Mimickers of anterior uveitis, scleritis and misdiagnoses- tips and tricks for the cornea specialist.

BACKGROUND: Anterior uveitis, inflammation of the anterior chamber and related structures, is a cohort of diseases that can present to almost any general or sub-specialty Ophthalmology practice. Its features classically involve anterior chamber cell and flare. Below the surface of these two signs exist a panoply of diagnoses. BODY: The purpose of this review is to provide a general framework for diagnoses of anterior uveitis that are often missed as well as non-uveitic pathologies that often mimic anterior uveitis. Diagnostic deviation in either direction can have vision-threatening and rarely life-threatening consequences for patients. Using a comprehensive literature review we have collected a broad spectrum of etiologies of anterior uveitis that are easily missed and non-uveitic pathologies that can masquerade as anterior uveitis. CONCLUSIONS: We present a focused review on specific misdiagnosed anterior uveitis pathologies and some of the conditions that can masquerade as anterior uveitis and scleritis.

Peripapillary intrachoroidal cavitation at the crossroads of peripapillary myopic changes.

AIM: To analyze the prevalence of peripapillary intra-choroidal cavitation (PICC) in eyes with gamma peripapillary atrophy (γPPA), in eyes with peripapillary staphyloma (PPS) and in those combining γPPA and PPS and to analyze border tissue discontinuity in PICC. METHODS: This prospective cross-sectional non interventional study included highly myopic eyes. Non-highly myopic eyes were used as control. Radial and linear scans centered on the optic nerve head were performed using spectral-domain optical coherence tomography. Variables were analyzed along the twelve hourly optical coherence tomography sections in both eyes of each subject. RESULTS: A total of 667 eyes of 334 subjects were included: 229 (34.3%) highly myopic eyes and 438 (65.7%) non highly myopic eyes. The mean age of the highly myopic group was 48.99±17.81y. PICC was found in a total of 40 eyes and in 13.2% (29/220) of highly myopic eyes. PICC was found in 10.4% (40/386) of eyes with γPPA, in 20.5% (40/195) of eyes with PPS and in 22.7% (40/176) of those combining γPPA and PPS. All the eyes with PICC showed the co-existence of γPPA and PPS whereas none of the eyes presenting only one of these entities exhibited PICC. A border tissue discontinuity in the γPPA area was found in all eyes with PICC. CONCLUSION: We confirm the presence of a border tissue discontinuity in the γPPA area of all eyes with PICC. These findings suggest the involvement of mechanical factors in the pathogenesis of PICC which may contribute to PICC-related visual field defects.

Myopic (Peri)papillary Changes and Visual Field Defects.

Purpose: Myopic eyes combining gamma peripapillary atrophy and peripapillary staphyloma were sorted according to the presence of intrachoroidal cavitation (PICCs) or its absence (combinations). Visual field defects (VFDs) and factors discriminating these groups were analyzed. Methods: These groups were sorted by optical coherence tomography. VFDs were assessed using the Humphrey® Field Analyzer 3, SITA standard. Ovality index (OI) was the ratio between the shortest and longest diameters of the disc. The proportions of PICCs, lamina cribrosa defects (LCDs) and clusters in each Garway-Heath's sector (A-F) were analyzed. All variables were compared between PICCs and combinations. A multivariate logistic regression analysis was performed ultimately. Results: Of the 93 eyes, we obtained, 20 PICCs and 73 combinations. The prevalence of VFDs and LCDs in PICCs were 65% (13/20) and 30% (6/20), respectively. PICCs 85% (17/20) and LCDs 12% (11/93) predominated in sector B (inferotemporal) and clusters 9.7% (9/93) in the corresponding sector. The proportion of VFDs was significantly higher in PICCs than combinations (p < 0.001). In sector B, the proportion of LCDs was significantly higher in PICCs than combinations (p = 0.011). The mean OI was significantly lower (p < 0.001) in PICCs than combinations. Multivariate logistic regression analysis concluded that mean OI (p < 0.001) was the only statistically significant factor discriminating PICCs and combinations. Conclusion: Mean OI discriminating PICCs from combinations is further evidence of a gradation of structural changes between them. It could be related to the higher proportion of VFDs in PICCs. The predominant distribution of PICCs infero-temporally supports PICC as a cause of uncertainty in glaucoma diagnosis in high myopia. Furthermore, the highest proportion of PICCs and LCDs in this sector highlights its vulnerability to damage in myopic eyes and deserves further investigation as it is also primarily involved in glaucoma.

