Klebsiella pneumoniae Endophthalmitis with Subretinal Abscess: A Case Series and Review of the Literature.

This study assessed prognostic factors and the role of vitrectomy in patients with subretinal abscesses secondary to K. pneumoniae endophthalmitis. We reviewed published studies, including three cases from our cohort. Among 50 eyes, 26 had poor visual outcomes (final visual acuity <20/800, eyeball removal, or phthisis bulbi). Poor outcomes correlated with delayed ocular symptom-to-diagnosis time, initial visual acuity <20/800, severe vitritis, and macular involvement of abscesses (p < 0.001, p = 0.008, p < 0.001, and p = 0.033, respectively). Vitrectomy had a trend towards reducing eyeball removal and phthisis bulbi rates compared with non-vitrectomy (10.8% vs 30.8%, p = 0.181). However, the final visual acuity was not different and the rate of retinal detachment tended to be higher in vitrectomized eyes (45.9% vs 15.4%, p = 0.095). The study suggested that vitrectomy and drainage of K. pneumoniae subretinal abscesses could be avoided in patients with a mild degree of vitritis.

Effect of fenestrated sterile drape and face mask on bacterial dispersion toward the periocular area during intravitreal injection.

This experimental crossover study was performed to investigate whether fenestrated surgical drapes (covering the nose and mouth but with an opening over the periorbital area) with or without patients' surgical face masks increase periorbital bacterial dispersion during simulated intravitreal injection conditions. Each of the 16 healthy volunteers performed 14 scenarios involving different mask and drape conditions in both silent and speaking situations. In each scenario, the subject lay down flat on the back with a blood agar plate being held at the inferior orbital rim perpendicular to the face to capture airflow from breathing/speaking. Another blood agar plate placed 50 cm away from the subject served as an experimental control. A total of 224 experiments were performed. Speaking situations significantly showed more colony forming units (CFUs) compared with their controls (P = 0.014). There were no significant differences in CFUs between wearing vs not wearing the masks (P = 0.887 for speaking and P = 0.219 for silent) and using vs not using the drapes (P = 0.941 for speaking and P = 0.687 for silent). Reusable and disposable drapes were also not significantly different (P = 1.00 for speaking and P = 0.625 for silent). Streptococcus spp., the oropharyngeal microbiota, were only cultivated from speaking scenarios. While refraining from speaking (for both practitioners and patients) is the mainstay of reducing bacterial dispersion and risks of post-injection endophthalmitis, the use of fenestrated surgical drapes or patients' face masks did not significantly affect the amount of bacterial dispersion toward the periorbital area.

CRVO associated with COVID-19 and MTHFR mutation in a 15-year-old male.

Purpose: To report a case of a central retinal vein occlusion (CRVO) associated with COVID-19 in a patient predisposed to clotting due to a genetic mutation in methylenetetrahydrofolate reductase (MTHFR). Observations: A 15-year-old male presented with 1 day of painless blurry vision in the left eye. Exam disclosed trace anterior chamber cell, anterior vitreous cell, optic nerve head edema, temporally displaced macular star, dilated tortuous veins, and diffuse intraretinal hemorrhages. Exam and FA was consistent with CRVO, however the macular star and OCT were suggestive of a neuroretinitis.The patient then presented to a children's hospital for further evaluation. A routine screen for COVID-19 via nasopharyngeal swab was positive with a high viral load. He also had a known history of an MTHFR mutation. Extensive laboratory and neuroradiologic evaluation excluded other infectious, inflammatory, and coagulopathic etiologies. Conclusions and Importance: This is a case of CRVO associated with COVID-19 infection and an underlying systemic hypercoagulable mutation, with an initial presentation that mimicked neuroretinitis. This case provides valuable diagnostic learning points and expands our knowledge of possible ocular complications of COVID-19.

Subretinal gnathostomiasis: A successful focal laser photocoagulation for a living parasite.

