Pediatric oral fluorescein angiography: a retrospective review from a single institution.

PURPOSE: To describe our experience with locally developed evidence-based guidelines for oral fluorescein angiography (FA) for retinal imaging in children. METHODS: The medical records of consecutive pediatric patients (≤18 years of age) at University of Pittsburgh Medical Center Children's Hospital Eye Center who underwent oral FA between November 1, 2018, and April 1, 2022, were reviewed retrospectively. Adherence to or deviation from the guidelines was noted at the time of testing. RESULTS: A total of 55 patients aged 3-18 with 79 examinations were included. No patient was excluded from the retrospective case review because of lack of recorded data. The main indications for oral FA included uveitis, retinal vasculopathy, disk pathology, and retinal lesions. Three children had transient side effects, and 1 had delayed urticaria 4 hours after examination. No child had anaphylaxis. One patient had suboptimal imaging due to nonadherence to the guidelines-recommended fasting protocol. All other examinations (78/79), where guidelines were followed, provided images adequate for clinical decision making. CONCLUSIONS: Based on our experience, we recommend that oral FA be considered, especially in children where intravenous access is less well tolerated while awake. Informed consent that includes the possibility of delayed side effects is advisable.

Comparison of retropupillary fixated iris claw lens versus sclera fixated lens for correction of pediatric aphakia secondary to ectopia lentis.

AIM: To evaluate the postoperative visual acuity and complications in eyes with ectopia lentis in children who underwent lens removal and then implantation of retropupillary fixated iris claw lens versus scleral fixated intraocular lens (SFIOL) implantation. MATERIALS AND METHODS: A retrospective analysis of pediatric cases who presented with lens subluxation secondary to ectopia lentis and who underwent lens extraction (57 eyes of 38 patients) with either retropupillary iris fixated intraocular lens (IOL) implantation (Group A - 36 eyes of 20 patients) and SFIOL implantation (Group B - 21 eyes of 18 patients) was done over a period of 5 years from March 2010 to February 2015. The main outcome measures were preoperative and postoperative best-corrected visual acuity (BCVA) and secondary postoperative complications. RESULTS: The study patients were divided into two groups: Group A patients were implanted with retropupillary iris claw lens whereas Group B patients were implanted with SFIOL. The mean age of presentation was 12 years, the mean follow-up period was of 24 months (range 14-36 months), and the median follow-up period was 26 months in both the groups. An improvement in the mean BCVA (LogMAR) was seen in both the groups. In Group A, the mean BCVA improved from 1.5 ± 0.2 preoperatively to 0.3 ± 0.2 postoperatively, whereas in Group B, the mean BCVA improved from 1.5 ± 0.3 preoperatively to 0.3 ± 0.2 postoperatively (P < 0.001). None of the eyes in either of the groups had any serious complications such as glaucoma, uveitis, cystoid macular edema, or endophthalmitis. CONCLUSION: Retropupillary iris fixation and scleral fixation of IOL are both safe and viable options for the correction of ectopia lentis in pediatric age group.

An interplay of microglia and matrix metalloproteinase MMP9 under hypoxic stress regulates the opticin expression in retina.

Inflammation plays a key role in the pathogenesis of retinal vascular diseases. We have shown earlier an increase in the activity of matrix metalloproteinases in the vitreous and tears of preterm born babies with retinopathy of prematurity (ROP) compared to those with no-ROP leading to a shift in the balance of angiogenic (vascular endothelial growth factor [VEGF], matrix metalloproteinase [MMPs], complement component [C3]) and anti-angiogenic (opticin, thrombospondin) in ROP eyes. We now confirmed that tear MMP levels in premature infants perfectly correlates with disease severity. Next, we demonstrated that a reduced opticin levels in ROP vitreous are regulated by MMPs secreted by activated microglia. Upon exposing the human microglia cell line (CHME3) to hypoxia, an increased expression of inflammatory proteins (MMP9, VEGF) was noticed while opticin reduced significantly (p = 0.005). Further, the reduced opticin's expression by microglial cells under hypoxia could be rescued by inhibiting the MMP activity using doxycycline and EDTA. The inhibition of MMP activity altered the expression of other key signaling molecules under hypoxia. Our study clearly explains that increased activity of MMPs under hypoxia regulates the expression of opticin as seen in the vitreous humor of ROP and could serve as a potential target for ROP management.