Intravitreal methotrexate in vemurafenib-induced uveitis.

PURPOSE: Vemurafenib, a BRAF inhibitor, has revolutionized the prognosis of late-stage melanoma patients, rising at the same time concerns about its potential adverse effects. Here is a case of vemurafenib-induced uveitis, peculiar in both its presentation and management. METHODS: Case report, diagnostic and therapeutic challenge. RESULTS: Uveitis is a known side effect of vemurafenib. It is generally bilateral, moderate, manageable with topical steroids and does not require cancer therapy cessation. We present a patient who suffered from a unilateral, severe uveitis following vemurafenib treatment that fully recovered thanks to intravitreal methotrexate injections, as conventional corticosteroid therapy was contraindicated. CONCLUSION: Uveitis can be a serious ocular adverse effect of vemurafenib, while its risks factors and mechanisms remain unknown. As BRAF inhibitors are now used on a regular basis, it is important for clinicians to be aware of this potentially sight-threatening side effect. Intravitreal methotrexate injections may be considered as an effective treatment choice in severe targeted agents-induced uveitis.

Active Ocular Toxoplasmosis Associated with Simultaneous Multiple Evanescent White Dot Syndrome.

PURPOSE: We describe the association between active ocular toxoplasmosis and Multiple Evanescent White Dot Syndrome (MEWDS). PATIENTS AND METHODS: Retrospective observational case report of a patient who presented with concomitant ocular toxoplasmosis and MEWDS (Erasmus University Hospital, Brussels, Belgium). Clinical record and multimodal imaging including fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral domain optical coherence tomography (SD-OCT) were analyzed. RESULTS: Multimodal imaging of a 25-year-old-woman that presented with concomitant active ocular toxoplasmosis and MEWDS is described. Both clinical entities regressed completely after 8 weeks under steroidal anti-inflammatory drugs and antibiotics. CONCLUSION: Active ocular toxoplasmosis can be associated with simultaneous multiple evanescent white dot syndrome. Further reports are needed to precise and characterize this clinical relationship and its management.Abbreviations: MEWDS: Multiple Evanescent White Dot Syndrome; Fundus Autofluorescence FAF; BCVA: Best-corrected Visual Acuity; FA: Fluorescein Angiography; ICGA: Indocyanine Green Angiography; SD-OCT: Spectral Domain Optical Coherence Tomography; IR: Infrared.

Analysis of Peripapillary Intrachoroidal Cavitation and Myopic Peripapillary Distortions in Polar Regions by Optical Coherence Tomography.

Purpose: To compare the peripapillary polar characteristics in eyes combining peripapillary staphyloma and gamma peripapillary atrophy according to whether peripapillary intrachoroidal cavitation (PICC) was present or absent (combination-group). Patients and methods: This prospective non-interventional cross-sectional study included 667 eyes of 334 subjects. From the polar peripapillary regions to the opening of Bruch's membrane, the following elements and their topographic relationships were analyzed using optical coherence tomography sections: configuration of the posterior curvature of the choroid, visibility of the subarachnoid space (SAS), and suprachoroidal detachment (SCD). Chi-squared and Fisher exact tests were used for statistical analysis. Results: The protrusion of the posterior choroidal wall, with anterior elevation on either side, observed in both groups progressed and transformed into a wedge-shaped deformity on the side of gamma peripapillary atrophy. This wedge configuration was significantly more frequent in PICC-group than in combination-group (p = 0.004 and p < 0.001) for the upper and lower poles, respectively. SAS was more frequently observed in PICC-group than in combination-group (p = 0.002 and p < 0.001) for the upper and lower poles, respectively. SCD was detected exclusively in PICC-group (p < 0.001, both poles). The wedge-shaped configuration and the SCD were aligned antero-posteriorly with the SAS. Conclusion: We confirmed that PICC is an SCD. We observed its constant alignment with the SAS. We suggest that the tensile forces of the optic nerve sheaths during adduction promote the collapse of the scleral flange onto the SAS, leading to PICC. Further studies are warranted to confirm this hypothesis.

