Pediatric Pars Planitis: A Review.

PURPOSE: To provide an overview of pediatric pars planitis. METHODS: Narrative literature review. RESULTS: Pars planitis refers to the idiopathic subset of intermediate uveitis in which there is vitritis along with snowball or snowbank formation occurring in the absence of an associated infection or systemic disease. It is thought to be a T-cell mediated disease with a genetic predisposition. Pars planitis accounts for 5-26.7% of pediatric uveitis cases. Presentation is commonly bilateral but asymmetric, often with insidious onset of floaters and blurred vision. Although pars planitis is known to be a benign form of uveitis in most cases, severe complications secondary to chronic inflammation may arise, with cystoid macular edema being the most common cause of visual morbidity. Mild vitritis in the absence of symptoms, vision loss, or macular edema may be observed. Patients with severe vitritis and/or associated vision-threatening complications require prompt aggressive treatment. A stepladder approach including corticosteroids, immunosuppressive agents, anti­tumor necrosis factor­alpha and pars plana vitrectomy and/or laser photocoagulation is the most commonly used method for treatment of pars planitis. CONCLUSION: Timely diagnosis and adequate treatment of pediatric pars planitis and associated complications are crucial in order to improve visual outcomes.

SS OCT and OCT-A Findings in Convalescent Inactive Vogt-Koyanagi-Harada Disease.

PURPOSE: This study aimed to determine predictive factors for low final visual acuity in patients with inactive Vogt-Koyanagi-Harada (VKH) disease using Swept-source OCT and OCT-angiography (OCT-A). METHODS: We conducted a prospective longitudinal study, including 21 patients (42 eyes), who were followed up for the first 24 months after disease onset. Patients were included at the acute phase and subsequently treated. Sequential qualitative and quantitative changes in OCT and OCT-A were assessed. Analytical statistical methods were employed to determine predictive factors for final visual acuity. RESULTS: Structural alterations including focal parafoveal outer nuclear layer atrophy, ellipsoid zone disruption, interdigitation zone disruption, and irregular and thickened retinal pigment epithelium line were observed in 57.1% of eyes at month 3, with no significant improvement over time. The presence of flow voids at months 6, 12, and 24 was significantly associated with low final visual acuity. Serous retnal detachment at presentation emerged as an independent risk factor for structural changes detected by SS-OCT during the first 2 years of the disease. Optic disc edema was predictive factor for both structural SS-OCT and OCT-A changes. CONCLUSION: This study underscores the importance of monitoring flow voids to predict final visual acuity and highlights the impact of serous retinal detachment at presentation on structural changes in patients with inactive VKH disease.

A Comparative Study between Occlusive and Non-occlusive Retinal Vasculitis: Data from a Referral Center in Tunisia, North Africa.

PURPOSE: To compare clinical profile and visual outcomes of occlusive versus non-occlusive retinal vasculitis (RV). METHODS: A retrospective comparative study. RESULTS: 284 patients were enrolled, including 124 patients with occlusive RV (ORV) and 160 patients with non-occlusive RV (NORV). Patients with ORV were older (p ≤ 10-3), predominantly male (p ≤10-3), with less bilateral involvement (31.5% vs 53,4%; p ≤ 10-3). Infectious RV was more frequently diagnosed in the ORV group than in the NORV group (48.8% vs 32.9%, p = .006). Behçet disease and ocular tuberculosis were the leading causes of ORV. Idiopathic RV, Behçet disease, and sarcoidosis were the most common causes of NORV. Independent predictive factors of poor visual outcome were worse baseline visual acuity in both groups (p = .006 and p ≤ 10-3, respectively), and retinal hemorrhages (p = .048) and optic atrophy (p = .040) in the ORV group. CONCLUSION: Occlusive and non-occlusive RV have distinctive clinical and etiological profile.

Comments on "Goyal M, Murthy SI and Annum S, Bilateral Multifocal Choroiditis following COVID-19 Vaccination".

