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.     

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.     

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.     

Intravitreal Adalimumab in Active Noninfectious Uveitis: A Pilot Study

Purpose: To evaluate the short-term efficacy of intravitreal adalimumab (IVA) for the treatment of eyes with active noninfectious uveitis.

Methods: Consecutive eyes with active noninfectious uveitis were injected with IVA at 0, 2, then every 4 weeks for total of 26 weeks.

Results: Six out of 7 patients (12 of 13 eyes) completed 26 weeks of treatment. One patient (1 eye) failed treatment. Seven out of 12 eyes had improvement of ≥2 ETDRS lines. Three out of three eyes had resolution of anterior chamber cells. And 9 of 10 eyes with vitreous haze had zero haze at 26 weeks. Five out of 8 eyes with macular edema had complete resolution. Median fluorescein angiography score improved from 14 to 4 on last follow-up.

Conclusions: IVA was effective in controlling the inflammation, decreasing the macular edema, and improving the best corrected visual acuity in the majority of eyes in this series.     

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.     

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.     

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.     

A Hemophagocytic Lymphohistiocytosis Patient that Presented with Unilateral Panuveitis

Purpose: To describe a case of hemophagocytic lymphohistiocytosis (HLH) with ocular changes prior to the systemic changes.

Methods: A 53-year-old man presented with the chief complaint of decreased vision in his right eye. The patient was examined by ocular examination, slit lamp examination, optical coherence tomography, laboratory examination, abdominal ultrasound, and bone marrow biopsy.

Results: Ocular examination revealed uveitis OD and optical coherence tomography revealed macular edema OD. Laboratory examination demonstrated cytopenia in two cell lines, hypofibrinogenemia, and elevated serum ferritin. Abdominal ultrasound findings indicated hepatosplenomegaly. The bone marrow biopsy specimen demonstrated histiocytes and significant hemophagocytosis, leading to a diagnosis of HLH.

Conclusion: Ophthalmic manifestation can be the first sign of HLH and progress to fatal systemic changes.     

Chikungunya Virus Infection Manifesting as Intermediate Uveitis

Purpose: To describe a case of intermediate uveitis caused by chikungunya virus infection in the Western Hemisphere.

Methods: Case report of a patient diagnosed with chikungunya infection presenting with blurry vision and floaters.

Results: Exam revealed a unilateral intermediate uveitis, with an extensive work-up positive for chikungunya virus immunoglobulin M and G titers. The patient responded to oral corticosteroids with signs and symptoms resolving over the course of 3 months’ treatment.

Conclusions: While anterior uveitis and retinitis are the most common ocular manifestations of chikungunya infection, we report here a case of chikungunya infection presenting as an intermediate uveitis, responding well to oral corticosteroids. This case demonstrates the varied presentation of chikungunya-related uveitis and highlights its consideration in the differential diagnoses of those who have had preceding systemic viral symptoms and uveitis.     

Surgical Treatment of Subretinal Fibrosis Caused Macular Detachment in Vogt-Koyanagi-Harada Disease: A Pioneer Study

Purpose: To describe surgical outcomes of macular detachment caused by subretinal fibrosis (SRF) in Vogt-Koyanagi-Harada disease (VKH).

Methods: Retrospective review of VKH patients who underwent SRF removal surgery.

Results: Seven eyes of six VKH patients with preoperative BCVA ranging from light perception to 20/250 were included. Six eyes underwent uncomplicated SRF removal with C3F8 or silicone oil (SO) tamponade and the following optional primary or subsequent procedures: intravitreal injection of triamcinolone acetonide, SO removal, lensectomy, or phacoemulsification with intraocular lens (IOL) implantation. All six eyes had attached macula and improved BCVA at the last visit (ranging from 20/2000 to 20/67) compared to baseline; the other eye, however, showed no light perception after surgery due to optic nerve injury.

Conclusions: In VKH patients, macular detachment caused by SRF can be treated with surgery with generally favorable outcomes. Extreme caution should be taken to avoid optic nerve injury.     

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.     

Short-term Intensive Immunosuppression: A Randomized,Three-arm Study of Intravenous Pulse Methylprednisolone and Cyclophosphamide in Macular Serpiginous Choroiditis

Purpose: To compare the efficacy of pulse cyclophosphamide with pulse dexamethasone in acute macular serpiginous choroiditis (SC).

Methods: A total of 30 patients with macular SC were prospectively randomized into three treatment groups: group D (pulse dexamethasone); group C (pulse cyclophosphamide); and combination (pulse group DCP) administered for 3 days. Macular SC was defined as any active lesion involving/threatening macula.

Results: A total of 30 patients were enrolled, with 10 patients in each group. Lesions completely healed at median duration of 2 weeks in each group, with significant improvement in visual acuity compared with pretreatment levels (p<0.05). Pulse cyclophosphamide was most effective in faster healing of lesions compared with other groups. There was no difference in gain in visual acuity between any of the groups (p = 0.32).

Conclusions: Cyclophosphamide may be an effective treatment modality for acute macular SC, though it may not have a long-term effect on disease relapse.     

