the group's ophthalmic care engagement. Timely eye examinations and outreach are necessary to reduce visual impairment in this older community. To assess eye care knowledge, attitudes, and practices, we conducted a cross-sectional study surveying Filipino-Americans within the nine San Francisco Bay Area counties. Associations between primary outcomes and sociodemographic factors were analyzed using chi-squared analysis and student's T-test. In our convenience sample of 256 surveys, a majority of participants are receiving appropriate eye care; those that lacked health and eye insurance, immigrated and are lower income did not receive optimal eye care. Study participants also demonstrated a lack of awareness of eye diseases and risk factors. Our results suggest that culturally sensitive eye health education materials are lacking and should be made accessible for this large and rapidly growing population.
Buch KA, Bouffard MA, Kardon RH, Wills A-MA, Privitera CM, Sharma M, Wray SH. Clinical Correlation Between Vertical Gaze Palsy and Midbrain Volume in Progressive Supranuclear Palsy. J Neuroophthalmol 2022;42(2):246-250.Abstract
BACKGROUND: Supranuclear vertical gaze palsies and slowed vertical saccades are characteristic clinic features of progressive supranuclear palsy (PSP). The "hummingbird sign," reflective of midbrain atrophy, is a classic radiographic sign of PSP. Correlation between eye movement abnormalities and radiographic findings in PSP has been reported previously. However, due to the use of clinical criteria not commonly employed in neuro-ophthalmic practice and neuroimaging techniques that are not widely available, it remains unclear whether correlation between midbrain structure and characteristic ocular-motor disturbances can be helpful to neuro-ophthalmologists seeking to adjudicate difficult or unusual diagnostic cases. METHODS: Patients with a diagnosis of probable PSP according to Movement Disorders Society criteria were studied retrospectively. A neuroradiologist calculated brainstem volumes in enrolled participants and normal controls. Spearman correlations were used to correlate the extent of eye movement limitation as assessed by 2 neuro-ophthalmologists with brainstem volumes. RESULTS: Fourteen participants with PSP and 15 healthy controls with similar age and gender distribution were enrolled and evaluated retrospectively. All 14 participants with PSP had undergone MRIs. Midbrain atrophy significantly correlated with the PSP rating scale (P < 0.001). PSP patients had significantly reduced volumes in the midbrain (P -0.0026), tegmentum (0.0001), tectum (0.0001), and medulla (P = 0.0024) compared with normal controls. Notes documenting quantified ocular motor function were available in 7 of 14 participants with PSP. Midbrain atrophy significantly correlated with in the extent of upward gaze limitation (P = 0.03). CONCLUSIONS: The severity of upward gaze limitation correlates with the severity of midbrain atrophy in patients with PSP. Recognition of this correlation may help to adjudicate diagnostic dilemmas and guide further evaluation.
Danford ID, Scruggs BA, Capone A, Trese MT, Drenser KA, Thanos A, Nudleman E, Amphornphruet A, Tipsuriyaporn B, Hubbard BG, Ells A, Harper AC, Goldstein J, Calvo C, Wallace-Carrete C, Berry D, Chang E, Leishman L, Shapiro M, Blair M, Mikhail M, Shields CL, Schwendeman R, Yonekawa Y, Gupta MP, Orlin A, Prakhunhungsit S, Mukai S, Berrocal A, Hartnett EM, Campbell PJ. The Prevalence of Retinal Disease and Associated Central Nervous System Disease in Young Patients with Incontinentia Pigmenti. Ophthalmol Retina 2022;Abstract
PURPOSE: To evaluate the prevalence of retinal disease on fluorescein angiography (FA) in patients with incontinentia pigmenti (IP) and to compare the severity of retinal disease in those with and without known central nervous system (CNS) disease. DESIGN: Multi-institutional consecutive retrospective case series SUBJECTS: New patients with a diagnosis of IP seen at the Casey Eye Institute, Oregon Health and Science University, Moran Eye Center, University of Utah, Wills Eye Hospital, or Bascom Palmer Eye Institute, University of Miami from December 2011 to September 2018. METHODS: Detailed ophthalmoscopic examination and FA were recommended to all new patients and performed on every patient who had parental consent. Ophthalmoscopic findings and FA images were graded for severity by two masked graders on a 3-point scale: 0 = no disease, 1 = vascular abnormalities without leakage, 2 = leakage or neovascularization, 3 = retinal detachment. Presence of known CNS disease was documented. Additional cases were obtained from a pediatric retina listserv for examples of phenotypic variation. MAIN OUTCOME MEASURES: Proportion of eyes noted to have disease on ophthalmoscopy compared with FA. Severity of retinal disease in those with and without known CNS disease. RESULTS: Retinal pathology was detected in 18/35 (51%) by indirect ophthalmoscopy and 26/35 (74%) by FA (p=0.048) in a predominantly pediatric population (median age = 9 months). Ten patients (29%) had known CNS disease at the time of the eye exam. A Wilcoxon ranked sums test indicated that the retinal severity scores for patients with CNS disease (median = 2) were significantly higher than the retinal severity scores for patients without CNS disease (median = 1), z = -2.12, p = 0.034. CONCLUSION: Retinal disease is present in the majority of patients with IP, and the ophthalmoscopic examination is less sensitive than FA for detection of disease. There may be a correlation between the severity of retinal and CNS disease.