The fundus photographs provided show deep yellowish lesions, multifocal exudative retinal detachments, and optic disc hyperemia in both eyes. Spectral domain optical coherence tomography scans shown demonstrate bilateral exudative retinal detachments involving the macula in the right eye. The exudative retinal detachment is characterized by the presence of subretinal fibrin and septa. The B-scan ultrasonography provided shows low- to medium-reflective choroidal thickening predominating around optic nerve head, in association with exudative retinal detachment in the right eye. All these findings are highly suggestive of acute Vogt-Koyanagi-Harada (VKH) disease rather than multifocal choroiditis.

Swept-source Optical Coherence Tomography Angiography in Active and Scarred Toxoplasmic Retinochoroiditis.

PURPOSE: To describe optical coherence tomography angiography (OCTA) findings in eyes with active and scarred toxoplasmic retinochoroiditis. METHODS: OCTA scans in active (25 eyes) and scarred (17 eyes) retinochoroiditis were retrospectively reviewed. RESULTS: In active lesions, OCTA findings included a non-detectable flow signal area in retinal vascular plexuses and choriocapillaris in all 25 eyes (100%), an abnormal intraretinal vascular process in 2 eyes (8%), and an associated area of retinal flow deficit secondary to branch retinal artery occlusion in one eye (4%). In scarred lesions, OCTA findings included a flow deficit area in retinal vascular plexuses and choriocapillaris in all 17 eyes (100%), a visibility of larger deeper choroidal vessels at the level of choriocapillaris in 9 eyes (53%), and a well-defined intraretinal vascular network in one eye (5.9%). Peripapillary scars were associated on OCTA with wedge-shaped loss of radial peripapillary capillaries with corresponding localized retinal nerve fiber layer defect and visual field loss. CONCLUSION: OCTA allows to non-invasively detect retinal and choroidal vascular changes in active and scarred toxoplasmic retinochoroiditis.

Distinguishing swept-source optical coherence tomography findings in active toxoplasmic retinochoroiditis.

OBJECTIVE: To define the swept-source optical coherence tomography (SS-OCT) features which distinguish ocular toxoplasmosis (OT) from other forms of retinochoroiditis. METHODS: This is a prospective diagnostic study enrolling 43 eyes of 43 patients with active toxoplasmic (TOXO) retinochoroiditis matched to 54 eyes (54 lesions) with non-TOXO retinochoroiditis evaluated by structural SS-OCT. RESULTS: The finding of retinal hyper-reflective round deposits, sub-lesional choroidal thickening, and sub-lesional retinal pigment epithelium elevation were more likely to be found in TOXO lesions with a positive likelihood ratio of 45.2 (95% CI: 6.45-316.56), 23.86 (95% CI: 6.09-93.36), and 9.79 (95% CI: 4.22-22.7), respectively. The presence of each of these findings was associated with a high level for positive predictive value (PPV) (88.63-97.29), negative predictive value (NPV) (88.3-92.45), sensitivity (83.72-90.69), and specificity (90.74-98.14). Two-parameter model binary logistic regression suggested that sub-lesional retinal pigment epithelium elevation and sub-lesional choroidal thickening were significant predictors of the diagnosis of OT (Wald = 11.905, p < 0.001; Wald = 14.881, p < 0.001; respectively). By adding hyper-reflective round deposits along the posterior hyaloid or the retinal surface the model improved its performance with very good diagnostic accuracy with area under the curve (AUC) values of 0.96 (95% CI: 0.9-0.99) for two parameters model and 0.98 (95% CI: 0.93-0.99) for the three parameters model. CONCLUSIONS: Our results show that three OCT findings including retinal hyper-reflective round deposits, sub-lesional choroidal thickening, and sub-lesional retinal pigment epithelium elevation are more likely to occur in OT patients as compared with non-OT patients.

Is There a True Neuroretinitis in Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) Syndrome ?

