Correlation between age and corneal edema in pediatric patients with Peters anomaly.

PURPOSE: To evaluate corneal edema in different-aged pediatric patients with Peters anomaly and to correlate in vivo with ex vivo histopathologic findings. METHODS: A retrospective cross-sectional study was performed. The medical records of patients diagnosed with Peters anomaly who underwent examination under anesthesia (EUA) between 2011 and 2015 were reviewed. Eyes in which central corneal thickness (CCT) measurements were taken were included. The thickest point in the CCT pachymetric map was used to objectively quantify corneal edema. Correlation between CCT and age was calculated. Additionally, a retrospective review of histopathologic studies of excised corneal buttons from pediatric eyes with Peters anomaly between 2011 and 2015 was performed. RESULTS: Eighteen eyes of 12 children were included. Mean age was 14 ± 15 months, and mean CCT was 842 ± 304 µm. A significant inverse correlation was noted between the CCT and the age of the patients, with lower CCT values in older children (r = 0.6; P = 0.01). Seven excised corneal buttons that underwent penetrating keratoplasty were reviewed. All corneal buttons showed absence of Descemet membrane and localized absence of endothelium. However, three specimens showed presence of corneal endothelium in areas of absent or attenuated Descemet membrane. CONCLUSIONS: In Peters anomaly, the CCT decreases with age, possibly due to a decrease in corneal edema. Histopathologic studies show cases of endothelial expansion in areas of absent or attenuated Descemet membrane. This may contribute to improved endothelial function and decreased edema with age.

Trends and Clinical Characteristics of Pediatric Patients Presenting to an Ophthalmology Emergency Department with an Initial Diagnosis of Optic Nerve Head Elevation.

Objective: Optic nerve head elevation may be the only clinical sign of a vision- and/or life-threatening condition, particularly in children who are asymptomatic or fail to report symptoms. The purpose of this study is to identify epidemiologic trends, clinical characteristics, and diagnostic outcomes of pediatric patients presenting to an ophthalmology emergency department (ED) with suspected optic nerve head elevation. Methods: This retrospective study identified pediatric patients (≤18 years) presenting to an ophthalmology ED over a five- year period with suspected optic nerve head elevation to describe demographic trends, clinical characteristics, and diagnostic outcomes. Results: Of the 213 patients, the majority were female (73.2%) and tended to be older than males (14.3 vs 11.2 years, p<0.001). The most common diagnoses were papilledema (52.6%), pseudopapilledema (31.5%), and optic nerve drusen (8.0%). When symptoms were present, most (73.7%) patients presented with headache (67.5%) or visual disturbance (54.8%). Of 144 neuroimaging studies obtained, 80 (55.6%) had positive findings and these were significantly associated with symptoms at presentation (p=0.017) and age (p=0.034), but not sex (p=0.791). Asymptomatic females and children >12 years old were more likely to have an abnormal MRI than males and children ≤ 12 (OR=1.56 and 1.22, respectively). Conclusion: Patient demographics such as age and sex in conjunction with concurrent symptoms are important factors in determining the urgency of neuroimaging in children with suspected optic nerve head elevation.

Characteristics of diplopia as the presenting symptom to an ocular emergency room.

The medical records of 350 patients (median age, 59 years) who presented emergently at Bascom Palmer Eye Institute from 2014 to 2019 for evaluation of diplopia were reviewed retrospectively. Diplopia was binocular in 305 cases (median duration, 5 days). Strabismus was present in 301 patients (horizontal in 49.2% of cases). The most common cause of binocular diplopia was cranial nerve palsy: of the 208 cases, 134 (64%) were microvascular. Nearly half of the neuroimaging scans obtained (102/205) were abnormal. Ten patients with abnormal scans required urgent neurosurgical evaluation.

Trends in pediatric ocular trauma presenting to an ophthalmology-specific emergency department during the COVID-19 pandemic.

