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.     

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.     

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.     

The Effect of Closed-Eye Tear Film Conditions on Blood-Isolated Neutrophils,In Vitro

Purpose: Eyelid closure results in influx of neutrophils onto the ocular surface, which are non-responsive to inflammatory stimuli. This investigation examined whether incubation of blood-isolated neutrophils in closed-eye conditions induce a tear-film neutrophil phenotype.

Methods: Blood-isolated neutrophils were incubated combining various conditions: hypoxia, corneal epithelial cells (HCEC), artificial tear solution (ATS).

Results: A hypoxic environment induced no differential effect on membrane receptor expression. Incubation in the presence of HCEC resulted in membrane receptor upregulation and increase in caspase activation.

Conclusions: Hypoxia, corneal epithelial cell exposure, or artificial tear fluid are insufficient to replicate a tear-film neutrophil phenotype using blood-isolated neutrophils.     

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.     

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.     

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.     

Assessment of Corneal and Tear Film Parameters in Rheumatoid Arthritis Patients Using Anterior Segment Spectral Domain Optical Coherence Tomography

Purpose: To study the corneal changes in rheumatoid arthritis (RA) patients in vivo, using spectral domain anterior segment optical coherence tomography (AS-OCT).

Methods: A case–control study was done on 43 RA patients and 40 controls. The disease activity score (DAS28-ESR) was calculated and all participants had lower tear meniscus, corneal thickness, and epithelial thickness evaluation using AS-OCT.

Results: The lower tear meniscus height (LTMH) and the lower tear meniscus area (LTMA) were significantly lower in the RA patients than in controls (p < 0.001). RA patients also had a significantly thinner central corneal thickness (p = 0.02) and their epithelium was found to be thinner in the superotemporal peripheral sector.

Conclusions: The LTMH and LTMA are significantly reduced in RA patients, despite the absence of clinical diagnosis of dry eye. RA patients have thinner corneal thickness and epithelial thickness than controls, which did not correlate with either disease duration or activity.     

Assessment of Central Corneal Thickness in Different Subtypes of Systemic Sclerosis

Purpose: To compare central corneal thickness between patients with diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis.

Methods: A total of 53 consecutive patients with systemic sclerosis were enrolled in this study. All subjects were screened for age, gender, classification of disease subtype, autoantibody profile, duration of disease, organ involvement, current treatment, tear break-up time, Schirmer test, and measurement of the central corneal thickness with an ultrasonic pachymeter.

Results: No statistically significant differences were found in terms of age, sex, and duration of the disease, and dry eye evaluation between systemic sclerosis subsets. The mean central corneal thickness was 535.3 ± 37.6 µm (range, 471–619 µm) in diffuse cutaneous systemic sclerosis patients, and 539.3 ± 37.1 µm (range, 484–651 µm) in limited cutaneous systemic sclerosis (p = 0.71).

Conclusions: Central corneal thickness in patients with diffuse cutaneous systemic sclerosis is not different from the one in limited cutaneous systemic sclerosis.     

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.     

Enucleation Refusal for Retinoblastoma: A Global Study

Background: Enucleation (eye removal) is often the only curative treatment for the childhood eye cancer retinoblastoma, yet parental refusal of enucleation commonly contributes to treatment delay and poor survival globally.

Methods: Physicians who treat retinoblastoma were surveyed to glean underlying reasons for treatment refusal.

Results: Refusal rates were higher when less time was spent with parents explaining retinoblastoma/enucleation, and where fewer support services were available. Reasons for refusal included parental belief in alternative treatments, culture, and social stigma.

Conclusions: We suggest strategies to increase parental compliance with enucleation and save the lives of children with retinoblastoma.     

Macular hole in Stargardt disease: Clinical and ultra-structural observation

Purpose: To report for the first time a case report of a Stargardt disease patient who developed a macular hole (MH) which was treated with a surgical approach.

Method: Case report.

Results: After vitrectomy a complete closure of the MH was obtained and the best-corrected visual acuity remained stable. No complications were reported after one year of follow-up. An ultra-structural examination of the removed internal limiting membrane (ILM) and epiretinal membrane (ERM) was carried out under electron microscopy.

Conclusion: Vitreo-retinal surgery could represent a therapeutic option to treat vitreo-retinal interface abnormalities associated with inherited retinal diseases.     

Rituximab and Acute Retinal Necrosis in a Patient with Scleromalacia and Rheumatoid Arthritis

Background: Rituximab is a widely used biologic agent, which has shown favourable results in the treatment of vasculitis. But immunosuppressive treatment also bears the risk of severe complications.

Methods: A patient with rheumatoid arthritis, progressive scleromalacia, and acute retinal necrosis on therapy with rituximab is reported.

Results: For the first time, a correlation between rituximab and acute retinal necrosis in a patient with progressive rheumatoid scleromalacia is shown.

