Retinal pigment epithelium–photoreceptor layer alterations in a patient with Sturge–Weber syndrome with diffuse choroidal hemangioma

Purpose: To describe an 8-year-old girl with diagnosis of Sturge–Weber syndrome (SWS) presenting with glaucoma, abnormal iris vessels, diffuse choroidal hemangioma, and small white dot-shaped “microdrusen-like” alterations of the retina in the right eye.

Patient and methods: Complete ophthalmological examination was performed with slit-lamp anterior segment assessment and fundus ophthalmoscopy. Near infrared reflectance (NIR) and enhanced depth imaging (EDI) spectral domain optical coherence tomography (SD-OCT) were carried out.

Results: Microdrusen-like retinal alterations observed with fundus ophthalmoscopy appeared as multiple hyperreflective dots surrounded by a hyporeflective ring on NIR. EDI SD-OCT showed increased choroidal thickness exceeding 1000 μm. B-scan cross-sectional examination on the hyperreflective dots revealed focal alterations of the retinal pigment epithelial (RPE)–photoreceptor layer.

Conclusions: The increase of the choroidal thickness due to the diffuse choroidal hemangioma caused alterations of the RPE–photoreceptor layer similar to reticular pseudodrusen or pachychoroid pigment epitheliopathy.     

Comparison of Enhanced Depth Imaging and Swept Source Optical Coherence Tomography in Assessment of Choroidal Thickness in Vogt–Koyanagi–Harada Disease

Purpose: To compare enhanced depth imaging (EDI-OCT) and swept source optical coherence tomography (SS-OCT) in assessment of Vogt–Koyanagi–Harada (VKH) disease.

Methods: All consecutive VKH patients seen at Singapore National Eye Centre during 2012–2013 were imaged using both modalities. Subfoveal choroidal thickness (SFCT) was measured by one masked trained observer.

Results: A total of 137 pairs of scans were obtained from 48 patients. SFCT was more likely to be measurable on SS-OCT than EDI-OCT (112, 81.8%; 84, 61.3%; p<0.001 Fisher’s Exact test). There was good inter-OCT correlation of SFCT when both scans were measureable (mean of the difference in SFCT ± 2 standard deviations (SD) of –14.5 ± 21.0 μm).

Conclusions: SS-OCT images are superior to EDI-OCT but the SFCT measurements are comparable when both are readable.     

Choroidal Neovascularization Associated with Multiple Evanescent White Dot Syndrome Treated with Intravitreal Ranibizumab

Purpose: To describe the clinical outcomes of intravitreal ranibizumab treatment for subfoveal choroidal neovascularization (CNV) associated with multiple evanescent white dot syndrome (MEWDS).

Methods: This is a prospective, interventional, case series. All recruited patients underwent a baseline intravitreal ranibizumab injection and were monitored monthly over a 12-month follow-up, following a pro-re-nata regimen.

Results: Four patients (four eyes) were included in the study. Mean best-corrected visual acuity (BCVA) changed from 0.60 ± 0.20 at baseline to 0.07 ± 0.05 logMAR at 12-month examination. Baseline central macular thickness reduced from 330 ± 32 µm to the final value of 228 ± 14 µm at the 1-year follow-up. Overall, a mean number of 2.2 ranibizumab injections were administered at the end of 12 months.

Conclusions: Intravitreal ranibizumab treatment represents a valuable therapeutic option for the management of CNV associated with MEWDS.     

Spectral-domain Optical Coherence Tomography Patterns in Intraocular Lymphoma

Purpose: To examine spectral-domain optical coherence tomography (SD-OCT) patterns in intraocular lymphoma (IOL).

Methods: Records of 13 patients (21 eyes) with IOL were retrospectively reviewed. SD-OCT was evaluated at initial visit and during follow-up.

Results: SD-OCT images at initial visit demonstrated disruption of the ellipsoid zone (8 eyes, 38.1%) and hyperreflective nodules at the retinal pigment epithelium (RPE) level (5 eyes, 23.8%). During follow-up, disruption of the ellipsoid zone (10 eyes, 47.6%) and hyperreflective nodules at the RPE level (7 eyes, 33.3%) were noted. In 5 eyes showing hyperreflective nodules at the RPE level, the hyperreflective nodules were reduced after treatment with intravitreal methotrexate.

Conclusions: Hyperreflective nodules in the outer retina and disruption of the ellipsoid zone were observed in nearly one-half of patients with IOL over time. SD-OCT may allow for early detection of small macular abnormalities and aid in monitoring of treatment efficacy in this disease.     

