A Case of Chorioretinitis with Retinal Angiomatous Proliferation

A 48-year-old woman had an acute blurred vision in the right eye immediately after drainage of liver abscess. Her best corrected visual acuity (BCVA) was 8/400; fundus photography suggested the diagnosis of endogenous endophthalmitis with chorioretinitis and vitritis. Due to the bad systemic condition, a systemic antibiotic combined with periocular triamcinolone (TA) was carried out first. Inflammatory cells in the vitreous cavity were decreased after treatment; however, fundus fluorescein angiography (FFA) showed abnormal dilation and leakage of the capillaries and retinal-choroidal anastomose, supporting that there was retinal angiomatous proliferation (RAP). Vitreous interleukin-6 (IL-6) was only slightly elevated; the ratio of interleukin-10 (IL-10) and IL-6 was less than 1, and the etiological test was negative. After receiving intravitreal vancomycin injection combined with periocular TA injection, the patient’s BCVA was improved from 16/400 to 20/400 with a reduction in vitreous inflammatory cells. However, the patient’s RAP was progressed and her BCVA was dramatically decreased to count finger/30 cm. After intravitreal injection of ranibizumab, the patient’s BCVA was 5/400 with a significant shrink in lesions and absorption of hemorrhage, exudation, and fluid. Thus, we suggest that early anti-inflammatory treatment in conjunction with anti-VEGF may achieve a better prognosis in patients with inflammatory retinal angiomatous proliferation (RAP).

Retinal Occlusive Vasculitis in a Patient with Hyperimmunoglobulin E Syndrome

Background. Hyperimmunoglobulin E syndrome (HIES), or Job’s syndrome, is a primary immunodeficiency disorder that is characterized by an elevated level of IgE with values reaching over 2000 IU ( normal < 200 IU ), eczema, and recurrent staphylococcus infection. Affected individuals are predisposed to infection, autoimmunity, and inflammation. Herein, we report a case of HIES with clinical findings of retinal occlusive vasculitis. Case Presentation. A 10-year-old boy with a known case of hyperimmunoglobulin E syndrome had exhibited loss of vision and bilateral dilated fixed pupil. Fundoscopic examination revealed peripheral retinal hemorrhaging, vascular sheathing around the retinal arteries and veins, and vascular occlusion in both eyes. A fluorescein angiography of the right eye showed hyper- and hypofluorescence in the macula and hypofluorescence in the periphery of the retina, peripheral arterial narrowing, and arterial occlusion. A fluorescein angiography of the left eye showed hyper- and hypofluorescence in the supranasal area of the optic disc. Macular optical coherence tomography of the right eye showed inner and outer retinal layer distortion. A genetic study was performed that confirmed mutations of the dedicator of cytokinesis 8 (DOCK 8). HSV polymerase chain reaction testing on aqueous humor and vitreous was negative, and finally, the patient was diagnosed with retinal occlusive vasculitis. Conclusion. Occlusive retinal vasculitis should be considered as a differential diagnosis in patients with hyperimmunoglobulin E syndrome presenting with visual loss.

Multimodal Imaging of Waldenstrom Macroglobulinemia-Associated Hyperviscosity-Related Retinopathy Treated with Plasmapheresis

While plasmapheresis is well known to significantly improve both retinal findings and systemic manifestations associated with Waldenstrom macroglobulinemia, few reports exist documenting changes in optical coherence tomography angiography (OCT-A). The authors present a case of a patient with Waldenstrom macroglobulinemia who had resolution of white-centered peripheral retinal lesions and parafoveal outer nuclear layer hyperreflective material following plasmapheresis. Applying image analysis software to before and after OCT-A images, the authors were able to show an objective decrease in retinal capillary and large vessel density following plasmapheresis. This technique can be used to guide treatment and surveillance for patients with hyperviscosity-related retinopathy.

Sickle Cell Trait Presenting as Unilateral Proliferative Retinopathy and Macular Thinning in a Pregnant Woman

Background. To report a case of a pregnant woman with sickle cell trait (SCT) who presented with unilateral proliferative sickle cell retinopathy. Case Presentation. A 26-year-old otherwise healthy pregnant woman presented with the complaint of visual loss in her left eye. The funduscopic examination showed vitreous hemorrhage, sea fan neovascularization, and pale optic disc. Optical coherence tomography revealed macular inner retinal layer thinning and foveal splaying (saucerization of the foveal pit). The investigations, including hemoglobin electrophoresis, verified the diagnosis of sickle cell trait. Blood pressure, fasting blood glucose tests, and tuberculin skin tests were normal. We treated the patient by peripheral retinal photocoagulation over the area of nonperfusion. Conclusion. Even though the sickle cell trait is generally considered as a milder form of sickle cell disease without severe retinal manifestations, pregnancy should be considered as a trigger that can induce proliferative changes and foveal splaying in this group of patients.