Necrotising retinopathy-like lesions as a manifestation of ocular sarcoidosis.

BACKGROUND: A 56-year-old Caucasian man presented with a 2-weeks history of decreased vision in the right eye. Vitritis, papillitis, cystoid macular oedema and inferior diffuse retinal infiltration were noticed. Extensive blood work-up, anterior chamber paracentesis with polymerase chain reaction (PCR) and Goldmann-Witmer coefficient, tuberculin skin test (PPD-test), fluorodeoxyglucose Positron Emission Tomography CT scan (FDG-PET/CT), lymph node biopsy and pars plana vitrectomy were performed. RESULTS: Aqueous and vitreous samples were negative for an infectious and a lymphoproliferative etiology. Enlarged hilar and mediastinal lymph nodes were detected by FDG-PET/CT and subsequently biopsied, allowing to confirm the diagnosis of sarcoidosis. After a few months of oral corticosteroid therapy, the inflammation resolved completely and was replaced by atrophic retinal scars. CONCLUSION: Necrotising retinopathy-like lesions mimicking an infectious process or a lymphoproliferative disorder can be an atypical manifestation of ocular sarcoidosis.

Central Serous Chorioretinopathy Associated with Desmopressin Nasal Spray: Causality or Unfortunate Association.

PURPOSE: To describe the possible association between central serous chorioretinopathy (CSCR) and desmopressin use. METHODS: The case histories of 2 middle-aged men with CSCR using desmopressin nasal spray were studied. RESULTS: The diagnosis of CSCR was made on the basis of clinical features and ancillary testing (fluorescein angiography and optical coherence tomography). Both patients were using desmopressin nasal spray for polyuria when they developed the first ocular symptoms. Both of them also had an independent risk factor for developing CSCR. CONCLUSION: We suggest that desmopressin-induced hypercortisolism might implicate the development of CSCR in some patients. A larger study on patients using desmopressin nasal spray would be beneficial to confirm the possible association between this form of therapy and the development of CSCR.

Structural and functional retinal changes in eyes with DUSN.

PURPOSE: To report novel spectral domain optical coherence tomography and electrophysiologic findings in diffuse unilateral subacute neuroretinitis. METHODS: Six patients with a diagnosis of diffuse unilateral subacute neuroretinitis were retrospectively ascertained. All patients had received oral treatment with albendazole; resolution of the inflammatory lesions without subsequent relapse was noted. Spectral domain optical coherence tomography was performed using a Spectralis HRA OCT (Heidelberg Engineering). The inner and outer retinal volumes were calculated for the macular area. The contralateral eyes acted as controls. All six patients underwent standardized full-field electroretinography and pattern electroretinography. Some had multifocal electroretinography. RESULTS: Inner retinal volume significantly differed between affected and control eyes (P < 0.02), but there was no significant difference in outer retinal volume. Electroretinography data showed a mixed pattern of inner and outer retinal dysfunction, with inner retinal dysfunction being greater; reduction in b:a ratio of the scotopic bright flash electroretinography was a consistent observation in those patients (5/6) with generalized retinal dysfunction. Two patients showed definite photoreceptor involvement, with probable involvement in a third. Of the four patients in whom serial data are available, there was definite evidence of progressive inner and outer retinal dysfunction in one patient, with inner retinal dysfunction being greater, and probably in a second patient. CONCLUSION: The data provide anatomical and functional evidence of both inner and outer retinal dysfunction in diffuse unilateral subacute neuroretinitis, even though the worm is usually assumed to be located in the subretinal space. The mechanism is unclear.