PURPOSE: To report a case of subretinal gnathostomiasis presenting with progressive subretinal tracts of a living parasite and successfully treated with focal laser photocoagulation. METHOD: Observational case report. PATIENT: A 29-year-old Thai male complained of blurred vision and floaters in his left eye for two weeks. An ocular examination showed multiple, whitish, subretinal tracks at the superotemporal retina. After 5 days of oral albendazole, a moving parasite was confirmed by multimodal retinal imaging. An immunoblotting analysis was positive for Gnathostoma species. RESULT: The patient was treated by laser photocoagulation with frequency-doubled Nd:YAG laser around and over the parasite. Oral albendozole was continued and naproxen was prescribed for four weeks. His vision improved to 20/20 and the inflammation subsided completely within three months. The patient has been followed for five years without local and systemic complications. CONCLUSIONS: Focal laser photocoagulation without systemic steroids could be a successful treatment for active subretinal gnathostomiasis with a satisfactory safety profile in a long-term follow-up.

The Prevalence of Retinal Disease and Associated CNS Disease in Young Patients with Incontinentia Pigmenti.

PURPOSE: To evaluate the prevalence of retinal disease on fluorescein angiography (FA) in patients with incontinentia pigmenti (IP) and to compare the severity of retinal disease in those with and without known central nervous system (CNS) disease. DESIGN: Multi-institutional consecutive retrospective case series. SUBJECTS: New patients with a diagnosis of IP were seen at the Casey Eye Institute at the Oregon Health and Science University (OHSU), Moran Eye Center, University of Utah, or Bascom Palmer Eye Institute, University of Miami from December 2011 to September 2018. METHODS: Detailed ophthalmoscopic examination and FA were recommended for all new patients and performed on every patient who had parental consent. Ophthalmoscopic findings and FA images were graded for severity by 2 masked graders on a 3-point scale: 0 = no disease, 1 = vascular abnormalities without leakage, 2 = leakage or neovascularization, and 3 = retinal detachment. The presence of known CNS disease was documented. Additional cases were obtained from a pediatric retina listserv for examples of phenotypic variation. MAIN OUTCOME MEASURES: The proportion of eyes noted to have disease on ophthalmoscopy compared with FA and the severity of retinal disease in those with and without known CNS disease. RESULTS: Retinal pathology was detected in 18 of 35 patients (51%) by indirect ophthalmoscopy and 26 of 35 patients (74%) by FA (P = 0.048) in a predominantly pediatric population (median age, 9 months). Ten patients (29%) had known CNS disease at the time of the eye examination. A Wilcoxon rank-sum test indicated that the retinal severity scores for patients with CNS disease (median, 2) were significantly higher than the retinal severity scores for patients without CNS disease (median, 1), z = -2.12, P = 0.034. CONCLUSIONS: Retinal disease is present in the majority of patients with IP, and ophthalmoscopic examination is less sensitive than FA for detection of disease. There may be a correlation between the severity of retinal and CNS disease.

In-Person Verification of Deep Learning Algorithm for Diabetic Retinopathy Screening Using Different Techniques Across Fundus Image Devices.

Purpose: To evaluate the clinical performance of an automated diabetic retinopathy (DR) screening model to detect referable cases at Siriraj Hospital, Bangkok, Thailand. Methods: A retrospective review of two sets of fundus photographs (Eidon and Nidek) was undertaken. The images were classified by DR staging prior to the development of a DR screening model. In a prospective cross-sectional enrollment of patients with diabetes, automated detection of referable DR was compared with the results of the gold standard, a dilated fundus examination. Results: The study analyzed 2533 Nidek fundus images and 1989 Eidon images. The sensitivities calculated for the Nidek and Eidon images were 0.93 and 0.88 and the specificities were 0.91 and 0.85, respectively. In a clinical verification phase using 982 Nidek and 674 Eidon photographs, the calculated sensitivities and specificities were 0.86 and 0.92 for Nidek along with 0.92 and 0.84 for Eidon, respectively. The 60°-field images from the Eidon yielded a more desirable performance in differentiating referable DR than did the corresponding images from the Nidek. Conclusions: A conventional fundus examination requires intense healthcare resources. It is time consuming and possibly leads to unavoidable human errors. The deep learning algorithm for the detection of referable DR exhibited a favorable performance and is a promising alternative for DR screening. However, variations in the color and pixels of photographs can cause differences in sensitivity and specificity. The image angle and poor quality of fundus photographs were the main limitations of the automated method. Translational Relevance: The deep learning algorithm, developed from basic research of image processing, was applied to detect referable DR in a real-word clinical care setting.

