FUNCTIONAL OUTCOMES OF YOUNG INFANTS WITH AND WITHOUT MACULAR EDEMA.

PURPOSE: The authors relate posterior segment microanatomy from perinatal spectral domain optical coherence tomography to visual acuity, brain abnormalities, and neurodevelopment. METHODS: Thirteen infants (11 preterm and 2 term birth), imaged in the nursery with portable spectral domain optical coherence tomography, had visual acuity and sensorimotor testing at age 9 months to 15 months (grating acuity) or 4 years to 5 years (optotype), and medical records reviewed for brain magnetic resonance imaging reports and Bayley scales testing at age 18 months to 24 months. RESULTS: Eight children with age-appropriate macular microanatomy without edema on perinatal spectral domain optical coherence tomography had optimal (≥ 20/40) or within normal limits (grating acuity) visual acuity. Five children with perinatal macular edema had suboptimal visual acuity (in 9/10 eyes) and sensorimotor deficits, magnetic resonance imaging abnormalities, or poor neurodevelopment. Macular edema persisted in 1 infant through 9-month corrected age. CONCLUSION: Maturation of the visual system and evolution of retinal anomalies can be monitored with posterior segment spectral domain optical coherence tomography. Retinal microanatomy observed in infancy might relate to subsequent vision and other central nervous system events, but additional studies are needed to determine the range of normal microanatomy in infants and how this relates to vision and neurodevelopment.

2021 U.S. Virgin Islands Zika health brigade: Providing recommended pediatric health screenings for children born to mothers with laboratory evidence of possible Zika virus infection during pregnancy.

BACKGROUND: The United States Virgin Islands (USVI) Department of Health (DOH) conducted a second Zika health brigade (ZHB) in 2021 to provide recommended Zika-related pediatric health screenings, including vision, hearing, neurologic, and developmental screenings, for children in the USVI. This was replicated after the success of the first ZHB in 2018, which provided recommended Zika-related pediatric health screenings to 88 infants and children exposed to Zika virus (ZIKV) during pregnancy. METHODS: Ten specialty pediatric care providers were recruited and traveled to the USVI to conduct the screenings. USVI DOH scheduled appointments for children included in CDC's U.S. Zika Pregnancy and Infant Registry (USZPIR). During the ZHB, participants were examined by pediatric ophthalmologists, pediatric audiologists, and pediatric neurologists. We report the percentage of participants who were referred for additional follow-up care or given follow-up recommendations in the 2021 ZHB and compare these referrals and recommendations to those given in the 2018 ZHB. RESULTS: Thirty-three children born to mothers with laboratory evidence of ZIKV infection during pregnancy completed screenings at the 2021 ZHB, of which 15 (45%) children were referred for additional follow-up care. Ophthalmological screenings resulted in the highest number of new referrals for a specialty provider among ZHB participants, with 6 (18%) children receiving referrals for that specialty. Speech therapy was the most common therapy referral, with 10 (30%) children referred, of which 9 (90%) were among those who attended the 2018 ZHB. CONCLUSIONS: Thirty-three children in a jurisdiction with reduced access to healthcare specialists received recommended Zika-related pediatric health screenings at the ZHB. New and continuing medical and developmental concerns were identified and appropriate referrals for follow-up care and services were provided. The ZHB model was successful in creating connections to health services not previously received by the participants.

OCULAR MANIFESTATIONS OF PORETTI-BOLTSHAUSER SYNDROME: FINDINGS FROM MULTIMODAL IMAGING AND ELECTROPHYSIOLOGY.

