A Case Series of "Solitary Idiopathic Choroiditis" and Proposal of a Nomenclature Change to "Idiopathic Scleroma".

OBJECTIVE: To define the characteristics of solitary idiopathic choroiditis (SIC) in a consecutive series of patients and propose a nomenclature change to idiopathic scleroma. MATERIALS AND METHODS: Electronic patient records were retrospectively interrogated to identify all patients diagnosed with SIC between 2002 and 2019 in a tertiary referral ophthalmic hospital in the United Kingdom. RESULTS: Thirty-four eyes of 34 patients were found to have SIC. The mean age at diagnosis was 48 years (range 24-78) and 23 patients (68%) were female. All lesions were located posterior to the equator, most frequently in the inferotemporal quadrant (13 eyes, 38%). The lesions had a mean largest basal diameter of 1.2 ± 0.4 disc diameters (range 0.5-2) and their distance to the optic disc had a mean of 1.2 ± 0.9 disc diameters (range 0-3.3). All lesions were intrascleral on enhanced depth imaging optical coherence tomography, demonstrating a hypo-reflective zone within the sclera, with an underlying hyper-reflective zone in some cases. No lesion enlarged or developed features consistent with active inflammation after a median follow-up time of 0.9 years (range 0-16.8). DISCUSSION/CONCLUSION: Optical coherence tomography shows SIC to be an intrascleral lesion. Furthermore, we found no evidence of any inflammatory component. A nomenclature change to idiopathic scleroma is appropriate to prevent unnecessary investigation.

Enhanced depth imaging of the choroid in patients with neovascular age-related macular degeneration treated with anti-VEGF therapy versus untreated patients.

PURPOSE: To compare the subfoveal choroidal thickness (SFCT) between patients with neovascular age-related macular degeneration (nAMD) who had multiple intravitreal injections of anti-vascular endothelial growth factor (anti-VEGF) agents and those with treatment-naïve nAMD. METHODS: This retrospective case-control study included 15 patients in group 1 (nAMD in one eye which had received at least three anti-VEGF injections and early AMD in the fellow eye) and 15 patients in group 2 (newly diagnosed nAMD in one eye which had not received any treatment and early AMD in the fellow eye). They underwent enhanced depth imaging optical coherence tomography (OCT), and two OCT readers manually measured the SFCT. Inter-ocular difference in SFCT (nAMD eye minus fellow eye) was calculated for each patient. RESULTS: The nAMD eyes in group 1 had received a median (range) of four (3-8) intravitreal injections of anti-VEGF agents, and the OCT scans were performed at a median (range) of 9 (4-17) months after the first injection. The median inter-ocular difference in SFCT in groups 1 and 2 were not significantly different (13.5 and 3.0 µm in groups 1 and 2 respectively, p=0.60). There was also no statistically significant difference in SFCT between nAMD and fellow eyes (p=0.16), although there was a trend for greater median SFCT in the nAMD eyes. CONCLUSION: The data from this small cohort suggests that no gross reduction in SFCT appears in nAMD patients after a time interval of at least 4 months between initiating repeated treatment with anti-VEGF therapy and OCT imaging. However, a study with a much larger sample size or longitudinal design is required to detect possible small fluctuations in SFCT in nAMD eyes receiving anti-VEGF therapy.

Use of spectral-domain optical coherence tomography to differentiate acquired retinoschisis from retinal detachment in difficult cases.

PURPOSE: This study presents a series of 10 patients who posed a diagnostic challenge regarding the diagnosis of acquired retinoschisis (RS) or retinal detachment (RD), where spectral-domain optical coherence tomography (OCT) with the Spectralis HRA and OCT device (Heidelberg Engineering, Heidelberg, Germany) was able to help make a correct diagnosis. METHODS: Ten patients (five with acquired RS and five with RD) were referred to a vitreoretinal clinic at Moorfields Eye Hospital for definitive diagnosis and further management. All patients underwent scans with the Spectralis HRA and OCT device. RESULTS: The Spectralis HRA and OCT was able to make a clear diagnosis of RS or RD in all patients. Of the five patients with RS confirmed on OCT, two were referred with RD, and of the five patients with RD, four were referred with RS. New OCT features of RS include the double schisis cavity phenomenon, intraretinal pillars through the schisis cavity, and irregularity of the inner surface of the attached outer leaf. CONCLUSION: Spectral-domain OCT is a useful tool in distinguishing RS from RD, eliminating previous diagnostic uncertainty. The Spectralis HRA and OCT device has the added advantages of being able to reliably image anterior retinal pathology and produce high-definition images.

