Assessment of Macular Microvasculature in Healthy Eyes of Infants and Children Using OCT Angiography.

PURPOSE: To assess macular vasculature in healthy infants and children using OCT angiography (OCTA). DESIGN: Prospective cross-sectional study. PARTICIPANTS: One hundred thirty-five normal maculae of 89 healthy infants and children (mean age, 8.5±5.3 years; range, 9 weeks-17 years) treated at the Duke University Eye Center. METHODS: We imaged 135 maculae of 89 pediatric patients using the standard Spectralis tabletop and investigational Spectralis with Flex module devices, both equipped with investigational OCTA software (Heidelberg Engineering, Heidelberg, Germany). OCT angiography images of the superficial vascular complex (SVC) and deep vascular complex (DVC) were analyzed for foveal avascular zone (FAZ) area and superficial and deep vessel density. We assessed effects of age, gender, race, axial length (AL), and central subfield thickness on FAZ and vessel density. Patients with both eyes imaged were assessed for agreement between the FAZ and vessel densities of the left and right eyes. MAIN OUTCOME MEASURES: The FAZ area, as well as vessel area density (VAD) and vessel length density (VLD) in the SVC and DVC. RESULTS: The FAZ varied significantly with race; white patients showed a significantly smaller FAZ than black patients (mean difference, 0.11 mm2; P = 0.004). The FAZ did not vary with age, gender, or AL (P > 0.05). In the SVC, VAD and VLD varied significantly with age (P < 0.001) and AL (R2 = 0.46; P < 0.001) but not gender (P > 0.05). The SVC VLD was significantly different between races and ethnicities (P = 0.037), but VAD was not (P < 0.05). In the DVC, VAD and VLD also varied significantly with age (P < 0.001) and AL (R2 = 0.46; P < 0.001) but not gender or race (P > 0.05). There was excellent agreement between the right and left eyes for FAZ (intraclass correlation [ICC], 0.97), SVC VLD (ICC, 1.00), and DVC VLD (ICC, 1.00). CONCLUSIONS: Quantitative studies of pediatric perifoveal vasculature should consider age, race, and AL. In eyes with unilateral disease, the perifoveal vasculature in the unaffected eye may be used as a control comparison because there is excellent agreement between eyes.

Improvement in health-related quality of life following strabismus surgery for children with intermittent exotropia.

PURPOSE: To investigate health-related quality of life (HRQOL) after strabismus surgery in children with intermittent exotropia (IXT) and relationships between HRQOL and surgical success. METHODS: A total of 197 children with IXT aged 3-11 years (and 1 parent of each child) were enrolled in a previously reported randomized clinical trial comparing two surgical procedures. The Intermittent Exotropia Questionnaire (IXTQ) was administered before surgery (baseline), and again at 6 and 36 months following surgery. The child version of the IXTQ was only completed by children 5-11 years of age (n = 123). Outcomes were classified as "resolved" (exodeviation of <10Δ, no decreased stereoacuity, and no other nonsurgical treatment for IXT or reoperation), "suboptimal" (exotropia ≥10Δ by simultaneous prism and cover test or constant esotropia ≥6Δ or loss of ≥2 octaves of stereoacuity), or "intermediate." Mean changes in Rasch-calibrated IXTQ domain scores (Child, Proxy, Parent-psychosocial, Parent-function, and Parent-surgery; converted to a 0-100 scale) were compared. RESULTS: Overall, mean IXTQ domain scores improved for all domains from baseline to 36 months after surgery, ranging from 10.7 points (Child IXTQ; P < 0.0001) to 34.5 points (Parent-surgery IXTQ; P < 0.0001). At 36 months after surgery, 62 (39%) children had resolved IXT, whereas 38 (24%) had suboptimal outcome. Greater improvement was found in all mean domain scores with resolved IXT (range, 19.8-46.0 points) compared with suboptimal outcome (all comparisons P < 0.05). CONCLUSIONS: Successful surgery for childhood IXT results in measurable improvement in a child's quality of life, in parental assessment of the child's quality of life, and in quality of life for the parent.

Health-related quality of life in children with untreated intermittent exotropia and their parents.