Combined Rothia dentocariosa and Streptococcus viridans Corneal Ulcer in an Immunocompromised Patient

Keratitis is a very common condition seen by ophthalmologists. However, many factors can complicate the treatment of this depending on the causative organism and other patient comorbid conditions. The objective of this clinical case report is to highlight the treatment of keratitis caused by Rothia dentocariosa. It also looks at the unique considerations in keratitis presentations for patients immunocompromised by chemotherapy agents. Our patient is a 58 yo female undergoing chemotherapy with folinic acid, fluorouracil, irinotecan, and panitumumab who presented with several days of a red, painful right eye with mucous discharge. Cultures were positive for Rothia dentocariosa and Streptococcus viridans. The patient ultimately underwent a conjunctival flap procedure as medical therapy with proper oral and topical antibiotics failed to resolve keratitis. This case is unique as previously, only a couple of cases of keratitis caused by Rothia dentocariosa have been reported and none of those patients were immunocompromised nor failed antibiotic therapy.

Progression of Subclinical Pachychoroid Neovasculopathy to an Active Neovascularization in the Presence of Acquired Vitelliform Lesions

We describe a unique case of bilateral acquired vitelliform lesions in a 67-year-old-female with pachychoroid associated with subretinal fluid in the right eye (OD) and a nonexudative choroidal neovascular membrane (CNVM) in the left eye (OS). Multimodal imaging performed at baseline and over the ensuing two years showed an increase in the OS vitelliform lesions with a concurrent transformation of quiescent CNVM to an exudative form. Further studies are warranted to gain better insight into the etiopathogenesis of these vitelliform lesions in pachychoroid and their potential role in instigating CNVM activation.

Temporal Approach, Digitally Assisted Phacovitrectomy in a Patient with Severe Kyphosis due to Axial Spondyloarthritis

Purpose. To describe a temporal approach, digitally assisted phacovitrectomy in a patient with severe kyphosis due to axial spondyloarthritis. Case Report. A 70-year-old male patient with proliferative diabetic retinopathy with vitreous hemorrhage and cataract and ankylosing spondylitis with severe kyphosis. A temporal approach, digitally assisted 25 G phacovitrectomy was performed with a Constellation platform and the NGENUITY visualization system. The Trendelenburg position was utilized. Conclusion. A temporal approach, digitally assisted phacovitrectomy may be used in select cases of severe kyphosis with positive outcomes.

Sinoorbital Mucormycosis Associated with Corticosteroid Therapy in COVID-19 Infection

Background. Mucormycosis is a rare and invasive fungal infection, affecting almost exclusively immunocompromised individuals. Immunosuppressive effects of corticosteroids which are widely prescribed in COVID-19 patients might be a predisposing factor for opportunistic infections even though the other factors should also be considered. Case Presentation. A middle-aged man without any significant past medical history was admitted to the hospital due to a severe COVID-19 infection. He received a high dose of corticosteroids as a part of the treatment. Five days after discharge, he presents with a headache and fever. Eventually, orbital mucormycosis was diagnosed for him and he was treated with antifungal medications. Conclusion. Opportunistic infections should be considered during the current pandemic of COVID-19, during which corticosteroids are widely prescribed.

Nonarteritic Anterior Ischemic Optic Neuropathy following COVID-19 Vaccination: Consequence or Coincidence

To report a patient with nonarteritic anterior ischemic optic neuropathy (NA-AION) occurring soon after the COVID-19 vaccination. A 55-year-old woman presented with a 4-day history of inferior visual field disturbance in the right eye 7 days after receiving the first dose of Pfizer-BioNTech COVID-19 vaccine. Examination revealed a best-corrected visual acuity of 20/20 in both eyes. A relative afferent pupillary defect was observed in the right eye. Fundoscopy revealed diffuse optic disc swelling in the right eye, which was prominent above the optic disc. Goldmann visual field testing identified an inferior altitudinal visual field defect with I/2 isopter in the right eye. Although typical complete inferior visual field defect was not detected, a diagnosis of NA-AION was made. The patient was followed without any treatment. During the 2-month follow-up period, the optic disc swelling was gradually improved, and visual acuity was maintained 20/20; however, the optic disc looked diffusely pale in the right eye. Although it is uncertain whether the development of NA-AION after COVID-19 vaccination was consequential or coincidental, we speculate that the close temporal relationship with COVID-19 vaccination suggests the possibility of vasculopathy on the microvascular network of optic nerve head as background of inflammatory or immune-mediated element to the timing of the onset of NA-AION. The aim of this case report is to present this biological plausibility and to elucidate potential ophthalmological complications.

Ophthalmic Diagnosis and Novel Management of Infantile Refsum Disease with Combination Docosahexaenoic Acid and Cholic Acid

Infantile Refsum disease is a rare peroxisomal biogenesis disorder characterized by impaired alpha-oxidation and accumulation of phytanic acid in the tissues. Patients often present with fundus changes resembling retinitis pigmentosa, developmental delay, sensorineural hearing loss, ataxia, and hepatomegaly. Traditionally, mainstay treatment for this condition has been a phytanic acid-restricted diet, although supplementation with either docosahexaenoic acid or cholic acid has rarely been described in the literature. We present a case of infantile Refsum disease in a child with retinitis pigmentosa-like ocular findings, sensorineural hearing loss, and self-resolving hepatic disease, who developed novel findings of macular edema refractory to carbonic anhydrase inhibitors. We describe management with a phytanic acid-restricted diet and combination docosahexaenoic acid, and cholic acid therapy, which helped to limit progression of her disease.