Because children remained at home throughout the COVID-19 pandemic, the potential existed for an increased rate of ocular injuries. We performed a retrospective chart review of children (≤18 years of age) presenting to an ophthalmology emergency department (ED) at a single institution from March 1 to August 31, 2020, to describe the trends in ocular trauma during the pandemic. Of the 10,738 ED patients, 6% (643) were children. Of these, 156 (24%) sustained ocular trauma. Although the overall number of visits was 44% lower than the same 6-month period in 2019, trauma-related visits were only 34% lower. The mean age was 9.1 ± 5.53 years (range, 4 months to 18 years) and over half (57%) of patients were male. Most closed-globe trauma was due to blunt (48.6%) or sharp (14.4%) objects, foreign bodies (23.3%), or chemical injuries (13.7%). Ten patients (6.4%) presented with an open globe. Blunt trauma was most common regardless of age; however, chemical injuries tended to occur in younger patients compared with all other diagnosis groups (4.7 ± 4.6 vs 9.7 ± 5.4 years; P = 0.0002). Although COVID-19 restrictions limited school, recreational activities, and team sports, ocular injuries remained prevalent in children.

Characteristics of diplopia in a pediatric population.

PURPOSE: To evaluate the characteristics of diplopia in children at a single tertiary eye care center. METHODS: The medical records of patients with diplopia onset at age 18 years or younger presenting during the period 2015-2018 were reviewed retrospectively. Demographic information, clinical characteristics, diagnoses, treatment, and outcome data were collected. The exact χ2 test was used to compare groups and select post hoc analyses were performed using the Fisher exact or exact χ2 tests. RESULTS: A total of 244 patients (average age, 12.2 years at presentation) were included. The most common clinical diagnoses were nonparalytic strabismus (49.2%), trauma (9.4%), and cranial nerve palsies (9%). There was no statistically significant difference in median age of diplopia onset in those with nonparalytic strabismus, cranial nerve palsies, and vision- or life-threatening conditions. There was a statistically significant difference in timing of onset of diplopia at presentation in vision- or life-threatening conditions compared to nonparalytic strabismus (P < 0.0001) and cranial nerve palsies (P = 0.01) and for neurologic symptoms in vision- or life- threatening conditions compared to nonparalytic strabismus (P = 0.032) and cranial nerve palsies (P = 0.0051). In patients with more than one neurologic symptom, the majority (58.3%) had a vision- or life-threatening condition. Initial interventions included observation (28.7%), prisms (18.4%), updating refractive correction (14.3%), and strabismus surgery (11.1%). In patients with follow-up data, 5.3% had resolution of diplopia prior to their clinic visit, and 46.6% had resolution after initial intervention. CONCLUSIONS: In our study cohort, most children who presented with diplopia had nonemergent conditions. In those with life-threatening conditions, diplopia tended to have an acute onset and associated neurologic signs and visual symptoms.

Oral acetazolamide as a medical adjuvant to retinal surgery in optic disc pit maculopathy in a pediatric patient.

PURPOSE: To describe a novel combination of surgical and medical management for the treatment of optic disc pit maculopathy. OBSERVATIONS: A 12-year-old obese girl with bilateral optic disc pits presented with decreased vision in the left eye (20/400). On dilated fundus examination, she was found to have a macula-involving serous retinal detachment. Pars plana vitrectomy, posterior hyaloid peel, internal limiting membrane peel, fluid-air exchange, temporal juxtapapillary endolaser, and C3F8 tamponade were performed. Postoperatively, there was persistence of subretinal fluid, so oral acetazolamide and a weight loss regimen were started. After 3 months of medical treatment, the subretinal fluid decreased significantly and visual acuity improved to 20/60. CONCLUSION AND IMPORTANCE: Currently, the mechanisms leading to optic disc pit maculopathy remain a topic of debate. As optic disc pits may provide a conduit between the subarachnoid and subretinal spaces, the reduction of intracranial pressure with the use of systemic carbonic anhydrase inhibitors may play a role in decreasing the subretinal fluid associated with select cases of optic disc pit maculopathy.

Clinical Diagnosis and Histopathologic Features of a Synthetic Fiber Granuloma.