BACKGROUND/PURPOSE: Poretti-Boltshauser syndrome is a rare, nonprogressive neurologic syndrome with characteristic cerebellar cysts on neuroimaging due to mutations in LAMA1. The ophthalmic findings in Poretti-Boltshauser syndrome are not well described. Here, we report the ophthalmic findings from multimodal imaging and electrophysiology of a patient with genetically confirmed Poretti-Boltshauser syndrome. METHODS: A 3-year-old boy with confirmed mutations in LAMA1 underwent examination under anesthesia with electroretinography and multimodal imaging including fundus photography, fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography. RESULTS: Dilated fundus examination was notable for retinal vascular anomalies, including a large area of nonperfusion in the temporal macula with corresponding retinal thinning on optical coherence tomography. There was an absence of a distinct foveal avascular zone and decreased density of both the superficial and deep vascular plexuses in the macula on optical coherence tomography angiography. There was diffuse loss of choriocapillaris architecture and decreased choroidal thickness. CONCLUSION: Patients with Poretti-Boltshauser syndrome may possess chorioretinal thinning and retinal vascular abnormalities appreciable on examination and multimodal imaging. These findings suggest a role for LAMA1 in retinal and choroidal vascular development.

Optical coherence tomography (OCT) measurements in black and white children with large cup-to-disc ratios.

Children with large optic nerve head cups often pose diagnostic difficulty due to concern over possible glaucoma. This study's purpose was to evaluate optical coherence tomography (OCT) measurement of the peripapillary retinal nerve fiber layer (RNFL) and macular thickness in healthy eyes of black and white children, comparing values for eyes with large cup-to-disc ratios against those with small cup-to-disc ratios (normal controls). Using Stratus OCT (OCT 3) (Carl Zeiss Meditec, Dublin, CA) a fast macular thickness and fast RNFL 3.4 protocol were performed on normal eyes of normal children. Included were children (aged 5-17 years) with normal ophthalmic examinations, drawn from data generated during an ongoing study of OCT in children. Excluded were eyes with corrected vision below 20/20, spherical equivalent >5 diopters, intraocular pressure >21 mm Hg, abnormal optic nerve rim, prematurity, family history of glaucoma. Clinical examination identified children with large cup-disc-ratios (0.5-0.8), and each was matched for age and race to a "normal control" with a small cup-to-disc ratio (<0.5). OCT-3 analyses were compared between eyes with optic nerve heads showing large cup-to-disc ratios (≥0.5) vs. those showing small cup-to-disc ratios (<0.5) for different sections of the macula and the peripapillary RNFL thickness. Analyses included OCT values and optic cup grading for 162 eyes of 162 children. White children with large cup-to-disc ratios had thinner mean RNFL thickness and macular volume compared to those with small cup-to-disc ratios (average RNFL = 100 ± 9 vs. 110 ± 8 µm, respectively, p = 0.0001, macular volume = 6.84 ± 0.41 vs. 7.03 ± 0.33 mm(3), respectively, p = 0.0186). On the other hand, black children with large cup-to-disc ratios had an equivalent mean RNFL thickness and macular volume compared to those with small cup-to-disc ratios average RNFL = 107 ± 10 vs. 107 ± 8 for both groups, macular volume = 6.81 ± 0.34 vs. 6.85 ± 0.28 respectively. Positive equivalence testing assumed a clinically significant difference of 10 µm for RNFL, and 0.2 mm(3) for macular volume. We report that white children with large cup-to-disc ratios have evidence of reduced/thinner RNFL and macular volume versus white children with smaller cup-to-disc ratios, as assessed by OCT. By contrast, this distinction was not found for black children in the present study.

Fixation switch and diplopia after full macular translocation surgery.

PURPOSE: We sought to identify and evaluate treatment of patients who switched fixation to the poorer-seeing eye and complained of persistent diplopia 6 to 12 months after full macular translocation surgery (MT360) and extraocular muscle surgery. METHODS: All patients enrolled in a prospective study undergoing MT360 and extraocular muscle surgery were included. All patients had sensorimotor examinations. Visual acuity and fixation preference between 6 and 12 months after MT360 and extraocular muscle surgery were analyzed. RESULTS: Preoperative median visual acuity was 20/100 in operated vs 20/640 in fellow eyes; after MT360, the values were similar to the preoperative values (n = 67). After MT360 but before extraocular muscle surgery, all patients preferred the fellow eye for ambulation. Six to 12 months after MT360, 58 of 67 (86%) patients fixated with the better-seeing eye (52 operated vs 6 fellow eyes); 4/67 (6%) fixated with the operated eye despite its poorer or equal visual acuity; 5 of 67 (8%) fixated with the poorer-seeing fellow eye, all 5 of whom experienced diplopia (ie, fixation switch and diplopia). Treatment of diplopia included Fresnel prism, additional extraocular muscle surgery, and occlusion. CONCLUSIONS: Fixation switch to the poorer-seeing eye can occur after MT360, despite a successful visual outcome in the operated eye and the diplopia is difficult to treat.