Topographic variation and interocular symmetry of macular choroidal thickness using enhanced depth imaging optical coherence tomography.

PURPOSE: To report and analyze factors influencing topographical and interocular variations in choroidal thickness (CT) in a healthy adult population. METHODS: One hundred eyes of 50 healthy subjects underwent visual acuity and axial length measurements and optical coherence tomography (OCT) with enhanced depth imaging (EDI). CTs at the fovea and at 3 mm nasal, temporal, superior, and inferior to the fovea were measured manually. Topographic variation, relative interocular differences in CT and predictors of CT were analyzed. The relationships between interocular differences in CT and differences in age and interocular axial length were explored. RESULTS: The mean (SD) foveal CT in the right and left eyes were 334 (95) and 333 (90) µm, respectively. For foveal CT, there was a high correlation between the two eyes (r = 0.90) with a relative interocular 95% limits of agreement of -80 to +83, and a median (range) absolute difference of 21 (0.4-135). There was no significant variation in the relative and absolute interocular differences in CT. Axial length was the main predictor of CT for nasal and foveal CT. Symmetry in CT in the horizontal and vertical meridians was seen in eyes with axial length shorter than 23.50 mm (P < 0.05). CONCLUSIONS: There was no significant relative interocular difference in CT. Axial length contributes to some of the variances in CT but has a significant influence on the CT profile. Although relative interocular difference is not significant, absolute interocular differences in CT may reach 85 µm.

A novel paediatric game-based visual-fields assessor.

AIM: To determine the feasibility of using a computer game to measure visual fields in children. METHODS: The authors describe the development and assessment of a novel computer-game apparatus to measure visual fields in children. It is based upon a computer game visible on a monitor, housed in an interactive model-castle structure. RESULTS: The authors conducted a total of 25 field tests with the final apparatus, in 25 eyes of 19 children, aged 4-14 years. On two occasions children failed to complete the exam, owing to hardware and software defects that were subsequently rectified. For the 23 completed fields, the median time for completion of the full test was 4.5 min. 16 out of 18 clinically normal patients showed normal fields; the only failures were in two 4-year-old children who still managed to complete fields, but with generalised reduced responses that were deemed abnormal by our predetermined criteria. For the five eyes with expected glaucomatous loss, all five visual fields measured were completed and showed abnormal fields consistent with their medical condition. Positive feedback was given from all children about their testing experience. CONCLUSIONS: It is feasible to develop a computer-game-based system to measure fields in children in a non-invasive, affordable and entertaining way.

A rare case of peripartum endogenous bacterial endophthalmitis.

We describe the case of a healthy, pregnant female who developed endogenous endophthalmitis at the time of delivery, and discuss the possible mechanism of infection and the management of this case. A 26-year-old Asian woman presented with a 3-week history of visual deterioration and pain in the right eye. There was no history of ocular trauma or surgery. The ocular symptoms developed one day after vaginal delivery of a healthy baby. The pregnancy had been uncomplicated until premature rupture of membranes one week prior to delivery. Right visual acuity was light perception. There was marked right anterior chamber activity with a hypopyon and fibrin. A B-scan ultrasound showed dense vitritis. Examination of the left eye was normal. Blood tests and a chest X-ray were normal. A vitreous tap was performed and bacterial culture grew Sphingomonas paucimobilis. Intravitreal antibiotics were injected (amikacin 0.4 mg/0.1 ml and vancomycin 2.0 mg/0.1 ml) and the patient was treated with oral moxifloxacin and corticosteroids. Right visual acuity improved to 6/9. This case highlights the need for clinicians to have a high level of awareness of endogenous bacterial endophthalmitis (a rare, potentially sight-threatening condition) in any patient with a painful eye or visual deterioration in the peripartum period, particularly if associated with complications such as premature rupture of membranes or perineal laceration.

Repeatability of manual subfoveal choroidal thickness measurements in healthy subjects using the technique of enhanced depth imaging optical coherence tomography.