PURPOSE: To determine whether health-related quality of life (HRQOL) scores improved or worsened over 3 years of observation in childhood intermittent exotropia without treatment. METHODS: A total of 111 children aged 3-11 years with intermittent exotropia were assigned to observation in a previously reported randomized trial comparing patching with observation. The intermittent exotropia questionnaire (IXTQ) was administered at baseline, 6 months, and 36 months. Rasch-calibrated IXTQ domain scores (Child, Proxy, Parent-psychosocial, Parent-function, and Parent-surgery) were compared between time points. The Child IXTQ was administered only to children ≥5 years of age (n = 78). RESULTS: Overall, Child IXTQ and Proxy IXTQ scores showed no significant change over 36 months (mean improvement from baseline to 36 months of 3.2 points [95% CI, -1.9 to 8.2] and -2.4 points [95% CI: -7.9 to 3.1], resp.). By contrast, Parent-psychosocial, Parent-function, and Parent-surgery domain scores all improved over 36 months (mean improvements of 12.8 points [95% CI, 5.9-19.6] and 14.2 points [95% CI, 8.0-20.3] and 18.5 points [95% CI, 9.7-27.3], resp.). CONCLUSIONS: HRQOL of children with intermittent exotropia remains stable with observation over 3 years (by both child and proxy report), whereas parental HRQOL improves.

The effect of nonmodifiable physician demographics on Press Ganey patient satisfaction scores in ophthalmology.

Press Ganey patient satisfaction scores are increasingly being used as a physician quality metric. In this retrospective review of over 25,000 patient surveys for 61 ophthalmologists, pediatric ophthalmologists and neuro-ophthalmologists received significantly lower patient satisfaction scores than their peers, suggesting that the problem for which a patient seeks care may affect whether he or she is satisfied with the care received. These findings should be taken into account when considering the validity of Press Ganey scores as an equitable, modifiable measure of physician performance, especially when considering factoring these scores into physician evaluation and reimbursement.

The flipped-classroom approach to teaching horizontal strabismus in ophthalmology residency: a pilot study.

PURPOSE: To compare the flipped classroom (home pre-taped lectures followed by in-class group exercise) to the traditional classroom (home reading assignment followed by in-class lecture) for horizontal strabismus didactics in ophthalmology residency. METHODS: All PGY2-4 residents from four U.S. ophthalmology residencies without prior residency flipped-classroom experience were invited to esotropia and exotropia sessions sequentially, with random order and assignment to flipped and traditional classrooms. Content test scores before and after the two classrooms were compared. Surveys were administered to assess participant experience. RESULTS: A total of 40 residents attended each session. Likert scale evaluation of preparatory material and classroom activity did not differ between sessions; however, divided by year of training, 70% of senior residents (PGY3-4) and 39% of first-year (PGY2) residents preferred the flipped classroom over the traditional classroom. Pre- and post-test scores for the flipped classroom exceeded those of the traditional classroom for the exotropia course (P = 0.01 and P = 0.001, resp.) but not for the esotropia course. There was significant improvement between pre- and post-tests for both styles of learning. CONCLUSIONS: The flipped classroom had a favorable effect on test scores for only one of the two strabismus subjects but was preferred over the traditional classroom among PGY3-4 residents.

Postoperative infection following strabismus surgery: case series and increased incidence in a single referral center.

PURPOSE: To identify and analyze cases of postoperative infection following strabismus surgery at a large referral center and to report the incidence, risk factors, and outcomes. METHODS: An electronic database search identified strabismus procedures at Duke Eye Center from July 1996 to October 2017. Diagnosis codes for periocular infections were used to further identify patients with possible infections following strabismus surgery. RESULTS: Of 9,111 strabismus surgeries, 13 (0.14%) met criteria for probable infection, all occurring since October 2012 (0/6580 before vs 13/2531 [0.51%] after; P < 0.0001). Mean age of infection cases was 11.4 years; 11 patients (85%) were under 18 years of age. Associated previous diagnoses were genetic abnormalities with associated developmental delay (n = 5 [38%]), previous skin or ear infection (n = 4 [31%]), and acute or chronic rhinitis (n = 3 [23%]). Infection site cultures revealed methicillin-resistant Staphylococcus aureus (n = 3 [23%]), methicillin-sensitive S. aureus (n = 3 [23%]), and Streptococcus pyogenes/group-A Streptococcus (n = 2 [15%]). Only 1 case had bilateral infection. Infection remained extraocular in all cases, but one eye lost light perception secondary to optic atrophy. No common surgeon/procedure/preparation-related risks were identified. CONCLUSIONS: A unifying explanation for the increase in post-strabismus surgery infections at Duke Eye Center was not identified. Potential risk factors include age <18 years, developmental delay, immune compromise, preceding nonocular infection, and bacterial colonization.