Choroidal Structural Changes in Tubercular Multifocal Serpiginoid Choroiditis

Purpose: To assess choroidal vascular changes among patients with tubercular multifocal serpiginoid choroiditis (TB MSC) using previously validated techniques.

Methods: Patients with TB MSC (n = 18) and healthy controls (n = 30) underwent enhanced-depth imaging optical coherence tomography (EDI-OCT) imaging. Using previously validated algorithm of image binarization, EDI-OCT scans were segmented to derive total choroidal area, luminal area, stromal area, and choroidal vascularity index (CVI).

Results: There was a statistically significant difference in the CVI between controls (66.90 ± 1.77%) and TB MSC patients (65.46 ± 2.53%; p < 0.001). There was significant reduction in CVI at follow-up (3 months) (63.77 ± 3.91%; p = 0.05). The choroidal thickness was higher in TB MSC compared to controls (278.90 ± 57.84 µm versus 329.33 ± 27.69 µm; p = 0.001).

Conclusions: CVI provides insight into structural changes in choroid in TB MSC. During the active disease, there is relative decrease in choroidal vascularity. As the lesions heal, choriocapillaris atrophy occurs with remodeling of choroid.     

Choroidal Thickness Changes in Rheumatoid Arthritis and the Effects of Short-term Hydroxychloroquine Treatment

Purpose: The objective of this study is to evaluate choroidal thickness (CT) in patients with rheumatoid artritis (RA) and the effects of short-term hydroxychloroquine (HCQ) treatment.

Methods: Thirty RA patients (group 1) and 30 normal subjects (group 2) were enrolled in the study. Group 1 was further divided into two subgroups as follows: group 1A (Before HCQ treatment) and group 1B (1 year after HCQ treatment). The CTs were measured using optical coherence tomography (EDI-OCT).

Results: The mean subfoveal choroidal thickness (SFCT) (µm) was 335.70 ± 64.58 in group 1A, 341.85 ± 63.95 in group 1B, and 358.51 ± 63.83 in group 2. SFCT was significantly lower in groups 1A and 1B than in group 2 (p = 0.020 and p = 0.028, respectively). Group 1B presented statistically significant thicker SFCT than group 1A (p = 0.033).

Conclusions: CT was significantly lower in RA patients than in normal subjects. HCQ treatment may, however, cause a statistically significant increase in SFCT. This increase is clinically negligible.     

Posterior Scleritis with Choroidal Effusion Secondary to Herpes Zoster Ophthalmicus

Purpose: To describe a case of posterior scleritis with serous choroidal detachment that occurred as an acute complication of herpes zoster ophthalmicus (HZO).

Methods: Retrospective case report.

Results: A 75-year-old female presented with 3 days of painful, decreased vision in her left eye 1 week after being diagnosed with HZO. She had unilateral crusted vesicular lesions in the V1 dermatomal distribution and corneal pseudodendrites. Funduscopic examination demonstrated a large choroidal detachment in her left eye. B-scan ultrasonography revealed unilaterally thickened sclera consistent with posterior scleritis. She was treated with oral prednisone and a 2-week course of intravenous acyclovir. Two weeks after the initiation of treatment, her vision had improved and she demonstrated complete resolution of her pseudodendrites, posterior scleritis, and choroidal detachment.

Conclusions: Prompt recognition and treatment of this unique combination of clinical manifestations of HZO resulted in significant improvement in vision and resolution of the scleritis and choroidal detachment.     

Juvenile Xanthogranuloma–A Rare Cause of Unilateral Orbital Swelling in an Elderly Patient

Objective: To report the clinical and pathologic features of an elderly patient with a unilateral orbital swelling and proptosis caused by Juvenile Xanthogranuloma diagnosed and confirmed by orbital biopsy.

Design: Interventional case report.

Participants: One patient.

Intervention: Steroids (Medrol dose pack) and radiation.

Main Outcome Measures: Unusual clinical presentation and pathological features of Juvenile Xanthogranuloma in the orbit.

Conclusions: Juvenile Xanthogranuloma affecting one orbit is very rare with unilateral involvement in an elderly patient. Steroids and radiation therapy were very effective in treatment and provided impressive results.     

Use of Aqueous Humor and Flow Cytometry in Ocular Sarcoidosis Diagnosis

Purpose: To report the use of flow cytometry on aqueous fluid to diagnose sarcoidosis in a patient with recurrent granulomatous anterior uveitis.

Methods: Case report.

Results: Flow cytometry on aqueous fluid demonstrated a CD4/CD8 ratio >9.5, consistent with a diagnosis of sarcoidosis.

Conclusions: Flow cytometry on aqueous fluid may offer an additional pathway for diagnosing sarcoid anterior uveitis.     