The authors report a case of a synthetic fiber granuloma to demonstrate the challenges in diagnosing these lesions and to highlight their histopathologic features. This is the first report in the literature to use histopatho-logic and immunofluorescence studies to characterize the subtype and distribution of macrophages in synthetic fiber granuloma. [J Pediatr Ophthalmol Strabismus. 2020;57:e92-e95.].

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.

Strabismus surgery outcomes without removal of scleral buckle in patients with previous retinal detachment repair.

PURPOSE: To report the motor and sensory outcomes of strabismus surgery following scleral buckle procedure for retinal detachment (RD) without removal of the scleral buckle. METHODS: The medical records of patients who underwent strabismus surgery without removal of scleral buckle following RD surgical repair at a tertiary referral center between 2002 and 2015 were reviewed retrospectively. Demographic data were recorded, and rates of surgical motor success (defined as horizontal deviation of ≤10Δ and vertical deviation of ≤4Δ) and sensory success (resolution of diplopia) were calculated. RESULTS: A total of 23 patients (mean age, 58.4 ± 24.4 years; 12 males) were included. The average time between the RD surgery and onset of strabismus was 11.05 ± 10.95 months (range, 1-42 months). The strabismus was horizontal in 6 patients, vertical in 2, and combined in 15. Eighteen patients (78%) presented with diplopia. Adjustable sutures were used in 18 patients. Final motor surgical success was achieved in 17 of 23 patients (74%), and diplopia improved in 17 of 18 patients (94%) who had preoperative fusional capability. There was no statistically significant difference in age, number of RD surgeries, macular status, time to strabismus surgery, visual acuity in the worse eye, or magnitude of preoperative horizontal and vertical deviation with regard to motor success rate and with persistence of diplopia postoperatively. CONCLUSIONS: In our study cohort, strabismus surgery without removal of the scleral buckle resulted in motor success and alleviated diplopia in the majority of patients with prior RD repair.

Association between congenital nasolacrimal duct obstruction and mode of delivery at birth.

PURPOSE: To investigate the association between mode of delivery, incidence of congenital nasolacrimal duct obstruction (CNLDO), and treatment outcomes. METHODS: The medical records of children diagnosed with CNLDO at a tertiary referral center between 2012 and 2017 were analyzed retrospectively. Patient demographics, pregnancy and birth history, clinical characteristics of CNLDO, and treatment outcomes were compared in patients delivered via Cesarean section (CS) versus vaginal delivery (VD). The rates of CS, as well as full-term and premature births, were also compared to Miami-Dade County normative values to eliminate the confounding effects of prematurity. RESULTS: A total of 104 patients were included. A significantly higher percentage of patients with CNLDO (61%) were delivered via CS (P < 0.0001). Among full-term babies, there was 55% greater risk (OR = 1.55; 95% CI, 0.98-2.43; P = 0.067) of CNLDO for CS birth compared to all other babies. Among preterm babies, there were no significantly greater odds of CNLDO for CS compared to VD births (P = 0.575). CNLDO did not resolve spontaneously in 50 patients, including 37 CS (74%) and 13 VD (26%) patients (P = 0.007). Among those patients who failed first-line probing, 86.2% were born via CS, whereas 13.8% were born via VD (P = 0.0009). CONCLUSIONS: CS is a risk factor for CNLDO, independent of gestational age. Children born via CS also tend to have a more complicated clinical course requiring additional surgical interventions.

Retinal pigment epithelium changes in pediatric patients with glaucoma drainage devices.

PURPOSE: Retinal changes secondary to hypotony are usually described as wrinkling or folding of the inner portion of the choroid, the retinal pigment epithelium (RPE), and the outer retinal layers in the macular area due to scleral wall collapse. We describe a new retinal finding in children with suspected hypotony after implantation of Baerveldt Glaucoma Implant (BGI). OBSERVATIONS: Four patients in our series developed significant RPE defects after BGI implant. The RPE defects appeared as elongated white lines observed solely in the posterior pole, in no particular pattern, and seemed to be worse in infants with anterior segment dysgenesis and with collagen disorders. CONCLUSION AND IMPORTANCE: Children have thinner and more elastic scleral walls than adults. This characteristic may cause the inward scleral wall to collapse when the eye is hypotonic. The resulting redundancy of the retina leads to wrinkling and RPE defects characterized by hypopigmented lines predominantly in the macular area. Such findings, to our knowledge, have not been previously reported in pediatric patients.

