Visual findings in children exposed to Zika in utero in Nicaragua.

Knowledge regarding the frequency of ocular abnormalities and abnormal visual function in children exposed to Zika virus (ZIKV) in utero but born without congenital Zika syndrome (CZS) is limited. We hypothesized that children exposed to ZIKV in utero born without CZS may have visual impairments in early childhood. We performed ophthalmic examination between 16 and 21 months of age and neurodevelopment assessment at 24 months of age with the Mullen Scales of Early Learning test (MSEL) on children enrolled in a cohort born to women pregnant during and shortly after the ZIKV epidemic in Nicaragua (2016-2017). ZIKV exposure status was defined based on maternal and infant serological testing. Visual impairment was defined as abnormal if the child had an abnormal ophthalmic exam and/or low visual reception score in the MSEL assessment. Of 124 children included in the analysis, 24 (19.4%) were classified as ZIKV-exposed and 100 (80.6%) unexposed according to maternal or cord blood serology. Ophthalmic examination showed that visual acuity did not differ significantly between groups, thus, 17.4% of ZIKV-exposed and 5.2% of unexposed had abnormal visual function (p = 0.07) and 12.5% of the ZIKV-exposed and 2% of the unexposed had abnormal contrast testing (p = 0.05). Low MSEL visual reception score was 3.2-fold higher in ZIKV-exposed than unexposed children, but not statistically significant (OR 3.2, CI: 0.8-14.0; p = 0.10). Visual impairment (a composite measure of visual function or low MESL visual reception score) was present in more ZIKV-exposed than in unexposed children (OR 3.7, CI: 1.2, 11.0; p = 0.02). However, the limited sample size warrants future investigations to fully assess the impact of in utero ZIKV exposure on ocular structures and visual function in early childhood, even in apparently healthy children.

Postoperative Correction and Drift After Vertical Rectus Muscle Transposition for Total Sixth Cranial Nerve Palsy.

PURPOSE: To determine the magnitude of change between the preoperative and postoperative alignment and amount of postoperative drift for two vertical rectus muscle transpositions (VRTs). METHODS: Retrospective review of medical records of patients with total sixth cranial nerve palsy who underwent VRT procedures. The primary outcome measure was the magnitude of esotropia in prism diopters (PD) at the preoperative and postoperative visits. RESULTS: Twenty-seven patients were included. Sixteen had full tendon transposition with Foster augmentation (FTT+FA) and 11 had partial tendon transposition with resection and simultaneous medial rectus recession (PTT+R+MRR). A larger correction was obtained with PTT+R+MRR (mean ± standard deviation [SD]: 52 ± 19 PD; range: 27 to 87 PD) when compared to FTT+FA (mean: 40 ± 13 PD; range: 15 to 68 PD). At postoperative month 2, a greater esotropic drift was noted in the PTT+R+MRR group (16 PD) than the FTT+FA group (6 PD). Although the difference in the amount of correction was not statistically significant (P = .071), the difference in the amount of drift was statistically significant (P = .009). CONCLUSIONS: There was a trend toward greater correction with PTT+R+MRR than FTT+ FA, but it was not statistically significant. FTT+FA had significantly less postoperative drift than PTT+R+MRR. The results suggest that a small immediate postoperative overcorrection may be desirable in some VRT procedures. [J Pediatr Ophthalmol Strabismus. 2019;56(4):238-242.].

Assessing nonsedated handheld cone flicker electroretingram as a screening test in pediatric patients: comparison to sedated conventional cone flicker electroretinogram.