Traumatic Retinal Detachment in Patients with Self-Injurious Behavior: An International Multicenter Study.

PURPOSE: To describe the clinical characteristics, surgical outcomes, and management recommendations in patients with traumatic rhegmatogenous retinal detachment (RRD) resulting from self-injurious behavior (SIB). DESIGN: International, multicenter, retrospective, interventional case series. PARTICIPANTS: Patients with SIB from 23 centers with RRD in at least 1 eye. METHODS: Clinical histories, preoperative assessment, surgical details, postoperative management, behavioral intervention, and follow-up examination findings were reviewed. MAIN OUTCOME MEASURES: The rate of single-surgery anatomic success (SSAS) was the primary outcome. Other outcomes included new RRD in formerly attached eyes, final retinal reattachment, and final visual acuity. RESULTS: One hundred seven eyes with RRDs were included from 78 patients. Fifty-four percent of patients had bilateral RRD or phthisis bulbi in the fellow eye at final follow-up. The most common systemic diagnoses were autism spectrum disorder (35.9%) and trisomy 21 (21.8%) and the most common behavior was face hitting (74.4%). The average follow-up time was 3.3 ± 2.8 years, and surgical outcomes for operable eyes were restricted to patients with at least 3 months of follow-up (81 eyes). Primary initial surgeries were vitrectomy alone (33.3%), primary scleral buckle (SB; 26.9%), and vitrectomy with SB (39.7%), and 5 prophylactic SBs were placed. Twenty-three eyes (21.5%) with RRDs were inoperable. The SSAS was 23.1% without tamponade (37.2% if including silicone oil), and final reattachment was attained in 80% (36.3% without silicone oil tamponade). Funnel-configured RRD (P = 0.006) and the presence of grade C proliferative vitreoretinopathy (P = 0.002) correlated with re-detachment. The use of an SB predicted the final attachment rate during the initial surgery (P = 0.005) or at any surgery (P = 0.008. These associations held if restricting to 64 patients with ≥12 months followup. Anatomic reattachment correlated with better visual acuity (P < 0.001). CONCLUSIONS: RRD resulting from SIB poses therapeutic challenges because of limited patient cooperation, bilateral involvement, chronicity, and ongoing trauma in vulnerable and neglected patients. The surgical success rates were some of the lowest in the modern retinal detachment literature. The use of an SB may result in better outcomes, and visual function can be restored in some patients.

Diagnostic and Management Strategies in Patients with Persistent Fetal Vasculature: Current Insights.

Persistent fetal vasculature (PFV), previously known as persistent hyperplastic primary vitreous, is a developmental malformation of the eyes that is caused by a failure of the hyaloid vasculature to regress in utero. PFV has been reported for decades; however, our understanding of the pathophysiology/pathogenesis of PFV, and the diagnostic and treatment modalities for PFV have evolved over time, and these advancements have improved diagnosis, treatment, and outcomes. However and in spite of these advancements, the heterogeneity of this disease continues to make PFV a diagnostic challenge. Here, we review what is currently known about various important aspects of PFV to update and enhance the knowledge of ophthalmologists who encounter and manage PFV in clinical practice.

Vitrectomy using the Eckardt temporary keratoprosthesis.

