VISUAL FUNCTION AND OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FEATURES IN CHILDREN BORN PRETERM.

PURPOSE: Preterm children have an increased risk of impaired vision from retinopathy, strabismus, and high refractive error. The aim of this study was to investigate the relationship between foveal parameters generated by optical coherence tomography angiography and visual function in preterm children. METHODS: Eighty eyes (32 eyes of former preterm infants and 48 age-matched full-term control eyes) were analyzed. Subjects underwent complete eye examinations including best-corrected visual acuity and retinal imaging with the Optovue XR Avanti optical coherence tomography angiography device. Foveal morphologic parameters including foveal depth, central foveal thickness, inner retinal area, and outer retinal area were measured on a central horizontal B-scan. Foveal vasculature parameters including foveal avascular zone, superficial capillary plexus-vessel density, and deep capillary plexus-vessel density were measured on optical coherence tomography angiography. RESULTS: The best-corrected visual acuity was significantly affected in preterm children compared with controls (P < 0.0001). The central foveal thickness (P < 0.0001), inner retinal area (P = 0.01), and outer retinal area (P = 0.03) were significantly increased in preterm compared with control eyes. Foveal depth (P < 0.001) and foveal avascular zone (P < 0.001) were significantly decreased in preterm compared with control eyes. The superficial capillary plexus-vessel density (P = 0.01) and deep capillary plexus-vessel density (P = 0.003) at the fovea (1 mm) were significantly increased in preterm compared with control eyes. The best-corrected visual acuity was negatively correlated with foveal depth (r = -0.42, P = 0.001) and foveal avascular zone (r = -0.53, P < 0.001), and positively correlated with central foveal thickness (r = 0.32, P = 0.01) and inner retinal area (r = 0.32, P = 0.01), indicating that worse visual acuity was associated with a smaller foveal avascular zone, shallower foveal depth, increased central foveal thickness, and larger inner retinal area. CONCLUSION: Foveal morphology and vasculature changes in preterm children were associated with impaired visual function. Further longitudinal studies are required to evaluate these changes over time.

MACULAR MICROVASCULAR NETWORKS IN HEALTHY PEDIATRIC SUBJECTS.

PURPOSE: To report optical coherence tomography angiography (OCTA) values in healthy pediatric eyes and to identify factors that may modify these values. METHODS: In this prospective observational cross-sectional study, macular OCTA images were acquired from healthy pediatric patients. Main outcome measures were 1) foveal avascular zone (FAZ) area at the level of the superficial retinal capillary plexus (SCP); 2) SCP and deep retinal capillary plexus (DCP) perfusion density (based on the area of vessels); 3) SCP and DCP vessel density (based on a map with vessels of 1-pixel width); and 4) CC perfusion density. Multiple regression analysis was performed to assess the effect of age, sex, ethnicity, refraction, and foveal macular thickness (FMT) on OCTA parameters. RESULTS: Seventy-seven eyes from 52 subjects (23 male and 29 female) were included in analysis. Mean age was 11.1 ± 3.3 years (range = 5.0-17.0 years). Twenty-nine (55.8%) subjects were white, 14 (27.0%) Hispanic, 8 (15.4%) Asian, and 1 (1.8%) African-American. Mean refraction was -0.1 ± 2.4 diopters (D) (range = -5.75 to +9.0 D). Mean FMT was 248.6 ± 18.6 µm. Larger FAZ area was significantly associated with older age (P = 0.014). Furthermore, larger FAZ area was associated with reduced FMT (P < 0.0001). Male sex was associated only with increased SCP perfusion density (P = 0.042). Increased CC perfusion density was associated with younger age (P = 0.022). CONCLUSION: We report data for pediatric OCTA parameters in healthy subjects. Several variables influence the density of macular microvascular networks, and these factors should be considered in the OCTA study of pediatric eye disorders.

Increased choriocapillaris vessel density in amblyopic children: a case-control study.