Optical coherence tomography as a tool for monitoring pediatric pseudotumor cerebri.

PURPOSE: To evaluate optical coherence tomography (OCT) measurement of peripapillary retinal nerve fiber layer (RNFL) and macular thickness in children with pseudotumor cerebri. METHODS: Prospective observational series of children with pseudotumor cerebri compared against controls matched for age and gender. We included 11 pediatric subjects with clinical pseudotumor cerebri and 37 normal controls. Subjects underwent a complete eye examination including stereo disk photographs and OCT. Peripapillary RNFL and macular thickness were evaluated using the Stratus OCT 3000. The peripapillary RNFL was evaluated with two protocols: Fast RNFL Map protocol with values divided in eight sections, made up of four quadrants, each with an inner and outer ring, and Fast RNFL Thickness (3.4) protocol, using values presented for four quadrants. A Fast Macular Thickness Map was also obtained, and results reported similarly included eight sections, made up of four quadrants, each with an inner and outer ring. RESULTS: The Fast RNFL Map protocol showed an increased RNFL thickness (pseudotumor cerebri vs controls) in the temporal and superior quadrants. The Fast RNFL Thickness (3.4) protocol showed a thicker average RNFL for pseudotumor cerebri eyes compared with controls (125.7 vs 106.5 microm, p < 0.0001). The Fast Macular Thickness Map showed an increased RNFL thickness in the nasal quadrant for the inner ring. The macular volumes for pseudotumor cerebri versus control eyes were 7.21 versus 6.97 mm(3), respectively (p = 0.005). CONCLUSIONS: OCT shows increased RNFL and macular thickness in pseudotumor cerebri and may be a useful clinical tool in these children.

Extraocular muscle surgery for extorsion after macular translocation surgery new surgical technique and clinical management.

PURPOSE: To report a new extraocular muscle surgery procedure for large-angle extorsion, and clinical management of subjective tilt and diplopia after full macular translocation (MT360). DESIGN: Consecutive retrospective case series. PARTICIPANTS: Seven patients with downward MT360 were evaluated after MT360, both before (preoperative) and after (postoperative) extraocular muscle surgery, with at least 6 months' follow-up. METHODS: Information gathered included demographics, visual acuity, ocular motility, torsion by Maddox rod, ocular history, and symptoms of visual disturbance. Surgery on extraocular muscles was performed based on the magnitude of torsion measured after MT360 surgery. MAIN OUTCOMES MEASURES: Maddox rod testing of torsion after MT360, and both preoperative and postoperative extraocular muscle surgery. RESULTS: Mean preoperative torsion was reduced from 45.4+/-11.3 degrees to 8.3+/-4.8 degrees (at 6 months after MT360) (P = 0.03). Extraocular muscle surgery slightly reduced the mean hypertropia of the operated eye (preoperative, 20+/-10 prism diopters [PD], vs. postoperative, 11+/-6 PD) (P = 0.06). Mean exotropia was affected minimally by extraocular muscle surgery (preoperative, 22+/-31 PD, vs. postoperative, 20+/-24 PD). Three patients required a second extraocular muscle surgery (performed on the fellow eye) to correct residual extorsion and diplopia. Overall, 85% (6/7) of patients were free of both diplopia and tilt after 1 or 2 extraocular muscle surgeries. CONCLUSIONS: Although our patients continued to have significant horizontal/vertical strabismus postoperatively, the extraocular muscle surgery performed was successful in reducing the torsional misalignment enough such that the remaining diplopia could be successfully ignored or suppressed.