PURPOSE: To clarify whether a true neuroretinitis is part of  idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. METHODS: Critical literature review. RESULTS: Neuroretinitis has been considered as a prominent feature of IRVAN syndrome. It has been described as late diffuse optic  disc staining on fluorescein angiography. However, patients with IRVAN syndrome usually do not present with visual function alterations consistent with optic neuropathy or neuroretinitis. The typical stellate exudative maculopathy seen in IRVAN syndrome is primarily caused by leakage from retinal arteriolar aneurysms at or near the optic disc, and it could be misinterpreted as a feature of neuroretinitis. CONCLUSION: There is no clinical evidence of true neuroretinitis at any of the disease stages in patients with IRVAN syndrome. The acronym « IRVARE ¼ (Idiopathic Retinal Vasculitis, Aneurysms, and Retinal Exudates) is proposed to better characterize this syndrome.

Multimodal Imaging of Acute Foveolitis following COVID-19 Vaccination.

PURPOSE: To report a case of unilateral acute foveolitis following COVID-19 vaccination. METHODS: A case report. RESULTS: A 24-year-old woman developed a sudden blurring of vision in the left eye (LE) 5 days after receiving the first dose of COVID-19 vaccine. Examination of the LE showed a visual acuity at 20/40, 2+ cells in the vitreous, and a small yellow-orange foveal subretinal lesion. Late-phase fluorescein angiography showed a mild diffuse retinal vascular leakage and a faint foveal hyperfluorescence. ICG angiography showed in the late-phase hypofluorescence of the centrofoveal lesion. OCT B-scan demonstrated a conical hyperreflective subfoveal lesion on the retinal pigment epithelium associated with disruption of the outer retinal layers. En-face OCT revealed granular hyperreflective specks mainly in the inner nuclear layer. Work-up results were unremarkable. The patient received oral prednisolone with subsequent full functional and anatomic recovery. CONCLUSION: Foveolitis may rarely occur as a complication of COVID-19 vaccination.

Rickettsial disease: An underestimated cause of posterior uveitis.

Rickettsioses are worldwide distributed infectious disease caused by intracellular small Gram-negative bacteria transmitted to humans by the bite of contaminated arthropods, such as ticks. Systemic disease typically consists of a triad of high fever, headache, and skin rash. It usually has a self-limited course, but severe, life-threatening complications can sometimes occur. It may be clinically difficult to differentiate rickettsial diseases from other febrile illnesses. Rickettsial infection has been largely underestimated as a cause of infectious uveitis for long decades in the past. Conversely, recent data show that ocular involvement is much more common than previously thought, with retinitis, retinal vasculitis, and neuroretinitis being the most typical and frequent findings. Early clinical diagnosis of rickettsial disease, while awaiting laboratory test results, is essential for prompt initiation of appropriate antibiotic treatment to prevent systemic and ocular morbidity. The prevention remains the mainstay of rickettsial infection control.

Preoperative Intralesional Bevacizumab Injection in Primary Pterygium in Tunisian Patients: A Randomized Controlled Prospective Study.

Purpose: To assess the efficacy and safety of a single preoperative intralesional bevacizumab injection as an adjuvant treatment before primary pterygium surgery. Methods: We conducted a randomized controlled interventional study from January 2019 to December 2020. The study included a total of 60 patients (60 eyes) with primary pterygium. We defined two groups of 30 patients each. Group A received an intralesional injection of bevacizumab (Avastin), 1 month before surgery (lesion excision and conjunctival autograft). Group B (control) had only the surgical treatment. Patients were followed up 7 days (D7), 1 month (M1), 3 months (M3), and 6 months (M6) postoperatively. Pre-, per-, and postoperatively, photographs of the lesions were taken, as well as a histopathological examination. The main outcome measures were the change in functional discomfort following intralesional bevacizumab injection and pterygium recurrence. Recurrence was defined as fibrovascular tissue growth invading the cornea. Therapeutic success was defined as the absence of pterygium recurrence in M6. Results: The mean age of the 60 patients was 54.17 ± 10.53. After bevacizumab injection, the preoperative functional discomfort score decreased significantly (P = 0.048). There was a significant improvement in grade and color intensity (P = 0.001). We noted no local nor systemic complications after intralesional injection of bevacizumab. After pterygium excision, the success rate was statistically higher in Group A (P = 0.047). There was no significant difference in either final best-corrected spectral visual acuity or astigmatism between the two groups. We noted a statistically significant association between recurrence and color intensity (P = 0.046), vascular density (P = 0.049), and the degree of elastic tissue degeneration (P = 0.040). Conclusion: A single preoperative subconjunctival injection of bevacizumab 1 month before surgery decreases the vascularity of newly formed blood vessels and hence may reduce the recurrence rate.