Oke I, Shah AS, Lorenz B, Basiakos S, Gokyigit B, Ugo Dodd M-M, Laurent E, Hunter DG, Goberville M, Elkamshoushy A, Tsai C-B, Orge F, Velez FG, Jeddawi L, Gravier N, Li N, Dagi LR, Dagi LR. Nasal Transposition of the Split Lateral Rectus Muscle for Strabismus Associated with Bilateral 3rd-Nerve Palsy. Am J Ophthalmol 2022;Abstract
PURPOSE: To determine success rate and complications associated with nasal transposition of the split lateral rectus muscle (NTSLR) for treating bilateral 3rd-nerve palsy. DESIGN: Retrospective, interventional case series METHODS: : Setting: International, multicenter registry Study population: All patients with bilateral 3rd-nerve palsy treated with NTSLR. OBSERVATION: Sensorimotor evaluations before and 6-months after unilateral or bilateral NTSLR. OUTCOME MEASURES: Post-operative horizontal alignment ≤ 15 prism diopters (PD), intraoperative technical difficulties, and vision-threatening complications. The association of patient demographics and surgical technique with each outcome was analyzed using multivariable logistic regression. RESULTS: Thirty-four patients were included with a median age of 46 years (Interquartile range [IQR], 25-54 years) at surgery. The most common etiologies were ischemic (29%), neoplastic (15%), and congenital (12%). NTSLR performed unilaterally with alternative surgery on the opposite eye (65%) resulted in a median post-operative exotropia of 18 PD (IQR, 7-35 PD), and when performed bilaterally (35%) resulted in post-operative exotropia of 14 PD (IQR, 5-35 PD). Success was achieved in 50% of cases, intra-operative technical difficulties reported in 18%, and vision-threatening complications occurred in 21%. Attachment of the lateral rectus muscle ≥ 10 mm posterior to the medial rectus insertion was associated with increased vision-threatening complications (OR, 9.0; 95% CI, 1.3-99). CONCLUSIONS: NTSLR can address the large-angle exotropia associated with bilateral 3rd-nerve palsy. Surgeons should be aware that posterior placement of the lateral rectus muscle can increase the risk of vision-threatening complications, particularly serous choroidal effusion.
Liebman DL, Tam EK, Lithgow MY, Kane JE, Fischbein NJ, Lefebvre DR, Chwalisz BK, Gaier ED. Optic Perineuritis Associated With Cryptococcal Meningitis Presenting With a "Hot Orbit" in a Patient With Chronic Lymphocytic Leukemia. J Neuroophthalmol 2022;42(2):272-277.Abstract
ABSTRACT: A 75-year-old man presented with 3 days of progressive left retro-orbital pain, eyelid swelling, tearing, and pain with extraocular movement. His medical history was significant for type II diabetes mellitus and chronic lymphocytic leukemia, stable on no therapy since diagnosis 8 years prior. The initial examination was significant for diffuse restriction of left ocular motility, marked lid edema, and mild dyschromatopsia. Computed tomography demonstrated asymmetric left periorbital soft tissue swelling and intraconal fat stranding with an irregular left optic nerve sheath complex and clear paranasal sinuses. He was hospitalized for orbital cellulitis and treated empirically with broad-spectrum intravenous antibiotics, but his visual acuity declined over the ensuing 2 days. Subsequent MRI demonstrated left-greater-than-right circumferential optic nerve sheath enhancement, and leptomeningeal enhancement. An orbital biopsy demonstrated monoclonal B-cell lymphocyte aggregation, whereas a lumbar puncture was positive for Cryptococcus antigen with subsequent demonstration of abundant Cryptococcus by Papanicolaou stain. The final diagnosis was optic perineuritis secondary to cryptococcal meningitis presenting with orbital inflammation. Although his clinical course was complicated by immune reconstitution inflammatory syndrome, symptoms and signs of optic neuropathy ultimately resolved after 1 month of intensive antifungal therapy.
Mei CY, Zhang Y, Pan L, Dong B, Chen X, Gao Q, Xu H, Xu W, Fang H, Liu S, McAlinden C, Paschalis EI, Wang Q, Yang M, Huang J, Yu A-Y. A One-Step Electrochemical Aptasensor Based on Signal Amplification of Metallo Nanoenzyme Particles for Vascular Endothelial Growth Factor. Front Bioeng Biotechnol 2022;10:850412.Abstract
In this study, a one-step electrochemical aptasensor was developed to detect the biomarker vascular endothelial growth factor (VEGF), an important protein in the pathogenesis of many retinal diseases, including age-related macular degeneration, diabetic retinopathy, retinopathy of prematurity, and retinal vein occlusion. The aptamer has a good affinity and can rapidly identify and capture VEGF based on its unique structure. We designed a VEGF aptasensor based on the aptamer recognition and complex metallo nanoenzyme particles as an electron exchange center and bridge between capture DNA and electrode. The aptamers maintained the hairpin structure to avoid nonspecific surface adsorption and expose the capture sequence outwards when the target was inexistent. Conversely, the aptamers opened the hairpin structure to release space to accomplish binding between VEGF and DNA, resulting in increased impedance. The performance of the electrochemical aptasensor is detected by electrochemical impedance spectroscopy (EIS). The limit of detection by EIS was as low as 8.2 pg ml-1, and the linear range was 10 pg ml-1-1 μg ml-1. The electrochemical aptasensor also showed high specificity and reproducibility.