Optical Coherence Tomography Normative Peripapillary Retinal Nerve Fiber Layer and Macular Data in Children 0-5 Years of Age.

PURPOSE: To determine reference values for the peripapillary retinal nerve fiber layer (pRNFL) and macula in children 0-5 years of age. DESIGN: Prospective cross-sectional study. METHODS: This study was set in a single large academic pediatric ophthalmology practice. Healthy, full-term children 0 to <6 years of age presenting for surgery under general anesthesia were prospectively recruited for participation. Excluded were children with systemic neurologic disease, optic nerve or retinal disease (even if unilateral) or any bilateral ocular disease process, and eyes with amblyopia, ocular disease, or spherical equivalent refractive error outside of -3.00 to +8.00 diopters. Following general anesthesia, OCT scans of the optic nerve and retina were acquired using an HRA+OCT Spectralis with Flex module (Heidelberg Engineering). Automated segmentation of the pRNFL and retinal layers was followed by manual correction. RESULTS: Data were obtained from normal eyes of 57 participants (mean age 2.28 ± 1.50 years). Mean global pRNFL thickness was 107.6 ± 10.3 µm. Mean global pRNFL thickness was not dependent on age but showed a negative relationship with axial length (P = .01). The mean total macular volume was 8.56 ± 0.259 mm3 (n = 38). No relationship was found between total macular volume and age. Ganglion cell layer, ganglion cell complex, and inner nuclear layer volumes showed an inverse relationship with age while the photoreceptor layers showed a logarithmic increase with age. CONCLUSIONS: Global pRNFL thickness measurements remain stable over time. Macular volume and thickness values of segmented retinal layers reflect the development of the macula with age.

Safety and efficacy of silicone rod frontalis suspension surgery for childhood ptosis repair.

PURPOSE: To evaluate the safety and efficacy of silicone rod frontalis suspension surgery for childhood ptosis. METHODS: The authors retrospectively studied 89 consecutive children (110 eyelids) who had silicone rod frontalis suspension surgery for ptosis at Duke University Eye Center from 1983 to 2004. Marginal reflex distance1 (MRD1) elevation of 2 mm or more (vs preoperative MRD1) was considered satisfactory. MRD1 was measured as the vertical distance from the corneal light reflex in primary gaze to the upper eyelid margin. The postoperative eyelid symmetry (< or = 1 mm = satisfactory) was the difference between the MRD1 of the surgical and fellow eyelid. RESULTS: Median age at surgery was 45 months (range: 3 to 223 months) and median follow-up was 17 months (range: 1 to 88 months). Ptosis types (number of eyelids) were unilateral congenital (53), bilateral congenital (30), third nerve palsy (16), Marcus Gunn jaw wink (7), trauma (2), and myasthenia gravis (2). Median MRD1 elevation was 2 mm or greater for all ptosis types, whereas satisfactory postoperative symmetry occurred in 60% of unilateral and 100% of bilateral congenital ptosis cases (last follow-up). Complications occurred in 10 eyelids (9%) and reoperation occurred in 10 eyelids (9%). CONCLUSION: The use of silicone rod frontalis suspension surgery for ptosis repair in pediatric patients is modestly effective, with few complications and easy removal and adjustment.

Microphthalmia, Dermal Aplasia, and Sclerocornea Syndrome: Endoscopic Cyclophotocoagulation in the Management of Congenital Glaucoma.

PURPOSE: To report on the use of endoscopic cyclophotocoagulation (ECP) to treat congenital glaucoma in a triple X female with microphthalmia, dermal aplasia, and sclerocornea (MIDAS) syndrome. OBSERVATIONS: The patient demonstrated linear streaks on the face and neck consistent with dermal aplasia. The corneas were scleralized with ectatic areas of corneal thinning, and the eyes were microphthalmic. Ultrasound biomicroscopy demonstrated congenital aphakia and iris stumps. The patient had elevated intraocular pressure (IOP) that responded to topical glaucoma therapy in the right but not the left eye. Intraoperative endoscopy of the posterior segment revealed multiple hypopigmented chorioretinal lacunae surrounding a pale, cupped optic nerve. ECP of the ciliary processes in the left eye led to marked improvement in IOP. CONCLUSIONS AND IMPORTANCE: Patients with MIDAS syndrome can develop congenital glaucoma secondary to angle dysgenesis. This is the first case report to demonstrate the safe and effective use of ECP to treat elevated IOP in a patient with MIDAS.