Screening for amblyopia in preverbal children with photoscreening photographs: IV. Interobserver variability in photograph grading: origin and method of reduction.

BACKGROUND: Earlier detection of childhood vision disorders is a prominent goal of vision screening. The Medical Technology and Innovation (MTI) PhotoScreener addresses this objective. Use of this camera does not require verbal feedback and may be administered early in a child's development. Decreasing the variability in photograph grading results will boost the utility of any photoscreening system. This report aims to understand and to decrease intra- and inter-observer variability in grading photoscreening photographs. METHODS: We dissected the photograph grading process and quantified the intra- and inter-observer agreement using intraclass plot correlation coefficients. We evaluated the outcome of a two grader verification system vs. adjudicated measurements with Receiver Operator Characteristic (ROC) curves. PARTICIPANTS: Data on 955 children under 5 years of age, normal except for refractive error, each with complete photoscreening and eye examination data, culled from two previous studies. MAIN OUTCOME MEASURES: Intra- and inter-observer agreement in measuring bright crescent dimensions and pupillary diameters. Sensitivity and specificity of detection of hyperopia. RESULTS: Measurements of bright crescents are associated with greater variability than are measurements of pupillary diameters. Recognition and omission of light "rim" measurements from photograph grading will result in superior inter-observer agreement. Photograph independent errors increase variability and may be corrected by remeasurement. A verification system in which the most discrepant 5% of measurements are redone results in ROC curves similar to adjudicated dimension. CONCLUSIONS: We conclude: 1) two novices grading photographs can do as well as one expert in most cases; 2) the proposed grading methodology has undergone statistical validation and can be used in other areas of ophthalmology and medicine; and 3) inter-observer variability, one of the limitations of photoscreening photograph grading, can be reduced. For 95% of the photographs, two novices achieve similar true positive and true negative values with or without adjudication.

Management of ocular torsion and diplopia after macular translocation for age-related macular degeneration: prospective clinical study.

PURPOSE: To report the results of a prospective clinical series to evaluate the management of both torsion and diplopia in a large group of patients after full macular translocation (FMT) and extraocular muscle surgery. DESIGN: Prospective interventional case series. METHODS: Information gathered included demographic, visual acuity, ocular motility, torsion by Maddox rod, ocular history, and symptoms of visual disturbance. Surgery on two, three, or four extraocular muscles was performed based on the magnitude of torsion measured after FMT surgery. RESULTS: Fifty-three patients were included for both objective and subjective outcomes and were evaluated after FMT, both before (preoperative) and after (postoperative) extraocular muscle surgery. Preoperative torsion for two- (n = 6), three- (n = 8), and four-muscle (n = 39) surgery groups was 21.2 +/- 4.6, 30.0 +/- 6.3, and 40.5 +/- 8.7 degrees, respectively. At 6 months, postoperative residual torsion was significantly reduced in each group (to 3.4 +/- 3.2, 5.6 +/- 5.5, and 4.5 +/- 6.8 degrees, respectively, for two-, three-, and four-muscle groups). Muscle surgery reduced mean hypertropia from 17 +/- 6 prism diopters preoperative to 4 +/- 10 prism diopters postoperative (P <.0001); mean exotropia was mildly reduced from 20 +/- 9 prism diopters preoperative to 13 +/- 11 prism diopters postoperative (P <.01). Subjective data regarding diplopia and tilted vision after FMT and muscle surgery were available on an additional 10 patients (n = 53 + 10 = 63). Overall, 41% (26/63) of these were free of both diplopia and tilt, whereas only 5% (3/63) had both symptoms constantly. CONCLUSIONS: Extraocular muscle surgery is effective at relieving the variable amounts of torsion produced by FMT when graded to match preoperative torsion. The majority of patients were free of disabling tilt and diplopia after extraocular muscle surgery.

Surgical treatment of upgaze palsy in Parinaud's syndrome.