PURPOSE: To investigate the choriocapillaris in children with amblyopia, using optical coherence tomography angiography (OCT-A). METHODS: Patients with amblyopia and age-matched controls were prospectively imaged using OCT-A. On OCT-A, the choriocapillaris measures 30 µm starting 31 µm posterior to the retinal pigment epithelium. The section of choriocapillaris under superficial retinal vessels was excluded from analysis to avoid shadowing or projection artifacts. The main outcome measure was choriocapillaris vessel density. Secondary outcome measures were foveal macular thickness and parafoveal macular thickness. RESULTS: A total of 20 eyes of 16 patients with amblyopia and 25 eyes of 25 controls were included. Mean age of amblyopic subjects was 7.6 ± 3.6 years; of controls, 9.3 ± 2.2 years (P = 0.10). Mean refractive error of subjects was 4.3 ± 6.2 D; of controls, 0.0 ± 1.6 D (P = 0.004). Mean choriocapillaris vessel density was 74.8 ± 5.8 in the amblyopic group and 71.1 ± 3.6 in the control group, which was significant even after adjusting for age and refractive error (P = 0.012). There was no difference between groups in foveal macular thickness or parafoveal macular thickness; however, outer parafoveal macular thickness (the inner boundary of the inner nuclear layer to the retinal pigment epithelium outer boundary) was significantly greater in amblyopic eyes than in control eyes, even after adjusting for age and refractive error (203 ± 11 µm and 189 ± 12 µm, resp. [P = 0.014]). CONCLUSIONS: In our study cohort, amblyopic eyes were found to have increased choriocapillaris vessel density as well as a greater outer parafoveal macular thickness, which may be due to alterations in outer retinal maturation.

MRI findings of contralateral oculomotor nerve palsy in Parry-Romberg syndrome.

PURPOSE: To study a case of right Parry Romberg syndrome associated with contralateral oculomotor nerve palsy using high-resolution cerebral and orbital magnetic resonance imaging (MRI). OBSERVATIONS: There were no brain MRI abnormalities. However, there was marked enophthalmos on the right with reduction of orbital fat. Extraocular muscles contracted normally in the right eye, but in the left eye, there was reduced contractility of the medial, inferior, and superior rectus muscles. The intraorbital motor nerves were unremarkable bilaterally, and the right optic nerve was tortuous. CONCLUSIONS AND IMPORTANCE: Parry Romberg syndrome is a disease of unknown etiology with various ophthalmologic manifestations. This case study contributes extensive MRI data to the limited literature on ophthalmological anatomic findings in a patient who had Parry Romberg syndrome with contralateral paralytic strabismus.

Spectral-Domain Optical Coherence Tomographic Angiography in Children With Amblyopia.

Importance: Amblyopia is the most common cause of visual impairment in childhood, with a prevalence of 1% to 4% in children in the United States. To date, no studies using noninvasive optical coherence tomographic angiography (OCTA) have measured blood flow in the retinal capillary layers in children with amblyopia. Objective: To evaluate the retinal and microvascular features using OCTA in children (<18 years) with amblyopia. Design, Setting, and Participants: This observational case-control study enrolled patients from September 1, 2016, through May 31, 2017, and was conducted from September 1, 2016, through June 30, 2017, at the Stein Eye Institute at UCLA (University of California, Los Angeles). Participants included 59 children (<18 years) with amblyopia and without amblyopia examined at a pediatric ophthalmology clinic or referred to the clinic by coinvestigators. All patients underwent comprehensive ophthalmological examination, including visual acuity, refraction, and ocular motility tests; anterior and posterior segment examination; and OCTA. Main Outcomes and Measures: Reduced superficial and deep retinal capillary vessel density on OCTA. Results: Of the 63 eyes evaluated, 13 (21%) were amblyopic and 50 (79%) were control eyes. Of the 59 patients, the mean (SD) age of patients with amblyopia was 8.0 (4.0) years and 10.3 (3.3) years for the controls; 33 patients (56%) were female; and 5 of 13 (39%) and 27 of 46 (54%) patients in the amblyopic and control groups, respectively, were identified as white. The macular vessel density of the superficial capillary plexus was lower in the amblyopic group than in the control group in both 3 × 3-mm and 6 × 6-mm scans. After adjusting for age and refractive error, the mean (SD) difference in the superficial capillary plexus in the 6 × 6-mm scan was statistically significant (49.3% [4.1] vs 51.2% [2.9]; P = .02). Macular vessel density of the deep capillary plexus in the 6 × 6-mm scans was also considerably different between groups: mean (SD) vessel density of the deep retinal capillary plexus was 54.4% (4.7%) in the amblyopia group and 60.1% (3.3%) in the control group, with a difference of 5.7% (95% CI, 3.4%-8.1%; P = .002). Conclusions and Relevance: The study found that OCTA reveals subnormal superficial and deep retinal capillary density in the macula of patients with amblyopia. Further studies are needed to determine the clinical relevance of this finding.

Pediatric Uveitis: Experience in Colombia.