Central corneal thickness: congenital cataracts and aphakia.

PURPOSE: To evaluate central corneal thickness (CCT) in normal children (controls) and in those with cataracts, pseudophakia, and aphakia. DESIGN: Prospective, observational case series. METHODS: CCT was measured in 369 eyes of 223 children. Subjects with glaucoma, anterior segment abnormalities, or intraocular pressure of more than 30 mm Hg were excluded. Group means were compared for controls and for eyes with pediatric cataracts, pseudophakia, and aphakia. RESULTS: The mean CCT of eyes with cataracts was more than that of controls (574 +/- 54 microm [n = 46] and 552 +/- 38 microm [n = 230], respectively; P = .001). After excluding from the cataract group those eyes with aniridia, Down syndrome, Marfan syndrome, or glaucoma surgery, the mean CCT (564 +/- 34 microm [n = 36]) was no longer greater than that of controls (P = .07). The mean CCT of pseudophakic eyes (598 +/- 56 microm [n = 29]) was greater than the mean CCT of controls (P < .001) and was similar to the mean CCT of eyes with cataracts (P = .06). The mean CCT of aphakic eyes (642 +/- 88 microm [n = 64]) was greater than the mean CCT of controls (P < .001), eyes with cataracts (P < .001), and eyes with pseudophakia (P = .003). CONCLUSIONS: In the absence of factors known to affect CCT (Down syndrome, Marfan syndrome, and aniridia), CCT is similar in eyes with pediatric cataracts and normal controls and increases after cataract surgery.

A double-masked randomized trial of postoperative local anesthetic for pain control in pediatric strabismus surgery.

PURPOSE: To determine to what extent local anesthetic reduces postoperative pain after pediatric strabismus surgery. METHODS: In this double-masked, randomized clinical trial of 50 children 13-91 months of age undergoing strabismus surgery, subjects were randomly assigned to one of three treatments given at the conclusion of surgery: topical lidocaine gel and sub-Tenon's (balanced salt solution) placebo (n = 16), topical placebo (hypromellose) and sub-Tenon's bupivacaine 0.75% (n = 17), or topical and sub-Tenon's placebo (n = 17). Pain was otherwise managed systemically in the usual fashion by the masked anesthesia team and assessed at regular postoperative intervals by a masked observer using an objective, validated pain scale. RESULTS: Average pain in the first 30 minutes was 6.57, 6.36, and 6.58 in the lidocaine, bupivacaine, and placebo groups, respectively, and was significantly lower (P = 0.016) for bupivacaine vs placebo. The bupivacaine group had significantly lower scores for pain after 30 minutes, total pain, and peak pain versus the lidocaine group. CONCLUSIONS: Sub-Tenon's bupivacaine may reduce postoperative pain in children undergoing strabismus surgery.

Multifocal iris melanoma in a 2-year-old managed with I-125 plaque brachytherapy.

We report a 23-month-old patient presenting with multifocal iris melanoma who underwent plaque brachytherapy with full corneal coverage. The lesion demonstrated several high-risk clinical and histopathologic features associated with iris melanoma in adults, including growth and angle seeding. The patient has been subsequently followed for 3.5 years with no evidence of recurrence. This report demonstrates the importance of serial examination of suspected melanocytic iris lesions in very young children and the effective treatment option of globe-sparing radiation therapy.

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.

Central corneal thickness in children: stability over time.