Ocular Manifestations of Emerging Arthropod-Borne Infectious Diseases.

PURPOSE: To review the clinical features, diagnosis, treatment modalities, and prognosis of arthropod-borne infectious diseases. METHODS: This is a narrative review on arthropod-borne infectious diseases including general and ophthalmological aspects of these infectious diseases. A comprehensive literature review between January 1983 and September 2020 was conducted in PubMed database. Epidemiology, clinical features, diagnosis, treatment, and prognosis of arthropod-borne infectious diseases were reviewed. RESULTS: Emergent and resurgent arthropod-borne infectious diseases are major causes of systemic morbidity and death that are expanding worldwide. Among them, bacterial and viral agents including rickettsial disease, West Nile virus, Dengue fever, Chikungunya, Rift valley fever, and Zika virus have been associated with an array of ocular manifestations. These include anterior uveitis, retinitis, chorioretinitis, retinal vasculitis, and optic nerve involvement. Proper clinical diagnosis of any of these infectious diseases is primarily based on epidemiological data, history, systemic symptoms and signs, and the pattern of ocular involvement. The diagnosis is confirmed by laboratory tests. Ocular involvement usually has a self-limited course, but it can result in persistent visual impairment. Doxycycline is the treatment of choice for rickettsial disease. There is currently no proven specific treatment for arboviral diseases. Prevention remains the mainstay for arthropod vector and zoonotic disease control. CONCLUSIONS: Emerging arthropod vector-borne diseases should be considered in the differential diagnosis of uveitis, especially in patient living or with recent travel to endemic countries. Early clinical diagnosis, while laboratory testing is pending, is essential for proper management to prevent systemic and ocular morbidity.

Hypopyon: Is-it Infective or Noninfective?

Hypopyon usually corresponds to the sedimentation of white blood cells, and it signifies severe intraocular inflammation. This key clinical sign may occur in association with a wide variety of infectious, inflammatory, and neoplastic conditions that may be sight- and, occasionally, life-threatening. A careful history and thorough clinical examination are the cornerstones for orienting the differential diagnosis, identifying the causative agent, and initiating prompt and appropriate treatment. This review outlines the clinical characteristics and management of hypopyon in relation with the underlying causative infectious or noninfectious ocular or systemic diseases.

Results of Intravitreal Anti-Vascular Endothelial Growth Factor Therapy in Inflammatory Choroidal Neovascularization.