PURPOSE: To assess the RETeval (LKC Technologies, Gaithersburg, MD) handheld electroretingram (ERG) device as a screening tool for cone dysfunction in pediatric patients by comparing it to conventional ERG. METHODS: Patients scheduled for ERG under general anesthesia (GA) underwent three tests: (1) RETeval standard 30 Hz cone flicker ERG using skin electrodes prior to GA, (2) E3 Diagnosys (Diagnosys LLC, Lowell, MA) conventional complete standard protocol full-field ERG using bipolar contact lens electrodes and handheld stimulus under GA, and (3) repeat RETeval testing under GA. The 30 Hz cone flicker amplitudes and implicit times from the three methods were compared. Negative and positive predictive values were calculated by applying a previously established 5 µV amplitude cut-off. RESULTS: Thirty patients ≤18 years of age were enrolled. Impaired conventional ERGs were found in 18 patients. Compared to conventional ERG under GA, RETeval cone flicker amplitudes were smaller before GA (mean difference, -42.2 ± 45.3 µV) and under GA (-37.1 ± 44.5 µV), likely due to skin electrode; and implicit times were shorter before GA (-1.06 ± 2.83 ms) and longer under GA (1.28 ± 4.12 ms), likely due to GA. Comparing RETeval responses before and under GA, the amplitudes were lower (-3.05 ± 6.82 µV), and implicit times were shorter (-2.25 ± 3.28 µV) before GA. Overall, the positive predictive value of the RETeval was 85%; the negative predictive value, 90%. CONCLUSIONS: The unsedated handheld RETeval 30 Hz cone flicker ERG is a feasible screening test for detecting cone dysfunction in pediatric patients. Full-protocol ERG is needed when screening ERG is reduced, equivocal, or clinically warranted.

Morganella as a cause of ophthalmia neonatorum.

A 2 day-old-girl with a complicated birth history, including prolonged rupture of membranes and Caesarian section delivery, presented with a purulent bacterial conjunctivitis of the left eye despite standard prophylaxis. Bacterial cultures yielded 1+ Morganella morganii, a facultative anaerobe and a rare cause of ocular pathology. The patient was treated with topical tobramycin four times daily for 7 days, resulting in resolution of her conjunctivitis.

Nonsedated handheld electroretinogram as a screening test of retinal dysfunction in pediatric patients with nystagmus.

PURPOSE: To assess the feasibility, sensitivity, and specificity of nonsedated handheld cone flicker electroretinogram (ERG) as a screening tool to detect retinal dysfunction in children with nystagmus. METHODS: Pediatric patients at a tertiary referral center from December 2015 to July 2016 were enrolled and placed into three age-matched groups: normal, nystagmus with a retinal dystrophy, and nystagmus without a retinal dystrophy. Unsedated 30 Hz cone flicker ERG responses were obtained using a handheld device (RETeval) from both eyes of each patient using skin electrode sensors after pupillary dilation. RESULTS: A total of 71 children were enrolled; amplitudes and implicit times were successfully obtained in 65 (92%): 31 (mean age ± SD, 5.6 ± 2.7 years; range, 1-12 years) without nystagmus and 34 with nystagmus. Nystagmus patients were grouped by those with (n = 15; mean age, 8.5 ± 4.5 [range, 2-17 years) and without (n = 19; mean age, 4.3 ± 3.0 [range, 6 months-10 years]) a retinal dystrophy. The patients with retinal dystrophies had significantly smaller amplitudes and prolonged or nonmeasurable implicit times than the other two groups (P < 0.001). Among nystagmus patients, amplitude was able to discriminate between those with and without retinal dystrophies with area under curve of 0.986 (SE = 0.016; P < 0.001). An amplitude <5 µV in combination with an implicit time of >33 ms warrants further evaluation. CONCLUSIONS: Unsedated handheld cone flicker ERG is a feasible screening test that effectively detects retinal dysfunction in children with nystagmus. In conjunction with clinical findings, the test helps reduce the need for sedated ERG in children.

Surgical Treatment of Adult-Onset Esotropia: Characteristics and Outcomes.