INTRODUCTION: Upgaze paralysis due to supranuclear defects can cause significant visual symptoms. There are only a few reports on the treatment of this problem in the literature. Patients with Parinaud's syndrome may also have retraction nystagmus and convergence on attempted upgaze that further complicates the treatment. METHODS: We performed a retrospective review of 48 patients with Parinaud's syndrome. Eleven patients were not able to elevate to the primary position and underwent surgery for the upgaze defect. Surgical treatments included transpositions in 2 patients, inferior rectus recessions in 8, which was combined with a superior rectus resection in one. RESULTS: Preoperative duction measurements demonstrated an average limitation on upgaze to -19 degrees (-10 to -30) below midline with zero representing midline. Postoperative measurements showed marked improvement to +19 degrees above midline (15 to 30). Inferior rectus recessions were as effective as transpositions in restoring function. In addition, there was a marked reduction in the retraction nystagmus and upgaze convergence as well. CONCLUSIONS: Visually significant upgaze limitation can be relieved with bilateral inferior rectus recessions in Parinaud's syndrome. Retraction nystagmus and convergence movements are also markedly improved.

Measurement of ocular torsion after macular translocation: disc fovea angle and Maddox rod.

PURPOSE: To compare two methods of measuring ocular torsion (the subjective Maddox rod [MR] test versus the objective disc-fovea angle [DFA] test) after macular translocation surgery. METHODS: Ocular torsion was measured on consecutive patients after macular translocation at Duke University Eye Center between August 2001 and April 2002. Both MR and DFA measurements of torsion were made at the same clinic visit 4 to 8 weeks after the translocation surgery and again within 3 months after extraocular muscle surgery to decrease torsion. MR and DFA measurements were each performed by a separate examiner who was blinded to the results of the other method. RESULTS: Thirty-five patients (35 eyes) were included for evaluation. Twenty-nine of these patients had intorsion measured by both MR and DFA after macular translocation but before extraocular muscle surgery (MR mean of 40.3 + 7.2 degrees v DFA mean of 47.0 + 7.9 degrees [P <.001]). The intrapatient reproducibility of the MR test was high (using four readings per session), with a mean coefficient variation of 4.8%. Twenty-five patients had residual torsion measured by both MR and DFA after extraocular muscle surgery (MR mean of 4.2 + 4.7 degrees v DFA of mean 4.8 + 7.0 degrees). There was good correlation between MR and DFA measurements of torsion (r(2) = 0.9). CONCLUSIONS: DFA measurement correlates well with MR measurement of torsion in patients after full macular translocation. This study verifies the reproducibility of MR to measure large angles of torsion and offers DFA as a simple corroborative test for measuring ocular torsion in patients with poor vision or cooperation.

Optical coherence tomography in paediatric glaucoma: time domain versus spectral domain.

BACKGROUND/AIMS: Spectral-domain (SD)- Optical Coherence Tomography (OCT) can track eye movements, has faster acquisition time and higher resolution than time-domain(TD)-OCT. The aim of the study was to assess the utility of SD-OCT in paediatric glaucoma and determine its agreement with TD-OCT. METHODS: Children who had SD-OCT(Spectralis, Heidelberg-Engineering,Germany) were retrospectively and prospectively identified from Duke paediatric glaucoma clinic. The peripapillary retinal nerve fibre layer (RNFL) and macular thickness and volume (MV) were compared amongst four groups: normal eyes, eyes with physiologic cupping (C:D >0.5 and <0.8, IOP <21), mild glaucomatous eyes (C:D <0.5, intra-ocular pressure (IOP) >21) and severe glaucoma (C:D>0.5, IOP>21). SD-OCT values were compared to TD-OCT(OCT-3, Carl-Zeiss-Meditec, Dublin, CA) values in a subset of subjects who had same day scans using both instruments. Children with neurologic disorders, refractive error >±5D, pseudophakia and prematurity were excluded. RESULTS: Included were 83 eyes of 83 children, mean age 11.9 ± 4.2 years. SD-OCT measurements of average RNFL thickness and MV differed among normals(n=24), physiologically cupped (n=31), mild (n=15) versus severe glaucoma (n=13): (RNFL:104 ± 9, 99 ± 6, 98 ± 9 vs 62 ± 18 µm, respectively, p<0.05; MV: 8.7 ± 0.3, 8.6 ± 0.3, 8.8 ± 0.4 vs 8.0 ± 0.6 mm³;, respectively, p<0.05). Same-day SD-OCT and TD-OCT measures in 53 eyes correlated linearly (RNFL r(2)=0.88; MVr(2)=0.58). SD-OCT measured lower RNFL and higher macular thickness than TD-OCT. Among eyes with severe glaucoma, 4 of 13 (30%) had unreliable TD-OCT but reliable SD-OCT. CONCLUSIONS: SD-OCT was easier to obtain than TD-OCT in children. SD-OCT and TD-OCT measurements correlated, but values were not interchangeable.