PURPOSE: To report the visual outcomes of intravitreal (IVT) anti-vascular endothelial growth factor (anti-VEGF) in inflammatory choroidal neovascularization (iCNV). METHODS: A retrospective study of 43 eyes of 38 patients with active choroidal neovascularization (CNV) related to ocular inflammatory disease, treated with IVT injections of anti-VEGF (bevacizumab, ranibizumab, or aflibercept), with or without associated systemic anti-inflammatory therapy, at Fattouma Bourguiba University Hospital, Monastir, Tunisia (24 eyes of 23 patients) and at Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy (19 eyes of 15 patients) from January 1, 2013, to December 31, 2018. RESULTS: The mean age was 35.5 ± 16.4 years. The sex ratio male:female was 0.27. Seventeen eyes (39.5%) of 17 patients (44.7%) had only anti-VEGF injections, and 26 eyes (60.5%) of 21 patients (45.3%) had anti-VEGF injections and associated systemic anti-inflammatory therapy. Bevacizumab was injected in 36 eyes (83.7%), ranibizumab in six eyes (14%), and aflibercept in one eye (2.3%). Mean follow-up was 20.3 ± 19.2 months (range, 6-106 months). Mean visual acuity improved from 0.8 ± 0.37 logMAR (approximate Snellen equivalent 20/125) to 0.51 ± 0.42 logMAR (approximate Snellen equivalent 20/63) (P < 0.001). Mean central macular thickness on optical coherence tomography decreased from 403.7 ± 121.9 to 293.7 ± 82.8 µm (P < 0.001). Mean gain of vision was 2.9 ± 3.1 lines. The mean number of injections was 2.5. Twenty eyes (46.5%) received a single injection. There were no side effects related to the IVT injections of anti-VEGF. CONCLUSIONS: CNV is a sight-threatening complication of uveitis. IVT anti-VEGF seems to be an effective and safe treatment for iCNV when inflammation is controlled.

Abducens nerve palsy with associated retinal involvement secondary to rickettsia typhi infection.

OBJECTIVE: To report a case of abducens nerve palsy with associated retinal involvement due to rickettsia typhi infection. MATERIAL AND METHODS: A single case report documented with multimodal imaging. RESULTS: A 18-year-old woman with a history of high-grade fever was initially diagnosed with typhoid fever and treated with fluoroquinolone. She presented with a 5-day history of diplopia and headaches. Her best-corrected visual acuity was 20/20 in both eyes. Ocular motility examination showed left lateral gaze restriction. Lancaster test confirmed the presence of left abducens palsy. Fundus examination showed optic disc swelling in both eyes associated with superotemporal retinal hemorrhage and a small retinal infiltrate with retinal hemorrhage in the nasal periphery in the left eye. Magnetic resonance imaging (MRI) of the brain and orbits showed no abnormalities. A diagnosis of rickettsial disease was suspected and the serologic test for Richettsia Typhi was positive. The patient was treated with doxycycline (100 mg every 12 h) for 15 days with complete recovery of the left lateral rectus motility and resolution of optic disc swelling, retinal hemorrhages, and retinal infiltrate. CONCLUSION: Rickettsial disease should be considered in the differential diagnosis of abducens nerve palsy in any patient with unexplained fever from endemic area. Fundus examination may help establish an early diagnosis and to start an appropriate rickettsial treatment.

Role of Anterior Segment Optical Coherence Tomography in Monitoring Epidemic Keratoconjunctivitis.

PURPOSE: To describe, through anterior segment optical coherence tomography (AS-OCT) images, the different phases of epidemic keratoconjunctivitis (EKC) and show the impact of topical steroid on the course of this disease. METHODS: A prospective observational study included 38 eyes (28 patients) with a presumed EKC complicated by subepithelial infiltrates (SEIs) and treated with topical fluorometholone. Slit-lamp examinations and serial AS-OCT were performed on days 7, 14, 30, 90, 180, and 210. The thickness of the whole cornea and thickness of the corneal epithelium were measured with AS-OCT and correlated to the clinical findings on slit-lamp examination in the different EKC phases. RESULTS: In all patients, on day 7, the AS-OCT showed hyperreflective dots that were limited to the epithelial layers of the cornea and slightly rising above the epithelial surface, corresponding to the confluence of the punctuations of punctate epithelial keratitis. On day 14, a hyperreflective line in the Bowman's layer with intact epithelium was noted. On day 30, this hyperreflective band extended rapidly toward the anterior stroma, becoming thicker and more intense, corresponding to the SEI. They gradually decreased in number, intensity, and extent following treatment by topical steroids in 71.4% of the cases. Only two patients had persistent SEI. They were refractory to 3-month treatment by steroids. In these patients, who had persistent SEI, AS-OCT showed that the hyperreflective areas became well-defined, plaque-like lesions with sharp margins associated with disruption of Bowman's layer, localized epithelial thickening, stromal thinning, and a decrease in pachymetry. CONCLUSION: AS-OCT can be a valuable tool that provides a range of characteristic patterns of EKC and helps in monitoring it.