PURPOSE: to present the now vitrectomy technique through a limbus via preexisting holes in the Eckardt TKP. METHODS: the surgical technique performed in a case of four-year-old girl presented with a complete hyphema and vitreous hemorrhage in the left eye. RESULTS: the vitrectomy was performed vial a limbal approach from the TKP without making additional sclerotomies. The vitreous hemorrhage was evacuated, the hyaloid elevated, the periphery examined with an excellent widefield view without scleral depression, an air-fluid exchange performed, and the air exchanged for silicone oil. Finally, the TKP was replaced with a permanent graft. The surgical video was presented additionally. CONCLUSION AND IMPORTANCE: Eckardt TKP allows for a limbal approach in aphakic or non-lens sparing vitrectomy surgery while maintaining a stable intraocular pressure, excellent peripheral and posterior access and while avoiding the need for pars plana sclerotomy placement which can be challenging in complex cases.

Select pediatric vitreoretinal disease in the setting of Turner's syndrome.

PURPOSE: To report 2 cases of pediatric vitreoretinal disease in the setting of Turner's syndrome. OBSERVATIONS: A 4-year-old girl with Turner's syndrome was referred for evaluation of a tractional retinal detachment in the right eye. Fundoscopic examination disclosed temporal dragging of the macula in the right eye, and vascular nonperfusion in the right and left eyes. Genetic testing revealed a novel frameshift mutation in the LRP5 gene consistent with familial exudative vitreoretinopathy (FEVR). The patient was treated with laser. A 14-year-old girl with Turner's syndrome presented with nyctalopia. Dilated fundus exam disclosed peri-foveal pigmentary changes and peripheral bone spicules. Full-field electroretinography demonstrated decreased rod and cone responses, consistent with retinitis pigmentosa (RP). CONCLUSIONS AND IMPORTANCE: Vitreoretinal disease, including RP and FEVR, is rarely observed in patients with Turner's syndrome.

Spectrum of peripheral retinal ischemia in Wyburn-Mason syndrome.

PURPOSE: We report two cases of Wyburn-Mason syndrome that illustrate the spectrum of peripheral retinal ischemia seen in this condition. OBSERVATIONS: A 12-year-old female presented with a retinal arteriovenous malformation and sclerotic vessels associated with retinal ischemia on fluorescein angiography, as well as an ipsilateral ophthalmic arteriovenous malformation on magnetic resonance imaging. An 11-year-old male presented with retinal vascular engorgement and tortuosity along with a central retinal vein occlusion and secondary neovascularization. CONCLUSIONS AND IMPORTANCE: Retinal ischemia in Wyburn-Mason syndrome is heterogeneous and may be progressive, with secondary complications that result in neovascularization. Furthermore, it is necessary to recognize that this is a systemic condition that requires neurological evaluation.

Extralesional microvascular and structural macular abnormalities in cytomegalovirus retinitis.

To evaluate extralesional microvascular and structural changes of the macula using optical coherence tomography angiography (OCTA) and structural OCT in cytomegalovirus retinitis (CMVR). An observational study of CMVR patients were performed. Complete ophthalmic examination, serial color fundus photography, structural OCT and OCTA were performed at baseline and follow-up visits for up to 12 months. The structural OCT was analyzed to evaluate macular areas within, bordering and beyond the CMVR lesions. Extralesional retinal capillary plexus of the macula were evaluated by OCT angiography and compared with the unaffected fellow eyes. Thirteen eyes from 13 patients were enrolled. At baseline, macular areas without CMVR lesions showed decreased vessel density (VD) of both the superficial (P = 0.0002) and deep (P < 0.0001) retinal capillary plexus in eyes with CMVR as compared with the corresponding macular areas of the unaffected fellow eyes. The decrease of VD persisted through the follow-up period for up to 12 months after adjusting for degree of vitreous haze. Structural macular OCT characteristics at the borders and beyond the lesions included intraretinal hyperreflective dots, cystoid macular edema, subretinal fluid and selective ellipsoid zone (EZ) loss. The selective EZ loss found in 6 of 12 eyes showed recovery in 4 eyes after receiving anti-viral treatment. In CMVR eyes, there were microvascular and microstructural abnormalities in the macular area without clinically visible CMVR lesions. Our results provided interesting insights into CMV infection of the retina.