Conclusions: Although rituximab is a promising biologic agent for the treatment of autoimmune diseases, it bears the risk of reactivation of viral infections, including the onset of acute retinal necrosis.     

Effect of Systemically Used Anti-Tumor Necrosis Factor-α Medication on the Corneal Epithelium and Stroma of Patients with Ankylosing Spondylitis

Purpose: To evaluate the effect of systemically used anti-tumor necrosis factor alpha (TNF-α) medication on the thickness of corneal epithelium and stroma in patients with ankylosing spondylitis (AS).

Methods: A total of 125 eyes of 69 participants were included in this retrospective study of three groups: healthy participants (Group 1), AS patients receiving anti–TNF-α medication (Group 2), and AS patients receiving a nonsteroidal anti-inflammatory medication (Group 3).

Results: According to anterior segment optical coherence tomography, the mean thickness of the corneal epithelium was significantly thicker in Group 2 than in Group 3 (51.6 ± 3.2 µm versus 50.4 ± 3 µm, p = 0.01), as was that of the stroma (475 ± 33 µm versus 443 ± 29 µm, p = 0.002).

Conclusions: Anti–TNF-α medication and/or avoidance of nonsteroidal anti-inflammatory drugs could improve the thickness of both the corneal epithelium and stroma in AS patients.     

Boston Type I Keratoprosthesis Implantation Following Autologous Submandibular Gland Transplantation for End Stage Ocular Surface Disorders

Purpose: To describe the results of Boston keratoprosthesis implantation in a patient with Stevens–Johnson syndrome following autologous submandibular gland transplantation.

Design: Observational case report.

Methods: We report the case of a man diagnosed with Stevens–Johnson syndrome. Autologous submandibular gland transplantation was performed in the right eye in 2008 to ameliorate tear film deficiency. Due to the improvement in the microenvironment of the ocular surface, Boston keratoprosthesis implantation was performed in the right eye in 2011.

Results: He maintained a VA of 20/100 for 12 months. At the last follow-up visit (54 months), his VA had declined to finger count level due to de novo glaucoma, which was treated with glaucoma drainage device implantation, cyclophotocoagulation and topical anti-glaucoma medications.

Conclusions: The procedures described provide patients with end-stage ocular surface disease an option to lessen the severity of keratoconjunctivitis sicca and to regain their VA.     

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.     

Stroke Due to Cerebral Vasculitis in a Patient with Relentless Placoid Chorioretinitis

Purpose: To report the first case of stroke in a patient with relentless placoid chorioretinitis.

Methods: Observational case report.

Results: A 20-year-old female with newly diagnosed relentless placoid chorioretinitis was urgently evaluated for unilateral paresthesias. She was found to have acute bilateral pontine strokes and cerebral vasculitis on magnetic resonance imaging of the brain and cerebral angiography.

Conclusions: We report the first case of stroke due to cerebral vasculitis in a patient with relentless placoid chorioretinitis. This case emphasizes the need for timely evaluation of neurological symptoms in patients with this ocular diagnosis.     

Photoreceptor Cell Injury Starts in the Initial Stage of Vogt–Koyanagi–Harada Disease

Purpose: To demonstrate photoreceptor cell injury in Vogt-Koyanagi-Harada (VKH) disease.

Methods: The SD-OCT and FAF tests of 16 patients diagnosed with VKH disease that was followed up from the initial stage to the convalescent stage were reviewed.

Results: In the initial stage of VKH disease, there was hyperautofluerescence around the macular edema. In the convalescent stage of VKH disease, loss of photoreceptor and injury of RPE have been detected by OCT scan at the same area, which was corresponded with the abnormalities in FAF.

Conclusions: The VKH disease causes photoreceptor injury starting in the initial stage of the disease.     

Ocular findings in a patient with Cockayne syndrome with two mutations in the ERCC6 gene

Background: Cockayne syndrome is a rare, autosomal recessive, multisystem disorder that causes a senile appearance. Ophthalmic abnormalities are frequently present. Here, we report a wide range of ocular findings in a child with Cockayne syndrome.

Materials and methods: The systemic and ocular findings were reviewed. A mutation analysis was performed in the patient and her parents.

Results: The patient underwent a complete ocular examination. Both eyes had low visual acuity, corneal epithelial degeneration, punctate opacities of the lens, and retina disorders. The systemic findings included growth deficiency and a senile appearance. Gene analysis showed mutations in exons 4 and 18 of the ERCC6 gene.

Conclusion: Multiple ocular abnormalities were observed in a patient with Cockayne syndrome. A detailed ophthalmic evaluation of children with Cockayne syndrome is advised.     

Radiofrequency surgery: 3500 years old and still young

Purpose: To review the history of Radiofrequency surgery, delineate the actual situation and describe the applications in eyelid surgery.

Design: Review.

Methods: Review of literature, personal communication with several pioneers in the field, and own experience.

Conclusion: Radiofrequency surgery has evolved from rude burning to a sophisticated surgical technique.