Leuconostoc as a Cause of Endophthalmitis Post-intravitreal Injection of Ranibizumab

Purpose: The authors report a case of endophthalmitis after intravitreal injection of ranibizumab.

Methods: After searching for extensive laboratory tests to isolate the etiologic agent

Results: The agent was determined as Leuconostoc mesenteroides, gram-positive cocci, vancomycin resistant.

Discussion: Considerations regarding this bacterium were done by calling attention to its rarity, difficulty of isolation, and action on secondary comorbidities as opportunistic pathogen.     

Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules

Purpose: To characterize nodular lesions of the retina and optic nerve with spectral-domain optical coherence tomography (SD-OCT) in patients with sarcoidosis.

Methods: This is a retrospective series of 6 eyes from 5 patients with an established diagnosis of sarcoidosis, with clinically detected nodules of the optic nerve or retina. All lesions were imaged with fundus photography and SD-OCT on presentation, and followed with serial imaging after treatment with corticosteroids and/or immunomodulatory therapy.

Results: Spectral OCT through the lesions revealed nodular hyperreflective processes obscuring the retinal layers or optic cup, with local structural changes, including subretinal and intraretinal fluid. After treatment with corticosteroids and/or immunosuppression in 4 followed patients, all lesions regressed with improvement in associated structural changes, but did not entirely disappear.

Conclusions: Spectral OCT can be useful in identifying lesion morphology and location, and in tracking the response to treatment in eyes with posterior-segment nodules, presumably secondary to sarcoidosis.     

Giant Orbital Hydrocystoma in Children: Case Series and Review of the Literature

Objective: To describe the clinical features, ancillary diagnostic studies, and treatment outcomes in a cohort of pediatric patients with giant orbital hydrocystomas.

Design: Retrospective case series.

Participants: Pediatric patients with giant orbital hydrocystomas treated in the practice of one surgeon (PDL).

Methods: A retrospective review of the clinical charts of pediatric patients with orbital hydrocystoma was performed and diagnostic information collected. Results were reviewed and compared with reported clinical data in the literature.

Main Outcome Measures: Clinical presentation and histopathological findings of pediatric orbital hydrocystomas.

Results: Three pediatric cases of giant orbital hydrocystoma were encountered, each with an unusual feature, including deep orbital location, occurrence following trauma, and eccrine pathology.

Conclusion: Giant orbital hydrocystomas may present in the pediatric population. Ophthalmologists should be cognizant of this entity when evaluating a child with a large, cystic orbital mass.     

In Vivo Confocal Microscopic Characteristics of Crystalline Keratopathy in Patients with Sclerokeratitis

Purpose: To report in vivo confocal features in a clinical case series of patients with sclerokeratitis presenting as crystalline keratopathy.

Methods: Five cases of crystalline keratopathy following sclerokeratitis are described. Confocal microscopic images of the cornea were captured in all cases to confirm the diagnosis by evaluating the morphology of the crystals.

Results: Unilateral and non-progressive peripheral crystalline keratopathy manifested after previous episodes of sclerokeratitis in the involved eye. Confocal microscopy revealed numerous, discrete, hyperreflective, needle-like, shiny crystals in the anterior and posterior stromal layers of the cornea. These deposits were oriented randomly and showed occasional confluence. An extensive ophthalmic and systemic evaluation did not reveal any other contributory factors.

Conclusion: Crystalline keratopathy, probably resulting from an immune-mediated response, is a possible manifestation of sclerokeratitis. This should be considered during long-term follow-up of such patients and differentiated from infectious crystalline keratopathy.     

Varicella Zoster-related Occlusive Retinal Vasculopathy—A Rare Presentation

Purpose: To report a case of occlusive retinal vasculopathy following varicella zoster infection in an immunocompetent adult.

Design: Observational case report.

Methods: A patient with defective vision following chickenpox was evaluated with fluorescein angiography, spectral domain optical coherence tomography and fundus auto fluorescence.

Results: Fundus showed multiple cotton wool spots and a well-demarcated zone of retinal ischemia in the posterior pole with normal optic disc without any evidence of anterior or posterior uveitis. Fluorescein angiography, spectral domain optical coherence tomography and fundus auto fluorescence findings revealed occlusive vasculopathy as the cause of defective vision.

Conclusions: We report a hitherto undescribed case of purely occlusive vasculopathy following varicella zoster infection without features of vasculitis or anterior and posterior uveitis in an immunocompetent individual.     

Evaluation of Macular Ganglion Cell-inner Plexiform Layer and Choroid in Psoriasis Patients Using Enhanced Depth Imaging Spectral Domain Optical Coherence Tomography

Purpose: To evaluate changes in the thickness of the central macula, macular ganglion cell-inner plexiform layer (mGCIPL), and subfoveal choroid in patients with psoriasis using spectral domain optical coherence tomography (SD-OCT).