Comparison of Pediatric Cataract Surgical Techniques Between Pediatric Ophthalmology Consultants and Fellows in Training: A Video-Based Analysis.

PURPOSE: To compare intraoperative technique and quality of surgical steps in pediatric cataract surgery between pediatric ophthalmology consultants and fellows in training. METHODS: In a video-based analysis by two fellowship-trained pediatric ophthalmologists, 42 surgical videos of pediatric ophthalmology consultants and 34 videos of fellows in training were graded based on the International Council of Ophthalmology's Ophthalmology Surgical Competency Assessment Rubrics (ICO-OSCAR). Six steps in surgery were analyzed: wound construction, anterior continuous curvilinear capsulorrhexis, irrigation and aspiration, intraocular lens implantation, primary posterior capsulorrhexis with anterior vitrectomy, and wound suturing. Cohen's Kappa was used to rate inter-observer agreement. RESULTS: Cohen's Kappa scores ranged from 0.6 to 0.8. The median scores for surgical steps for both analyzed groups were similar. The mean duration of surgery was shorter for consultants (24 minutes, range: 10 to 45 minutes) than for fellows (40 minutes, range: 15 to 70 minutes). The median age of patients operated on by consultants was younger (24 months, range: 2 to 180 months) than fellows (58 months, range: 10 to 150 months). CONCLUSIONS: The quality of the surgical steps performed by pediatric ophthalmology consultants and fellows in training was similar. [J Pediatr Ophthalmol Strabismus. 2019;56(2):83-87.].

Optical coherence tomography angiography in acute unilateral nonarteritic anterior ischemic optic neuropathy: A comparison with the fellow eye and with eyes with papilledema.

Purpose: The purpose of this study is to detect the optic nerve head (ONH) and peripapillary perfusion in eyes with acute nonarteritic anterior ischemic optic neuropathy (NAION) compared to the fellow normal eyes using optical coherence tomography angiography (OCTA) and to compare with nonischemic disc edema (papilledema). Methods: Retrospective analysis of patients with unilateral NAION who underwent OCTA was performed. All patients underwent comprehensive ocular examination including visual field testing. ONH was imaged using 6 mm × 6 mm scan by Topcon DRI Triton® OCT system. Vascularity loss was analyzed using ImageJ software in diseased eyes in comparison to normal fellow eyes and eyes with papilledema. Results: Twenty-one patients (15 males, 6 females) with unilateral NAION and 9 patients (18 eyes) with papilledema were included in the study. In eyes with NAION, two distinct patterns of loss of vasculature were noted - (a) diffuse loss of microvasculature cuff and vascular network around the optic disc in all the patients (100%) and (b) additional area of sectoral loss of vasculature extending from the disc in 12 of the 21 eyes (57.14%). All 18 eyes with papilledema showed loss of the microvasculature cuff; however, none showed the focal pattern of vascular defect. The mean area of the peripapillary vascular zone in eyes with NAION was significantly lesser than that in normals. Of the 12 eyes with NAION with focal loss of vasculature, 11 correlated with visual field defects (91.6%). Conclusion: Deficient peripapillary choroidal vasculature is present in NAION and has a different pattern than in nonischemic disc edema and can cause corresponding visual field deficits.

Retinal Findings on OCT in Systemic Conditions.

PURPOSE: Imaging technology has advanced by leaps and bounds in the recent past and has resulted in a much greater understanding of ocular diseases. The aim of this review article is to summarize optical coherence tomography (OCT) findings of various systemic conditions. METHOD: A systematic literature search of the Medline/PubMed database was performed. English articles up to April 2015 were included. Terms used for search included: Alzheimer's Disease; Multiple Sclerosis; Parkinson's Disease; Behçet's Disease; Schizophrenia; Migraine; Obstructive Sleep Apnea Syndrome; Neurofibromatosis; Sickle Cell Disease; Renal diseases; Lupus Retinopathy; Valsalva Retinopathy; Whiplash Retinopathy; Shaken-Baby Syndrome; Choroidal metastases; Intracranial Hypertension; Drug toxicity; Deferoxamine; Sildenafil; Tamoxifen; Hydroxychloroquine; Chloroquine; Ethambutol; Lead; Sickle Cell Disease; and Thalassemia along with OCT. RESULTS: Studies have shown that inner retinal thinning could be the earliest sign of neurological diseases and may help to differentiate individuals with abnormalities. Outer retinal damage was noted in cancer-related retinopathy and secondary to drug toxicity as a diagnostic sign. This review article summarizes the OCT findings and their importance in early diagnosis, treatment, and follow-up in a varying spectrum of systemic diseases including neurological diseases, hematological diseases, cancer-related retinopathies, and systemic drug toxicity. CONCLUSION: OCT findings are useful to predict the probability of a disease, to diagnose it early, to differentiate between healthy and unhealthy tissue, and to assess the effect of therapeutic interventions in many systemic diseases.