PURPOSE: To describe the clinical features of uveitis in children treated at two ophthalmologic centers in Bogotá, Colombia, in a 13 year-period. METHODS: Retrospective observational clinical record review of pediatric children with diagnosis of uveitis. RESULTS: In total, 310 children were evaluated, 51.9% were female, mean age of 10.1 years. Posterior uveitis was the most common location (58.7%), of insidious onset (87.4%) and chronic course (78.1%). The most common etiology was infection (58.4%) caused by toxoplasmosis (76.8%). There was a statistically significant difference in visual acuity between anterior (20/68) and intermediate uveitis (20/70), compared with posterior uveitis (20/434) (p<0.05). CONCLUSIONS: This is the first study to report the clinical features of pediatric uveitis in Colombia, where infectious etiologies are the leading cause. It will improve awareness and knowledge of pediatric uveitis in developing countries, and contribute to the development of public health policies of pediatric visual health. Received 12 September 2015; revised 23 February 2016; accepted 25 February 2016; published online 18 May 2016.

Tubulointerstitial Nephritis and Uveitis Syndrome: Case Report and Review of the Literature.

PURPOSE: To review the literature on tubulointerstitial nephritis and uveitis (TINU) syndrome, and to report a case of a patient with relapsing polychondritis (RP) and TINU syndrome. METHOD: TINU syndrome is a rare oculorenal inflammatory disorder. It is more common in young women with autoimmune conditions, infections, systemic disease, and previous use of medications. We report the case of a 62-year-old woman with relapsing polychondritis and a 2-year history of acute, recurrent, asymmetric, bilateral, anterior, non-granulomatous uveitis accompanied by tubulointerstitial nephritis. RESULTS: The patient was diagnosed with TINU syndrome associated with relapsing polychondritis. No cases of this association have been reported in the literature. The clinical features of TINU syndrome are discussed based on the published works. CONCLUSIONS: TINU is an uncommon syndrome; only about 200 cases have been reported in the literature related to infections, systemic disease, and previous use of medications such as antibiotics and non-steroidal anti-inflammatory drugs (NSAIDs). We found that it can be associated with relapsing polychondritis; therefore, it is important to investigate symptoms of this disease since TINU syndrome can co-exist with it.

Treatment of vascular activity secondary to atypical choroidal nevus using intravitreal bevacizumab.

OBJECTIVE: To report the optical coherence tomography (OCT) findings of 27 eyes treated with intravitreal bevacizumab for intraretinal and subretinal vascular activity associated with atypical choroidal nevi. METHODS: This was an Internal Review Board-approved retrospective review of 27 eyes of 27 patients with choroidal nevus treated for secondary vascular activity with intravitreal injections of bevacizumab, performed by a single surgeon (TGM) at the Bascom Palmer Eye Institute. All patients were rigorously evaluated before the procedure and followed thereafter with ophthalmic examinations, refractive analysis, fundus photos, optical coherence tomography (OCT), and ocular echography. Patient demographics, tumor characteristics, dates of bevacizumab injections, and spectral-domain (SD)-OCT findings at each injection were recorded. Macular edema was graded as per SD-OCT findings for the initial and final visit. RESULTS: The mean age was 66.6 years (range, 40-86 years), with ten males and 17 females. Mean, median, and range baseline best corrected visual acuity (BCVA) were 20/53, 20/40, and 20/20-4/200, respectively. After a mean follow up of 29 months, the final BCVA mean, median, and range were 20/50, 20/40, and 20/20-20/400, respectively. The final BCVA ranged from 20/20 to 20/25 in nine eyes, while only six eyes had an initial BCVA within the same range. All patients demonstrated OCT findings of vascular activity suggestive of choroidal neovascularization (CNV). Initial SD-OCT findings included intraretinal cysts in eleven eyes, intraretinal fluid in six eyes, subretinal fluid in 14 eyes, pigment epithelial detachment in six eyes, epiretinal membrane in five eyes, and subretinal neovascularization in 14 eyes. On fundus photos, four eyes presented retinal hemorrhage. A mean of eight (range of 1-31) intravitreal bevacizumab (1.25 mg/0.05 cc) injections were given in all cases. A total of 37% (10/27) of eyes had complete or partial regression of vascular activity. The mean initial OCT classification for macular edema was 3 and a mean grade of 3 was maintained at the final follow-up OCT. All 27 choroidal nevi remained stable, and there were no adverse effects from the bevacizumab injections. CONCLUSION: To our knowledge, this is the largest published case series of eyes treated with intravitreal bevacizumab for vascular activity associated with choroidal nevus. Intravitreal bevacizumab seems to be effective in the treatment of CNV secondary to choroidal nevus, and OCT can be a useful tool in the follow up of these patients, to assess the regression of CNV and to monitor macular edema.

Microincisional vitrectomy for retinal detachment in I-125 brachytherapy-treated patients with posterior uveal malignant melanoma.