Methods: The measurements of macular, mGCIPL thicknesses and subfoveal choroidal thickness (SFCT) obtained by SD-OCT of psoriasis patients (n = 46). These measurements were compared with those of 50 healthy controls.

Results: The macular, mGCIPL, and choroidal thicknesses did not differ between the controls and psoriatic subjects (p>0.05). When the patients were divided into two distinct groups, only the SFCT was significantly thicker in the severe psoriasis group compared with the mild psoriasis group (p = 0.003).

Conclusions: These findings suggest that choroidal alterations are seen without macular changes in patients with psoriasis. Severe psoriasis appears to be related to increases in SFCT as a consequence of possible inflammatory cascades that are part of the disease’s pathogenesis.     

Multiple Evanescent White Dot Syndrome Following Intradermal Influenza Vaccination

Purpose: To present a case report of MEWDS following an intradermal influenza vaccination.

Methods: Review of the clinical, laboratory, photographic, and angiographic records of a patient with MEWDS.

Results: 27-year-old female that presented to our clinic with photopsias OD. These symptoms began 2 weeks following an influenza intradermal vaccination.

Fundus examination and diagnostic testing were consistent with MEWDS.

Conclusion: While rare, MEWDS can occur following influenza vaccination.     

Retinal capillaritis in a CRB1-associated retinal dystrophy

Purpose: To report a case of CRB1-associated retinal dystrophy characterized by vitritis, retinal capillaritis, and cystoid macular edema (CME).

Methods: A case report.

Results: An 8-year-old boy was diagnosed with intermediate uveitis and treated with corticosteroids. He was subsequently diagnosed with retinal dystrophy and found to have two CRB1 mutations.

Conclusions: Retinal capillaritis, vitritis, and CME could be inflammatory features of CRB1 retinal dystrophy in our young patient.     

Use of Intravitreal Bevacizumab in a 9-Year-Old Child with Choroidal Neovascularization Associated with Autosomal Recessive Bestrophinopathy

Introduction: Bestrophinopathies result from mutations within the BEST1 gene; although multiple gene mutations have been identified, the recessive form is often the form which gives rise to the rarer complication of choroidal neovascularization. We describe a child with treated choroidal neovascularization secondary to Best disease with a newly identified genetic mutation.

Methods: Case report.

Results: A 9-year-old child reported unilateral blurred vision; the acuity deteriorated over the following months to 3/18 due to the development of a choroidal neovascular membrane. She was treated with three injections of bevacizumab with recovery to 6/12 vision and no subsequent recurrence over the follow-up period of 2 years, and no secondary complications from the drug. Genetic analysis revealed a novel heterozygous mutation in the BEST1 gene, with no evidence of disease in the family.

Conclusions: We describe a novel mutation within the BEST1 gene of the heterozygous form giving rise to vitelliform lesions and secondary neovascularization successfully treated in a child with a course of bevacizumab. The genetic testing has implications on genetic counseling in such patients and the genetic analysis of all such patients ought to be routinely considered.     

Intraocular Lymphoma Masquerading as Recurrent Iridocyclitis: Findings Based on in vivo Confocal Microscopy

Purpose: To emphasize the application prospects of in vivo confocal microscopy (IVCM) in distinguishing intraocular lesions from inflammatory and neoplastic diseases.

Methods: Retrospective case report.

Results: A patient with neoplastic masquerade syndrome initially underwent IVCM examination. After six separate intravitreal injections of 400 mg/0.1 ml methotrexate, IVCM revealed a complete remission of intraocular lymphoma.

Conclusions: Although IVCM findings alone are not enough to diagnose intraocular neoplasm with absolute certainty, they can provide useful indication for distinguishing between intraocular inflammatory diseases and neoplasms.     

Dengue Fever Presenting as Purtscher-like Retinopathy

Purpose: To report the fundus manifestations and spectral-domain optical coherence tomographic (SD-OCT) features of dengue fever presenting as Purtscher-like retinopathy.

Methods: Retrospective review of two cases of dengue fever.

Results: Color fundus photograph revealed the presence of cotton-wool spots in a Purtscher-like configuration in the posterior pole of all study eyes. SD-OCT demonstrated increased reflectivity signal in the inner retinal layers, and after a variable follow-up period, there was complete disappearance of cotton-wool spots and persistence of the hyperreflectivity signal.

Conclusion: We report two unique cases of dengue fever associated with retinal lesions in a configuration of Purtscher-like retinopathy.