Abnormal Complement Activation and Inflammation in the Pathogenesis of Retinopathy of Prematurity.

Retinopathy of prematurity (ROP) is a neurovascular complication in preterm babies, leading to severe visual impairment, but the underlying mechanisms are yet unclear. The present study aimed at unraveling the molecular mechanisms underlying the pathogenesis of ROP. A comprehensive screening of candidate genes in preterms with ROP (n = 189) and no-ROP (n = 167) was undertaken to identify variants conferring disease susceptibility. Allele and genotype frequencies, linkage disequilibrium and haplotypes were analyzed to identify the ROP-associated variants. Variants in CFH (p = 2.94 × 10-7), CFB (p = 1.71 × 10-5), FBLN5 (p = 9.2 × 10-4), CETP (p = 2.99 × 10-5), and CXCR4 (p = 1.32 × 10-8) genes exhibited significant associations with ROP. Further, a quantitative assessment of 27 candidate proteins and cytokines in the vitreous and tear samples of babies with severe ROP (n = 30) and congenital cataract (n = 30) was undertaken by multiplex bead arrays and further validated by western blotting and zymography. Significant elevation and activation of MMP9 (p = 0.038), CFH (p = 2.24 × 10-5), C3 (p = 0.05), C4 (p = 0.001), IL-1ra (p = 0.0019), vascular endothelial growth factor (VEGF) (p = 0.0027), and G-CSF (p = 0.0099) proteins were observed in the vitreous of ROP babies suggesting an increased inflammation under hypoxic condition. Along with inflammatory markers, activated macrophage/microglia were also detected in the vitreous of ROP babies that secreted complement component C3, VEGF, IL-1ra, and MMP-9 under hypoxic stress in a cell culture model. Increased expression of the inflammatory markers like the IL-1ra (p = 0.014), MMP2 (p = 0.0085), and MMP-9 (p = 0.03) in the tears of babies at different stages of ROP further demonstrated their potential role in disease progression. Based on these findings, we conclude that increased complement activation in the retina/vitreous in turn activated microglia leading to increased inflammation. A quantitative assessment of inflammatory markers in tears could help in early prediction of ROP progression and facilitate effective management of the disease, thereby preventing visual impairment.

A simple and novel grading method for retraction and overshoot in Duane retraction syndrome.

Strabismus in Duane retraction syndrome is frequently associated with significant globe retraction and overshoots. However, there is no method to objectively grade retraction and overshoot. Our purpose is to describe a novel objective grading method. This novel and simple grading method has excellent agreement. It will help standardise measurements and guide the clinician in taking the decision for surgery and predicting its outcome.

Neuro-ophthalmic manifestations of prematurity.

Increasing rates of preterm births coupled with better survival of these infants have resulted in higher prevalence of systemic and ocular complications associated with prematurity. In addition to retinopathy of prematurity, infants who are born preterm may suffer from severe visual impairment as a result of hypoxic ischemic encephalopathy, hypoglycemia, and other metabolic imbalances. The effect of these processes on the anterior visual pathway may result in optic atrophy, optic nerve hypoplasia or optic disc cupping and affection of the posterior visual pathway leads to cortical visual impairment (CVI). Other ocular associations include strabismus, nystagmus, and ocular motor abnormalities such as tonic down gaze and defective saccades and pursuits. Cortical and subcortical involvement also manifests as defects in functional vision and these have not yet been completely understood. Children with CVI may have visual field defects, photophobia, defective visual processing, and deficient color vision. Since most of these children also suffer from additional systemic disabilities, evaluation, and management remains a challenge. However, early diagnosis and initiation of rehabilitation therapy can prove to be of significant benefit in these children.