PURPOSE: To describe the characteristics of the strabismus, surgical management, and outcomes of patients who underwent surgery for adult-onset esotropia. METHODS: This was a retrospective case study of patients at an academic tertiary referral center who underwent surgical treatment of esotropia acquired at or after age 18 years. Primary outcome measures were resolution of diplopia in primary position and a deviation of 10 prism diopters or less in primary gaze. Additional clinical parameters were assessed. RESULTS: Of 248 patients with adult-onset esotropia who underwent strabismus surgery, all experienced diplopia preoperatively except those with sensory esotropia. The four most common diagnoses were cranial nerve VI palsy in 36% (90 of 248), thyroid eye disease in 18% (45 of 248), age-related distance esotropia in 15% (38 of 248), and decompensated latent esodeviations in 13% (31 of 248) of patients. A variety of surgical procedures were employed, and adjustable sutures were used in 79% (196 of 248). Approximately 80% (158 of 197) of patients present at the 2-month postoperative follow-up visit were aligned within 10 prism diopters, and 72% (140 of 195) experienced resolution of diplopia. Success rates were significantly higher in patients with adjustable sutures. Reoperation rates were low overall at 15% (37 of 248) and were highest in cranial nerve VI palsies and lowest in age-related distance esotropia. Dose-response calculations showed a non-significantly smaller effect per millimeter of recession and resection in cranial nerve VI palsy and age-related distance esotropia. CONCLUSIONS: The causes of adult-onset esotropia are diverse. A variety of surgical approaches are employed and, in conjunction with adjustable sutures, provide a good rate of diplopia resolution and acceptable ocular alignment. [J Pediatr Ophthalmol Strabismus. 2017;54(2):104-111.].

Systemic Amyloidosis and Extraocular Muscle Deposition.

Isolated amyloid deposition in an extraocular muscle is a rare event but can be a presenting feature of systemic amyloidosis. A 67-year-old woman with an acquired exotropia and hypertropia was found to have unilateral diffuse extraocular muscle enlargement on magnetic resonance imaging. Owing to the progressive nature of her strabismus and the negative laboratory testing for thyroid disease, she underwent an extraocular muscle biopsy that revealed amyloid deposition. Further workup demonstrated a monoclonal gammopathy consistent with systemic amyloidosis. This case demonstrates the need to consider amyloidosis in the differential diagnosis of patients presenting with an atypical acquired strabismus. We review other reports of isolated amyloid deposition in extraocular muscles and its association with systemic amyloidosis, emphasizing the importance of the ophthalmologist in the early recognition of this disease to prevent irreversible, life-threatening end organ damage.

Effect of the oxygen saturation target on clinical characteristics of early- versus late-onset retinopathy of prematurity.

PURPOSE: To investigate the relationship between postmenstrual age of onset of retinopathy of prematurity (ROP) and the need for treatment, while examining the effects of two different neonatal oxygen saturation protocols on this relationship. METHODS: A retrospective chart review was conducted for eligible inborn infants born before and after the institution of a new oxygen protocol adjusting target oxygen saturation from 90%-99% to 85%-93%. Early versus late-onset ROP was defined as first presence of any stage disease on examination at <36 versus ≥ 36 weeks' postmenstrual age, respectively. RESULTS: The median birth weight/postmentrual age of infants was 840 g per 26.1 weeks (early-onset ROP) versus 952.5 g per 28 weeks (late-onset ROP; P < 0.01 vs P < 0.01). ROP developed in 119 of 369 (32.2%) of high oxygen target infants, and 100 of 373 (26.8%) infants in the low-target group (P = 0.11). Cumulatively, 35 of 144 (24.3%) of early-onset and 8 of 69 (11.6%) of late-onset patients required treatment (P = 0.03). Maximal severity of disease after treatment, including retinal detachment frequency, was similar in early- and late-onset patients, independent of the oxygen protocol (P = 1.00). CONCLUSIONS: The clinical behavior of type 1 ROP is similar in early-and late-onset disease, regardless of oxygen saturation targets. Type 1 ROP disease occurred in 11.6% of patients with late-onset ROP.