Bilateral, Solitary, Extramacular Vitelliform Retinal Lesions in a Patient With Best Disease.

Purpose: This work describes the first published case of Best vitelliform macular dystrophy (BVMD) with bilateral, solitary, extramacular retinal lesions. Methods: A case report is presented. Results: An 8-year-old girl with a family history of BVMD was referred for suspicious peripheral retinal lesions. Multimodal imaging disclosed bilateral, solitary, extramacular lesions consistent with the vitelliform lesions found in BVMD. There were no abnormalities in the macula. Conclusions: This is the first documented case of solitary, bilateral, extramacular vitelliform lesions in BVMD.

Novel Frizzled-4 Mutation Is Associated With Familial Exudative Vitreoretinopathy Mimicking Persistent Fetal Vasculature.

This is a report of a 13-month-old boy who presented with a large unilateral fibrovascular stalk and bilateral peripheral retinal avascularity. Although consistent with both persistent fetal vasculature and familial exudative vitreoretinopathy, genetic testing disclosed a novel pathogenic mutation in the frizzled class receptor 4 gene (FZD4, c.427_428delCT). [J Pediatr Ophthalmol Strabismus. 2020;57:e4-e7.].

X-Linked Retinoschisis and a Coats-Like Response in the Setting of Retinopathy of Prematurity.

PURPOSE: This case report describes a unique case of a young patient with retinopathy of prematurity (ROP), a unilateral Coats-like response, and X-linked retinoschisis (XLRS). METHODS: A 9-year-old boy with a history of regressed ROP presented with a unilateral Coats-like response, subretinal exudation, and XLRS. Examination and imaging findings demonstrated a highly unique combination of bilateral retinoschisis and a dramatic unilateral Coats-like response with a large schisis cavity. RESULTS: Treatment with laser photocoagulation and anti-VEGF therapy led to resolution of the subretinal exudative changes. CONCLUSIONS: This is the first published description to our knowledge of a patient with a Coats-like response, XLRS, and a history of regressed ROP with resolution after treatment.

A unique case of coats plus syndrome and dyskeratosis congenita in a patient with CTC1 mutations.

Coats plus syndrome (CP) is a rare condition characterized by bilateral exudative retinal telangiectasias with associated systemic disorders primarily affecting the brain, bone and gastrointestinal tract due to a mutation in the CTC1 gene. CTC1 mutations are also known to cause dyskeratosis congenita (DC), which is an inherited bone marrow failure syndrome characterized by skin pigmentation abnormalities, nail dystrophy, and oral leukoplakia. This is the first reported case of a patient diagnosed with both CP and DC caused by compound heterozygous CTC1 gene mutations. Moreover, one of the variant mutations found in this patient has never been published before.

Late-Onset Retinal Findings and Complications in Untreated Retinopathy of Prematurity.