Evolution of an Astrocytic Hamartoma of the Optic Nerve Head in a Patient with Retinitis Pigmentosa - Photographic Documentation over 2 Years of Follow-Up.

AIM: To report photographically the evolution of an astrocytic hamartoma of the left optic nerve head over a 2-year follow-up in a patient with retinitis pigmentosa. METHODS: A 14-year-old boy was seen in the medical retina clinic with a 3-year history of night blindness. Best corrected visual acuity was 6/18 in both eyes. Colour vision was normal in both eyes and confrontation fields showed peripheral constriction. Fundus examination revealed bone spicule pigmentary changes at the retinal mid periphery typical of retinitis pigmentosa and superficial globules at the margins of both optic nerve heads. Electrodiagnostic tests confirmed moderately severe rod cone dystrophy with macular involvement bilaterally. RESULTS: Two years later, the ocular examination was unchanged except for the appearance of the optic nerve head lesion in the left eye. There was an increase in the size of the lesion which was diagnosed as an astrocytic hamartoma. Further investigations were recommended to exclude neurofibromatosis and tuberous sclerosis. CONCLUSION: Astrocytic hamartomas of the optic nerve head and optic nerve head drusen have both been described in patients with retinitis pigmentosa. They can be a diagnostic dilemma although drusen are more common (10%). To differentiate these two entities it is very important to document any growth during the follow-up period which is suggestive of astrocytic hamartoma rather than optic disc drusen.

Complete resolution of a giant pigment epithelial detachment secondary to exudative age-related macular degeneration after a single intravitreal ranibizumab (lucentis) injection: results documented by optical coherence tomography.

AIM: To describe a patient with a giant pigment epithelial detachment (PED) secondary to exudative age-related macular degeneration (ARMD) successfully treated with a single intravitreal ranibizumab (Lucentis) injection (0.5 mg/0.05 ml). METHODS: An 89-year-old woman presented with a six-day history of reduced vision and distortion in the left eye. Best-corrected visual acuity in that eye was 6/15. Fundoscopy revealed a giant PED and exudates temporally to the fovea. Optical coherence tomography showed a PED associated with subretinal and intraretinal fluid. Fluorescein angiography confirmed the diagnosis of an occult choroidal neovascularization. Treatment with intravitreal injections of ranibizumab (Lucentis) was recommended, although the increased risk of retinal pigment epithelium (RPE) rip was mentioned. RESULTS: Four weeks after the first intravitreal Lucentis injection, the visual acuity in the left eye improved to 6/7.5, with a significant improvement of the distortion and a complete anatomical resolution of the PED confirmed by optical coherence tomography. CONCLUSION: Giant PED secondary to exudative ARMD can be successfully treated with intravitreal ranibizumab, despite the increased risk of RPE rip. To our knowledge, this is the first case presenting with complete resolution of PED after a single ranibizumab injection.

Optic neuropathy, renal failure and pulmonary sarcoidosis in a 50-year-old man: where is the link?

Eye disorders are frequently associated with renal diseases, mostly linked to underlying causes such as hypertension, diabetes or autoimmune diseases. Conversely, advanced uraemic states may also lead to progressive vision impairment. The present report concerns a 50-year-old patient who presented with a bilateral, painless, progressive vision loss, a moderate systemic inflammation and chronic renal failure due to hypertension nephrosclerosis. Steroids were given and haemodialysis was initiated, resulting in vision improvement. At 4 months later when the steroids were stopped, the patient developed dyspnoea, cough, fever and fatigue of unclear origin. A lung biopsy showed non-caseating granuloma consistent with pulmonary sarcoidosis. Re-challenge with steroids rapidly improved the respiratory disease. Ophthalmological examinations performed early and later in the course excluded anterior ischaemic optic neuropathy and ocular manifestations of sarcoidosis, leading to a diagnosis of uraemic optic neuropathy. This rare ophthalmological disorder should be promptly recognised since haemodialysis and steroid therapy are highly effective.