Diplopia after strabismus surgery.

The presence of diplopia is an undesirable result following strabismus surgery. There are a variety of scenarios where diplopia exists prior to strabismus surgery, and, after surgery, has either been alleviated or decreased to a magnitude amenable to prism correction. In other cases, the patient does not experience diplopia prior to the strabismus surgery, but there exists a definite risk of diplopia after the surgery. In the current review, I examine the literature to help determine the incidence of diplopia after strabismus surgery.

Longitudinal reproducibility of optical coherence tomography measurements in children.

PURPOSE: To evaluate the longitudinal reproducibility of optical coherence tomography (OCT) measurements in normal and glaucomatous eyes of children. METHODS: In this 2-setting prospective study, OCT-3 was used to obtain fast retinal nerve fiber layer (RNFL) and macular thickness scans. In the first study setting, the normal eyes of healthy children were scanned on presentation, at 2 weeks, and 3 years, with axial length measured at the first and last examinations. In the second setting, OCT scans of patients in the pediatric glaucoma clinic were performed over 4 years as clinically indicated. Eyes were classified as "normal" (normal eyes and those with physiologic cupping but normal intraocular pressure [IOP]); "mild glaucoma" (increased IOP and a normal optic nerve appearance); or "advanced glaucoma" (severe cupping or progressive glaucoma). Intraclass correlation coefficients were used to evaluate the reproducibility of measurements on the same day and over time. RESULTS: In the first setting, 8 normal eyes were included. Axial length increased 0.11 ± 0.04 mm/year over an average of 3.3 years (P = 0.03); there was no statistically significant change in RNFL thickness (P = 0.30). In our second setting, 27 normal eyes and 37 eyes with glaucoma were included. Intraclass correlation coefficients across the 3 visits for total macular volume were 0.80-0.91 and for average RNFL were 0.73-0.95. CONCLUSIONS: Global OCT measurements in children were reproducible over years and were not affected by normal increase in axial length. OCT shows promise as an objective tool for longitudinal assessment of children.

Quantifying vertical angle kappa after macular translocation surgery: a new use for the synoptophore.

PURPOSE: To report the measurement of vertical angle kappa and its application in patients after full macular translocation (MT360) and extraocular muscle surgery (to correct the resultant large angle torsion), in a prospective study. METHODS: A custom slide was designed to quantify vertical angle kappa using the synoptophore. The measurement of the vertical angle kappa was performed in the eye that had the MT360, after the macular translocation surgery, extraocular muscle surgery (to correct torsion), and silicone oil removal, all in the same translocated eye. Information gathered included demographic, visual acuity, ocular motility, torsion, past ocular history, and synoptophore examination. RESULTS: Fifty-three subjects met the inclusion criteria (patients who underwent upward MT360, with silicone oil tamponade and extraocular muscle surgery from 3/1999 to 6/2003, and who had visual acuity better than 20/200). Twenty-nine of these subjects were able to complete angle kappa measurement. The mean vertical angle kappa was 4.6 +/- 1.4 degrees (n = 29) measured after MT360 and extraocular muscle surgery. The magnitude of vertical angle kappa did not correlate with either the amount of torsion after MT360, or the limitation of eye movement after both MT360 and extraocular muscle surgery. CONCLUSIONS: Vertical angle kappa can be measured using the synoptophore, and is increased above normal in eyes after MT360 and extraocular muscle surgery.