PURPOSE: The aim of this study was to investigate the repeatability of manual measurements of choroidal thickness in healthy subjects imaged on spectral domain optical coherence tomography (OCT) using the enhanced depth imaging (EDI) technique. METHODS: Fifty consecutive, healthy, young, adult volunteers with no known eye disease were enrolled prospectively. Two good-quality horizontal and vertical line scans through the fovea were obtained for each eye. Using the manual calipers provided by the software of the proprietary device, two experienced OCT readers measured the subfoveal choroidal thickness (SFCT) of the horizontal and vertical line scans for all eyes. The readers were masked to each other's readings. Intraobserver, interobserver, and intrasession coefficients of repeatability (CRs) were calculated. RESULTS: Mean (standard deviation [SD]) age of the study subjects was 38 (5) years (range, 30-49 years). Mean (SD) subfoveal choroidal thickness was 332 (90) µm (right eyes) and 332 (91) µm (left eyes). Intraobserver CR was approximately 23 (95% confidence interval [CI], 19-26) µm, whereas interobserver and intrasession CRs were greater at 32 (95% CI, 30-34) and 34 (95% CI, 32-36) µm, respectively. There was no significant difference in SFCT between all pairs of SFCT measurements except for the two intrasession vertical line scans. CONCLUSION: A change of >32 µm was likely to exceed interobserver variability in SFCT. Future studies are required to estimate the repeatability of SFCT measurements in patients with chorioretinal pathology.

Choroidal imaging in inherited retinal disease using the technique of enhanced depth imaging optical coherence tomography.

PURPOSE: The aim of this study is to image and describe the in vivo choroidal changes in various retinal dystrophies using the technique of enhanced depth imaging (EDI) optical coherence tomography (OCT) and to correlate these findings with the clinical appearance. Associations between choroidal change and genotype, visual acuity and results of retinal electrophysiology are also explored. DESIGN: Retrospective observational case series. METHODS: Twenty patients attending the medical retina clinics at Moorfields Eye Hospital underwent EDI OCT choroidal scans as part of the scanning protocol when they underwent OCT imaging with the Spectralis HRA and OCT. The choroidal images were obtained by moving the Spectralis camera close enough to obtain an inverted image of the retina. The scans were read by two experienced OCT readers assessing the choroidal thickness as well as the choroidal contour for focal areas of choroidal thinning corresponding to the areas of RPE/outer retinal atrophy. The spectrum of patients included those with Stargardt macular dystrophy, macular dystrophies secondary to known mutations such as peripherin/RDS, uncharacterised macular dystrophies, Best disease, bifocal chorioretinal atrophy, Bietti crystalline retinal dystrophy and choroideraemia. RESULTS: The choroidal appearance was symmetrical in all patients who had both eyes scanned. Ten patients showed no choroidal thinning, five had focal mild to moderate choroidal thinning, three had focal severe choroidal thinning, and two patients had diffuse severe choroidal thinning. There was no association between choroidal thinning and visual acuity [Fisher's exact test, p = 0.350 (right eye), p = 1.000 (left eye)], or extent of retinal dysfunction on electrophysiology (Fisher's exact test, p = 1.000). CONCLUSION: Enhanced depth imaging using spectral domain OCT can be used to identify choroidal changes in inherited retinal disease. The pattern of choroidal change correlates well with the clinical appearance. It appears that the extent and pattern of choroidal thinning is dependent on the stage of the disease in some cases, and in others the causative gene defect.

Vitrectomy in cases of nonaccidental injury.

Nonaccidental head injuries may present in several ways, requiring prompt recognition and management because of the risk of death or permanent neurologic and visual impairment. Dense amblyopia and high anisometropic myopia induced by prolonged occlusion of the macula by hemorrhage is a concern in these infants, although the prognosis also depends on the extent of associated brain injury. There are few reports of the visual and general outcome of children with a diagnosis of nonaccidental injury who have undergone vitrectomy. This report describes the outcome in six eyes of four patients diagnosed as having nonaccidental injury who underwent vitrectomy. The results are more favorable than in previous reports.

Radiation maculopathy complicated by intraretinal neovascularization treated with intravitreal bevacizumab.

PURPOSE: The purpose of this study was to describe a case of radiation maculopathy as a result of intraretinal neovascularization, treated with intravitreal bevacizumab. METHODS: This is an interventional case report. A 51-year-old man was treated with radiotherapy and chemotherapy for nasopharyngeal carcinoma. He developed radiation maculopathy associated with intraretinal neovascularization in the left eye. He underwent a course of 3 intravitreal injections of bevacizumab (1.25 mg/0.05 mL). RESULTS: The visual acuity in the left eye improved with complete closure of the intraretinal neovascularization and no recurrence 17 months after the last bevacizumab injection. CONCLUSION: This case of radiation-induced intraretinal neovascularization responded well to bevacizumab treatment. These lesions may respond more favorably if treated earlier.