PURPOSE: To investigate late retinal findings and complications of eyes with a history of retinopathy of prematurity (ROP) that did not meet treatment criteria and did not receive treatment during infancy. DESIGN: Retrospective, nonconsecutive, noncomparative, multicenter case series. PARTICIPANTS: Three hundred sixty-three eyes of 186 patients. METHODS: Data were requested from multiple providers on premature patients with a history of ROP and no treatment during infancy who demonstrated late retinal findings or complications and included age, gender, gestational age and weight, zone and stage at infancy, visual acuity, current retina vascularization status, vitreous character, presence of peripheral retinal findings such as lattice retinal tears and detachments (RDs), retinoschisis, and fluorescein findings. MAIN OUTCOME MEASURES: Rate of RDs and factors conferring a higher risk of RDs. RESULTS: The average age was 34.5 years (range, 7-76 years), average gestational age was 26.6 weeks (range, 23-34 weeks), and average birth weight was 875 g (range, 425-1590 g). Findings included lattice in 196 eyes (54.0%), atrophic holes in 126 eyes (34.7%), retinal tears in 111 eyes (30.6%), RDs in 140 eyes (38.6 %), tractional retinoschisis in 44 eyes (11.9%), and visible vitreous condensation ridge-like interface in 112 eyes (30.5%). Fluorescein angiography (FA) was performed in 113 eyes, of which 59 eyes (52.2%) showed leakage and 16 eyes (14.2%) showed neovascularization. Incomplete vascularization posterior to zone 3 was common (71.6% of eyes). Retinal detachments were more likely in patients with a gestational age of 29 weeks or less (P < 0.05) and in eyes with furthest vascularization to posterior zone 2 eyes compared with zone 3 eyes (P = 0.009). CONCLUSIONS: Eyes with ROP not meeting the treatment threshold during infancy showed various late retinal findings and complications, of which RDs were the most concerning. Complications were seen in all age groups, including patients born after the Early Treatment for Retinopathy of Prematurity Study. Contributing factors to RDs included atrophic holes within peripheral avascular retina, visible vitreous condensation ridge-like interface with residual traction, and premature vitreous syneresis. We recommend regular examinations and consideration of ultra-widefield FA examinations. Prospective studies are needed to explore the frequency of complications and benefit of prophylactic treatment and if eyes treated with anti-vascular endothelial growth factor therapy are at risk of similar findings and complications.

Ocular manifestations in a family with brachio-oculo-facial syndrome.

We report 3 siblings with brachio-oculo-facial syndrome (BOFS) who present with the predominant ocular phenotype. This syndrome has rarely been reported in multiple first-degree relatives.

A unique case of vision loss in a patient with hypotrichosis and juvenile macular dystrophy and primary ciliary dyskinesia.

PURPOSE: We describe a unique case of CDH3-related hypotrichosis with juvenile macular dystrophy (HJMD) and DNAH5-related primary ciliary dyskinesia (PCD) with progressive vision loss in a young Indian female without positive family history. Both mutations in this patient have not been previously described in the literature. OBSERVATIONS: An 11-year-old girl of Indian descent from a consanguineous family presented to our clinic with poor central visual acuity, recurrent sinopulmonary infections, hypotrichosis, and gradual hearing loss. Fundus examination was significant for atrophic retinal pigmented epithelial (RPE) changes involving both the macula and periphery of both eyes with central foveal hypoautofluorescence. Optical coherence tomography (OCT) demonstrated RPE loss and significant disruption of the ellipsoid layer in both eyes. Full-field electrophysiology tests on initial presentation demonstrated low cone amplitude reduced to <70% of normal range without prolongation. OCT angiography of the RPE and choriocapillaris demonstrated possible flow voids in the central macular region of both eyes. Genetic testing showed that the proband was homozygous for variants CDH3 c.1660A > C; p. Thr554Pro and DNAH5 c.6688-1G>T. CONCLUSION: and Importance: We report two novel variants in the CDH3 and DNAH5 genes that are important for future mutational analysis of both HJMD and PCD respectively. A relationship between the cadherin protein dysfunction in CDH3 mutations and the ciliopathy of DNAH5 mutations has not been established. HJMD is known to cause a longitudinal deterioration of cone and rod mediated function, therefore recognizing the symptoms, visual impairment, physical examination, and photographic and electrophysiological findings is crucial in counseling the patient, the family, and fellow clinicians.

Delayed Onset of Retinopathy of Prematurity Associated With Mitochondrial Dysfunction and Pearson Syndrome.

Retinopathy of prematurity (ROP) is a biphasic disease in which the first phase is characterized by high oxygen tension leading to vaso-obliteration in the retina. Pearson syndrome is a rare multisystem mitochondrial disease with a defect in cellular respiration. The authors describe a patient with Pearson syndrome and delayed onset of ROP at a postconceptual age of 42 weeks. The proposed mechanistic theory was the increased oxygen use associated with the metabolic impairments in Pearson syndrome counterbalancing the effects of supplemental oxygen during the vaso-obliterative stage of ROP. [J Pediatr Ophthalmol Strabismus. 2019;56:e60-e64.].