PURPOSE: To analyze functional and anatomical outcomes following 23/25(+) gauge microincisional pars plana vitrectomy surgery (MIVS) in patients with radiation-related retinal detachment after successful 125-iodine (I-125) brachytherapy treatment for malignant uveal melanoma. PATIENTS AND METHODS: Retrospective case series of 102 consecutive eyes of 102 patients with history of uveal melanoma treated with I-125 brachytherapy that underwent MIVS at the Bascom Palmer Eye Institute. All cases were evaluated for surgical complications and local tumor control. Extended follow-up included Snellen's best-corrected visual acuity, intraocular pressure evaluation, quantitative echography, indirect ophthalmoscopy, and fundus imaging with optical coherence tomography/wide-field photography. RESULTS: All patients had radiation-related complications, including retinal detachment (102 eyes), vasculopathy (91 eyes), optic neuropathy (32 eyes), and/or vitreous hemorrhage (8 eyes). Sixty-seven patients had vitreoretinal traction. Average follow-up after MIVS was 19.5 months, and from plaque removal was 57.7 months. Interval from plaque to MIVS was 38.1 months. Initial visual acuity was 20/258, which improved to 20/101 at 1 month, 20/110 at 3 months, 20/116 at 6 months, and 20/113 at 12 months (P < 0.05). No eyes required enucleation. Melanoma-related mortality was 0.9% (1/102). There was no intra- or extraocular tumor dissemination, and no tumor recurrence. CONCLUSION: MIVS was effective in improving visual function and anatomy in patients with radiation-related retinal detachment. Tumors decreased in size and there was no evidence of recurrence or tumor dissemination. This combined procedure addresses the modifiable causes of visual loss in patients with previously treated malignant uveal melanoma and has the potential to enhance their visual function.

Intravitreal bevacizumab combined with plaque brachytherapy reduces melanoma tumor volume and enhances resolution of exudative detachment.

BACKGROUND: The purpose of this study was to evaluate intravitreal bevacizumab as an adjuvant treatment to plaque brachytherapy in the treatment of choroidal melanoma. METHODS: This was a retrospective, consecutive study of 124 patients treated from 2007 to 2009 for choroidal melanoma with plaque brachytherapy. Patients were treated with I-125 plaque brachytherapy with 2 mm margins and 85 Gy to the tumor apex. Consecutive patients were injected intravitreally with 2.5 mg/0.1 mL bevacizumab at a site away from the primary tumor and immediately following plaque removal. Choroidal melanomas were observed using indirect ophthalmoscopy, wide-angle photography, and ultrasound. The main outcome measures were tumor volume, resolution of exudative retinal detachment, and visual acuity. RESULTS: One hundred and twenty-four patients met our inclusion criteria and were included in the analysis. The mean patient age was 65.7 years, and the mean apical tumor height was 4.0 ± 2.7 mm and basal diameter was 12.7 ± 3.0 mm. Mean follow-up was 24 months. Prior to treatment, 100% of tumors had exudative retinal detachment, and pretreatment visual acuity was 20/55 (median 20/40). Tumor control was 100%, metastasis was 0% at last follow-up, and 89.8% had complete resolution of exudative retinal detachment, with a mean time to resolution of 3.36 months. At one month, 43% had complete resolution of exudative retinal detachment, which increased to 73% at 4 months. Visual acuity was 20/62 (median 20/40) at 4 months, with stabilization to 20/57 (median 20/40) at 8 months, 20/56 (median 20/30) at 12 months, and 20/68 (median 20/50) at 24 months. Tumor volume following combined therapy was shown to be reduced by 22.2% at 3 months, 28.9% at 6 months, 39.3% at 12 months, and 52.2% at 24 months (all P < 0.001). All patients tolerated the procedure well without systemic side effects. CONCLUSION: Intravitreal bevacizumab may be used as an adjuvant agent following plaque brachytherapy. Treated choroidal melanomas show reduction in tumor volume as well as resolution of exudative retinal detachments.

Combined bevacizumab and triamcinolone acetonide injections for macular edema in a patient with astrocytic hamartomas and tuberous sclerosis.

Retinal astrocytic hamartoma or retinal astrocytoma is the best-known ocular manifestation of tuberous sclerosis complex, a neurocutaneous syndrome characterized by the development of multiple disseminated hamartomas. It can have several clinical presentations, ranging from unilateral transparent, noncalcified lesions to bilateral multinodular, mulberry-like calcified tumors. Symptoms appear if the tumor involves the macula and can cause visual loss on the basis of progressive retinal degeneration. The authors report a case of a patient with tuberous sclerosis, bilateral astrocytic hamartomas, and macular edema with intraretinal hemorrhage in the left eye that has responded well to treatment with bevacizumab and intravitreal triamcinolone acetonide.