PURPOSE: To evaluate central corneal thickness (CCT) in children over time. DESIGN: Prospective observational case series. METHODS: CCT was measured by ultrasonic pachymetry in 69 eyes of 38 subjects age 3 to 14 years recruited from the Duke University Eye Center. Subjects included patients with and without glaucoma. RESULTS: For eyes on no glaucoma medication, the mean change in CCT was -1.9 +/- 14 microm (n = 30; mean time between CCT measurements, 567 days). For eyes on stable glaucoma medication, the mean change in CCT was +8.1 +/- 26 microm (n = 27; mean time between CCT measurements, 580 days). For eyes with a change in medical therapy between visits, the mean change in CCT was -3.8 +/- 24 microm (n = 8, mean number of days between visits 723). Four eyes underwent trabeculectomy between measurements and experienced a mean change in CCT of -9.0 +/- 6 microm with a mean of 1257 days between measurements. These CCT changes were not statistically significant. CONCLUSIONS: CCT of children ages 3 to 14 years appears stable over a one- to two-year interval.

Central corneal thickness in children: Racial differences (black vs. white) and correlation with measured intraocular pressure.

PURPOSE: To test the hypotheses that the mean central corneal thickness (CCT) of healthy black children is thinner than that of healthy white children and to confirm the relationship between increased CCT and measured intraocular pressure (IOP) in children. METHODS: This prospective observational case series included 178 eyes of 92 children aged 9 months to 17 years without anterior segment abnormalities or a clinical diagnosis of glaucoma. CCT was measured by ultrasonic pachymetry and IOP was measured by Goldmann or Tono-pen technique. RESULTS: The mean CCT for the 102 eyes of 52 white children was 562+/-35 microm versus 543+/-37 microm for the 66 eyes of 35 black children (P = 0.02). There was a positive relationship between CCT and IOP (P = 0.0002). For every 100 mum increase in CCT, the IOP increased by 2.2+/-0.6 mm Hg. CONCLUSIONS: The mean CCT of black children is thinner than that of white children. There is a positive relationship between increasing measured IOP and CCT among children with normal corneas and anterior segments. The interpretation of elevated IOP in eyes with abnormal anterior segment anatomy and thickened corneas awaits further study.

Macular and retinal nerve fiber layer analysis of normal and glaucomatous eyes in children using optical coherence tomography.

PURPOSE: To evaluate macular and nerve fiber layer (NFL) thickness in normal and glaucomatous eyes of children 3 to 17 years old using optical coherence tomography (OCT-3). DESIGN: Observational cross-sectional study. METHODS: One hundred fifty-six eyes of 79 patients were enrolled in this institutional study. Fifty-two eyes (33.3%) met criteria for glaucoma and 104 (66.7%) were normal. There were 44 female (55.6%) and 35 male (44.3%) subjects whose ages ranged from 3 to 17 years old (mean 9.5 years, standard deviation 3.5 years, median 9 years). The OCT-3 (Carl Zeiss Meditec, Dublin, California) was used to obtain a fast macular thickness map as well as a fast retinal NFL map of each eye. Data from specific locations around the macula, as well as total macular volume, was analyzed. Similarly, the retinal NFL scan reports average NFL thickness from specific locations around the optic nerve. Data from the superior temporal and inferior temporal sections was analyzed. RESULTS: There was a statistically significant difference in macular thickness and NFL thickness when normal eyes were compared against those with glaucoma, in all quadrants studied (all P values

A novel method for teaching key steps of strabismus surgery in the wet lab.

The surgical laboratory environment plays an important role in modern surgical education. Learning how to complete the key steps of strabismus surgery prior to actual surgery may enhance efficiency and sensitize the surgeon to potential complications. Cadaveric extraocular muscle is difficult to manipulate and specimens are not readily available; therefore, an alternative is needed to teach strabismus surgery techniques. We developed a wet lab to practice the key steps of strabismus surgery using commercially available bacon as an extraocular muscle substitute and cadaveric pig eyes for conjunctival and scleral tissue in a framework of peer teaching, expert supervision, and validated written feedback.

Latanoprost for the treatment of pediatric glaucoma.

Latanoprost is a prostaglandin F(2alpha) analog that reduces intraocular pressure (IOP) by 20-40% in adults with open-angle glaucoma or ocular hypertension. The efficacy and safety of latanoprost in children has not been widely reported, but there are at least three peer-reviewed publications involving the use of this drug in the treatment of pediatric patients with glaucoma. Most of the patients in these studies showed disappointingly little IOP effect from this drug, but some children, particularly older children and those with juvenile-onset open-angle glaucoma, do have a significant ocular hypotensive effect with latanoprost. Systemic and ocular side effects in children on latanoprost are infrequent and mild.

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.