Clinical management outcomes of childhood glaucoma suspects.

PURPOSE: To investigate the outcomes of childhood glaucoma suspects. DESIGN: Retrospective case series. METHODS: Records of childhood glaucoma suspects were identified using financial claims data; medical history, baseline biometric and exam findings were recorded. Conversion from suspect to glaucoma was determined based on the Childhood Glaucoma Research Network criteria. The study adheres to the tenets of the Declarations of Helsinki. RESULTS: 214 subjects were enrolled, with median age at initial presentation of 6.37 years (interquartertile range: Q1 = 2.46, Q3 = 8.90). 22 (10.2%) subjects developed glaucoma, 64 (29.9%) had ocular hypertension but no glaucoma, 9 (4.2%) had high-risk condition or syndrome without either ocular hypertension or glaucoma after a mean follow up of 39 +/- 34 months. Neither a family history of glaucoma nor patient gender was significantly different between the groups. 40.2% of subjects (86 of 214) had two or more episodes of intraocular pressure (IOP) > 21 mmHg, among which 25.6% (22 of 86) developed glaucoma after a mean duration of 32.8 +/- 33.5 months. CONCLUSIONS: Up to 25% of children with 2 or more episodes of elevated IOP may develop glaucoma. In 50% of suspects who converted to glaucoma, elevated IOP was not present at the initial evaluation. There is no significant difference in gender, family history, or baseline central corneal thickness between suspects who developed glaucoma compared to the rest. While suspects who converted to glaucoma had higher average, maximum and minimum IOP measurements, there is no clear cutoff between the groups.

Combination of anterior segment optical coherence tomography modalities to improve accuracy of rectus muscle insertion location.

Anterior segment optical coherence tomography (AS-OCT) is useful for locating the extraocular muscle insertion prior to strabismus surgery; however, its accuracy decreases in reoperations. This masked retrospective study investigated whether a combination of AS-OCT modalities improves accuracy. The distance between the corneoscleral limbus and extraocular muscle insertion was measured with AS-OCT, first using the standard grayscale modality alone and then refined with the color modality. The AS-OCT measurement was considered accurate when within 1.00 mm of the intraoperative caliper measurement. A total of 139 AS-OCT images were analyzed from 74 patients (mean age, 52 years), including 60 medial rectus (19 reoperations), 61 lateral rectus (11 reoperations), 10 superior rectus, and 8 inferior rectus muscles. Compared to grayscale alone, the combination grayscale/color modalities improved AS-OCT accuracy from 77% to 87% (P = 0.03), reflecting an increase from 83% to 94% (P = 0.01) in primary surgeries and from 53% to 60% (P = 0.60) in reoperations.

Strabismus surgery outcomes in eyes with glaucoma drainage devices.

PURPOSE: To report strabismus surgery outcomes in eyes with prior implantation of glaucoma drainage devices (GDD). METHODS: The medical records of patients who underwent strabismus surgery for ocular misalignment that developed after implantation of a GDD over a 13-year period at a single institution were examined retrospectively. Patient characteristics, deviation types, preoperative measurements, surgical procedures, and postoperative measurements were analyzed. RESULTS: Of the 16 patients included, 14 had exotropia (34Δ ± 16Δ) and 11 had vertical deviations (15Δ ± 7Δ), of which 9 had concurrent exotropia and vertical deviations. Preoperatively, 9 patients had diplopia. The surgical approach was tailored to address the deviation most noticeable to the patient-horizontal, vertical, or both if the vertical component could be addressed by horizontal muscle supra- or infraplacement. Three patients underwent simultaneous horizontal and vertical surgery. All patients underwent strabismus surgery on an eye with a GDD. Surgical motor success (defined as horizontal deviation ≤10Δ, vertical ≤4Δ) was achieved in 42% of horizontal and 57% of vertical deviations. Postoperatively 74% of deviations decreased by ≥50% in magnitude. Diplopia resolved in 50% of patients who presented with preoperative diplopia. One patient had intraoperative bleb perforation, but none had postoperative hypotony at any follow-up visit. Only 2 required a second strabismus surgery. CONCLUSIONS: Strabismus surgery with preservation of the filtering bleb following implantation of a glaucoma drainage device is a low risk procedure that can improve ocular alignment and related symptoms, despite a low motor success rate by standard criteria.