Progression of retinopathy during pregnancy in type 1 diabetes mellitus.

PURPOSE: The incidence and risk factors for progression of retinopathy during pregnancy in women with type 1 diabetes mellitus were retrospectively evaluated. METHODS: Fifty-four insulin-dependent diabetic patients at a teaching hospital in Saudi Arabia were followed throughout the pregnancy/puerperium with serial ophthalmic examination. Dilated fundus examination was performed in each trimester and puerperium. RESULTS: Progression of diabetic retinopathy in the study occurred in 13/54 (24%) patients--2/22 (9.1%) patients had no diabetic retinopathy initially, 4/20 (20%) had non-proliferative diabetic retinopathy (NPDR) and 7/12 (58.3%) had proliferative diabetic retinopathy (PDR). Of the eight patients with PDR who had no laser treatment before pregnancy, six (75%) showed progression but only one of the four patients who had PDR and laser treatment prior to pregnancy experienced progression of retinopathy. Eight patients in total received panretinal photocoagulation to arrest the progression of retinal disease during pregnancy and only one of them had laser treatment prior to pregnancy. CONCLUSION: Laser photocoagulation for severe NPDR or early PDR prior to pregnancy may protect against rapid progression of PDR. Visual impairment resulting from progression of PDR can be prevented by aggressive laser treatment during pregnancy. Duration of diabetes>15 years, poor glycaemic control and hypertension are high-risk factors in the progression of diabetic retinopathy in pregnancy.

Giant macular hole in Alport syndrome.

CASE REPORT: A 29-year-old woman with Alport syndrome presented with unilateral visual deterioration over an 8-year period. She had the rare ocular complication of a giant macular hole. She developed a small macular hole in the other eye. COMMENTS: The ocular features of Alport syndrome are described, including proposed mechanisms for hole formation.

An unusual case of keratoconus.

We report a rare case of severe keratoconus and acute corneal hydrops in a 6-year-old girl. We describe the management of this challenging case and the outcome.

Oral gabapentin treatment for symptomatic Heimann-Bielschowsky phenomenon.

PURPOSE: To describe a patient with symptomatic Heimann-Bielschowsky phenomenon (HBP) treated successfully with oral gabapentin. DESIGN: Interventional case report. METHODS: A 57-year-old patient with retinitis pigmentosa had a 12-month history of vertical oscillopsia. The right visual acuity was 20/200 and the left visual acuity was 20/60 with glasses. He had a coarse, slow, pendular, vertical movement of the right eye consistent with Heimann-Bielschowsky phenomenon. His treatment commenced with oral gabapentin with gradual increase in dose to 2400 mg/d. Eye movement recordings were performed before and after treatment. RESULTS: He became considerably less symptomatic on gabapentin. The right visual acuity improved to 20/80 with glasses. Eye movement recordings confirmed markedly reduced vertical oscillations of the right eye. The patient continues on gabapentin with no side effects and sustained effect. CONCLUSIONS: The vertical nystagmoid movement in HBP can be considerably reduced and symptoms remarkably improved when treated with oral gabapentin.

Giant cell (temporal) arteritis: an overview and update.

Giant cell arteritis is a systemic disease that continues to be a sight-threatening medical emergency requiring prompt recognition and treatment in order to avoid devastating ophthalmic consequences. Although there have been advances in the genetic and immunologic understanding of the underlying pathogenesis of the disease, the exact etiology of the condition, to date, remains unclear. Visual manifestations of giant cell arteritis are the common mode of presentation, making the ophthalmologist critically responsible for early diagnosis and treatment. Although temporal artery biopsy remains the only confirmatory procedure, newer laboratory investigations and blood flow studies with fundus fluorescein angiography have aided in the diagnosis of temporal giant cell arteritis. Maintenance of a high index of clinical suspicion is essential to institute prompt adequate treatment, especially in atypical cases. Corticosteroids remain the mainstay of treatment of giant cell arteritis. Recently, immunosuppressive agents as secondary steroid-sparing drugs have been used, particularly in some steroid-resistant cases. A wider recognition of the disease will minimize the prevalence of irreversible visual loss among patients with giant cell arteritis.