OPTICAL COHERENCE TOMOGRAPHY FEATURES OF CHOROIDAL NEOVASCULARIZATION AND THEIR CORRELATION WITH AGE, GENDER, AND UNDERLYING DISEASE.

PURPOSE: To investigate the influence of age, gender, and underlying disease on the optical coherence tomography (OCT) features of choroidal neovascularization (CNV) secondary to inflammation, myopia (mCNV), and age-related macular degeneration (AMD-CNV). METHODS: Demographic and clinical data of eyes with treatment-naive inflammatory CNV, mCNV, and Type 2 AMD-CNV were collected. Optical coherence tomography images were reviewed to determine the presence of pitchfork sign, pigment epithelial detachment, subretinal fluid (SRF), intraretinal cysts, subretinal hyperreflective material, atrophy, and outer retinal disruption graded 1 to 4. The influence of demographics and underlying etiology on OCT signs was investigated. RESULTS: One hundred and eighty-five eyes from 179 patients were enrolled. The mean [SD] age was 36 [±14.4], 62 [±18], and 77 [±8] for the inflammatory CNV, mCNV, and AMD-CNV, respectively (P < 0.001). Multiple linear regression showed that the presence of pitchfork sign was negatively associated with age (P < 0.0001), regardless of underlying disease. By contrast, the SRF, pigment epithelial detachment, intraretinal cysts, and the outer retinal disruption were all positively influenced by age, regardless of gender and underlying disease (all P < 0.01). Logistic regression showed that none of the OCT signs increased the likelihood for diagnosis of inflammatory CNV. By contrast, the absence of SRF was suggestive for mCNVs, and the presence of pigment epithelial detachment and SRF was suggestive for AMD-CNVs. CONCLUSION: The age of the patient had a significant effect on the OCT appearance of the CNV, particularly the presence of a pitchfork sign, regardless of the underlying etiology. The absence of SRF was suggestive for a diagnosis of mCNVs. The presence of SRF and pigment epithelial detachment was suggestive for AMD-CNVs.

Sequential bilateral Behçet's neuroretinitis associated with prepapillary vitreous exudate: case report.

OBJECTIVE: To describe a case of Behçet disease (BD) uveitis manifesting with sequential bilateral neuroretinitis associated with prepapillary inflammatory vitreous exudate (PIVE). MATERIAL AND METHODS: A single case report documented with multimodal imaging. RESULTS: A 37-year-old man developed neuroretinitis with associated PIVE in the left eye. He was diagnosed with ocular toxoplasmosis and treated accordingly based on positive serologic testing and negative work-up for other entities, including BD. The disease course was favorable, but 1 year later a similar neuroretinitis developed in the right eye. Extraocular features of BD became evident only at the time of the second eye involvement, and the patient received corticosteroid and immunosuppressive therapy. Swept source (SS) OCT showed at the acute phase in both eyes a typical "mushroom-shaped" prepapillary hyperreflectivity of the PIVE. SS OCT angiography (OCTA) demonstrated a corresponding prepapillary hypointense area due to shadowing effect, decreasing in size while scanning deeper layers. It also detected peripapillary retinal hypervascularity in both eyes and a sectoral area of flow signal loss in the first involved left eye. Visual acuity improved following the resolution of the PIVE and associated acute inflammatory changes in both eyes. The left eye showed residual optic disc pallor and retinal nerve fiber layer defects. CONCLUSION: Sequential bilateral neuroretinitis associated with PIVE may occur before other clinical features of BD become evident. SS OCT and OCTA can provide useful information for the diagnosis and management of this rare, but typical, ocular manifestation of BD uveitis.