Correlation of echographic and photographic assessment of optic nerve head cupping in children.

PURPOSE: To determine the diagnostic value of B-scan echography in optic nerve head (ONH) cupping estimation in children. METHODS: The medical records of pediatric patients who had previously undergone examination under anesthesia and for whom both adequate B-scan echography images and optic nerve head (ONH) photographs and were available were reviewed retrospectively. The cup:disk ratio was estimated with a grading scale of 0-1.0 and rounded to the nearest tenth; degree of cupping was estimated from B-scan echography (small, medium, or large) by 5 masked graders (3 glaucoma specialists and 2 ophthalmic sonographers) on 2 separate occasions. Inter- and intraobserver agreement in echographic and photographic cupping assessment by the masked graders as well as correlation of echographic and photographic cup size estimation was evaluated. RESULTS: A total of 36 children were included. Glaucoma specialists reliably assessed cup:disk ratio with moderately good consistency across specialists (average intraclass correlation coefficient [ICC] for intraobserver agreement, 0.86; average ICC for interobserver agreement, 0.71). Sonographers were extremely reliable in assessment of cup size when examining echographic images (ICC for both inter- and intrarater variability, 1.0). Echographic estimate of cup size correlated poorly with cup:disk ratio (ICC, 0.34). CONCLUSIONS: B-scan echography is a reliable and consistent diagnostic tool in estimating the degree of ONH cupping in children and can be very useful in patients in whom direct visualization is not feasible. Failure to account for disk size may have contributed to the poor correlation between echographic cup size and photographic cup:disk ratio.

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.

Paediatric infectious keratitis: a case series of 107 children presenting to a tertiary referral centre.

BACKGROUND/AIMS: Corneal ulcers can result in severe visual impairment in children. The recent trends of paediatric microbial ulcerative keratitis in the USA are unknown. The purpose of this study is to report the risk factors, microbiological profile and treatment outcomes of paediatric microbial keratitis in South Florida. METHODS: A university-based tertiary eye care centre retrospective case series between 1992 and 2015. Medical records of 107 paediatric patients (age <18 years) with the diagnosis of microbial ulcerative keratitis were analysed. Patient demographics, culture data, microbial susceptibility, management trends and patient outcomes were collected. RESULTS: Mean age of patients was 13±4.6 years (range 0.2-17 years). The most common associated risk factor was contact lens wear (77.6%), followed by ocular trauma (8.4%). Systemic factors were present in 4.7% of cases. Cultures were taken from 89 patients. A total of 74 organisms were isolated from the 52 corneal scrapings with growth, yielding a 58.4% positivity rate. Seventeen microbial species were identified, with a predominance of Pseudomonas aeruginosa (46.2%), followed by Stenotrophomonas maltophilia (19.2%) and Fusarium (13.5%). Combined fortified antibiotics were the most common treatment (51.4%). Mean follow-up time was 40.6±91.6 weeks (range: 0.3-480 weeks). The mean visual acuity improved from 20/160 to 20/50 (p<0.0001). No therapeutic penetrating keratoplasty was needed. CONCLUSIONS: In this study, contact lens wear was the most frequent risk factor in infectious keratitis in children. P. aeruginosa was the most common microorganism present in our setting. The majority of the cases responded well to medical management.