Topical carbonic anhydrase inhibitors in macular edema associated with Alström syndrome

Background: Alström syndrome is a rare genetic ciliopathy caused by a mutation in the ALMS1 gene. The syndrome is characterized by cone-rod dystrophy, dilated myocardiopathy, childhood obesity and sensorineural hearing loss. To date, cystoid macular edema has not been reported.

Methods: A female affected by Alström syndrome developed bilateral cystoid macular edema evidenced by optical coherence tomography. A topical carbonic anhydrase inhibitor was prescribed.

Results: Complete resolution of the cystoid macular edema was achieved, though visual acuity did not improve.

Conclusions: Topical carbonic anhydrase inhibitors may have a role in the treatment of macular edema in syndromic retinal dystrophies such as Alström syndrome.     

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.     

Tocilizumab in Uveitic Macular Edema Refractory to Previous Immunomodulatory Treatment

Purpose: To analyze the efficacy of tocilizumab in uveitic macular edema (ME) resistant to various immunomodulatory drugs.

Methods: Patients received tocilizumab every 4 weeks intravenously. Central foveal thickness (CFT) was assessed by optical coherence tomography (OCT).

Results: Five patients (8 eyes) who were ineffectively pretreated with systemic prednisolone, at least one immunosuppressive drug, and at least one biologic drug for uveitic macular edema were included in the study. At 3 months, a response of ME (≥25% reduction in CFT) was observed in 6 eyes (75.0%) of 5 patients. During follow-up, complete resolution of ME was achieved in 5 eyes (62.5%) of 4 patients. Improvement of BCVA was observed in 3 eyes of 3 patients, and stabilization in 3 eyes of 3 patients. Tocilizumab was well tolerated, and no severe side effects occurred.

Conclusions: Treatment with tocilizumab can be considered in chronic uveitic macular edema even if previous immunomodulatory therapy has failed.     

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.     

Impact of Superior Oblique Transposition on Primary Position Deviation,a Pattern and Intorsion in Third Nerve Palsy

Purpose: To evaluate the role of superior oblique transposition on primary position alignment, A pattern, and intorsion in third nerve palsy.

Methods: Ten patients with isolated, unilateral third nerve palsy were included in this prospective study. The patients were treated by conventional surgery on horizontal recti together with superior oblique transposition by Scott’s procedure in the paretic eye.

Results: Pre-operative primary horizontal deviation was 60-80 PD (mean 70.00±7.45 PD). Pre-operative primary hypotropia was 15-22 PD (mean 18.80±2.48 PD). Mean A-pattern was 17.80±2.65 PD. All patients included in the study had some degree of objective torsion as measured by Guyton’s method. One patient had grade I objective intorsion, 2 had grade II, 6 had grade III, and 1 had grade IV objective intorsion.

Primary position horizontal alignment (up to ±8 PD) was achieved in 9 patients. Primary position vertical alignment (up to ±8 PD) was achieved in all 10 patients.

Only 2 of 10 patients had A-pattern of 10 PD, in the remaining 8 patients it was eliminated (P<0.05). Postoperatively, 5 patients had no objective intorsion and 5 had grade I intorsion, and none of the patients had hypertropia or paradoxical eye movements.

Conclusion: Superior oblique transposition by Scott’s procedure along with conventional surgery on horizontal recti in third nerve palsy results in better horizontal and vertical alignment, and improves A-pattern and intorsion, thus leading to better binocular interaction.     

Roth Spots in Ocular Toxoplasmosis

Purpose: To report a case with unilateral preexisting ocular toxoplasmosis (OT) and newly occurred active retinochoroidal inflammation associated with white-centered retinal hemorrhages (Roth spots) in the healthy eye.

Design: Case report.

Methods: A 20 year-old man presented with a decrease of visual acuity in his right eye associated with 2+ cells in the anterior chamber. Ophthalmoscopy revealed an active retinochoroidal lesion on the upper nasal border of the optic disc associated with local hemorrhage and vitritis. The posterior pole presented white-centered flame-shaped retinal hemorrhages.

Results: Toxoplasmosis serology showed an IgG titer >300IU/ml and a negative IgM titer. A complete blood count revealed no abnormalities; other serologies were negative. After 2 weeks of treatment with sulfadiazine, pyrimethamine, folinic acid and prednisone, although the peripapillary lesion was still active, the Roth spots disappeared.

Conclusion: OT may be one differential diagnosis of patients suffering Roth spots in association with retinochoroidal inflammation.     

Peripapillary sparing in RDH12-associated Leber congenital amaurosis

Background: Peripapillary sparing is a characteristic that is traditionally described as pathognomonic for Stargardt disease.

Materials and methods: We present a multimodal assessment of four Leber congenital amaurosis (LCA) cases with congenital macular atrophy and severely attenuated electroretinogram findings caused by bilallelic mutations in RDH12.

Results: Fundus autofluorescence imaging revealed a general loss of retinal pigment epithelium across the macula except for the peripapillary region in both eyes of all patients. Spectral domain-optical coherence tomography confirmed relative preservation in this area along with retinal thinning and excavation throughout the rest of the macula. LCA was diagnosed based on clinical exam and retinal imaging, and subsequently confirmed with genetic testing.

Conclusions: Peripapillary sparing is a novel phenotypic feature of RDH12-associated LCA.