Bilateral Inferior Rectus Schwannoma and Strabismus: A Case Report.

Schwannomas, also known as neurilemomas, are peripheral nerve sheath neoplasms. They can be sporadic or associated with genetic syndromes including neurofibromatosis type 2 (NF2). Schwannomas may lead to symptoms by exerting pressure on nearby structures, such as nerve and muscle fibers. In this study, we present the case of a 22-year-old female with a history of NF2 who, upon examination, presented with a visibly enlarged salmon-colored mass involving the left inferior rectus that she had since the age of 12 years. Ocular examinations revealed a small left hypertropia and exotropia in all gazes. Magnetic resonance imaging confirmed bilateral involvement of the inferior rectus muscles. She had a partial excisional biopsy of the mass involving the left inferior rectus muscle that confirmed the presence of schwannoma. This case highlights the importance of comprehensive evaluation of sensory and motor functions as well as considering orbital schwannomas in cases of strabismus, especially within the context of neurofibromatosis.

Cerebral vasculitis presenting as acute posterior multifocal placoid pigment epitheliopathy in a 16-year-old male.

Purpose: To report on a case of the successful treatment of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) in a pediatric patient with a prior diagnosis of cerebral vasculitis. Observations: A 16-year-old male with a prior diagnosis of cerebral vasculitis presented without ocular complaints. Visual acuity was 20/20, and color vision remained normal. Fundus examination revealed yellowish-white placoid lesions and retinal pigmented epithelial changes involving the posterior pole. A work-up including a rapid plasma reagin test, complete cell blood count, comprehensive metabolic panel, and urinalysis was within normal limits. A head computed tomography angiography without contrast and a brain magnetic resonance imaging scan were compatible with acute and past episodes of ischemia. Ancillary testing was compatible with an assessment APMPPE. Immunosuppressive and monoclonal antibody therapy resulted in the improvement and remission without residual neurologic deficits and with a BCVA of 20/20. Conclusionand Importance: This case suggests that a diagnosis of cerebral vasculitis should prompt physicians to consider an ophthalmic evaluation that includes a dilated fundus exam, regardless of the presence or absence of ocular symptoms. Ophthalmic findings may affect the diagnostic processes, particularly concerning infectious and non-infectious etiologies, or potentially neoplastic diseases.

Consequences of Purtscher-Like Retinopathy in a Patient With Systemic Lupus Erythematosus: A Case Report.

We report on a case of Purtscher-like retinopathy (PLR) secondary to systemic lupus erythematosus, which caused retinal ischemia, retinal neovascularization, vitreous hemorrhage, and, ultimately, a combined tractional and rhegmatogenous retinal detachment. A 23-year-old male presented with decreased visual acuity in the left eye (OS). He had a recent history of systemic lupus erythematosus and was being treated with systemic corticosteroids. At presentation, his visual acuity was counting fingers in the OS; a fundus examination of the OS revealed the presence of macular edema associated with flame hemorrhages, diffuse cotton-wool spots surrounding the macula, and vascular sheathing with the retina attached, all of which were consistent with PLR. Five months later, his fundus examination (OS) showed severe retinal ischemia and active neovascularization. The patient was scheduled for pan-retinal photocoagulation (PRP) laser therapy, but he was lost to follow-up. Subsequently, he returned two months later with progressive damage and was treated with PRP in the OS. An additional two months after PRP treatment, an examination revealed combined tractional and rhegmatogenous retinal detachment. Ultimately, the patient required a pars plana vitrectomy.

West Indian Punctate Keratopathy and "Puerto Rican (PR) Spots".

This study describes the clinical characteristics of a cohort of Hispanic patients living in Puerto Rico who were found to have West Indian punctate keratopathy (WIPK). This case also introduces the term "PR (Puerto Rican) spots" to describe the dots observed in the eyes of individuals with WIPK who have a documented history of residing in Puerto Rico. The methods of the study consist of a retrospective chart review of patients presenting with WIPK. The patient data were entered retrospectively into a new database and analyzed. Eighteen patients who had WIPK were identified. The median age at presentation was 60.5 years (range: 49-72); 61.1% were female. At presentation, only one patient had both eyes affected. The median number of PR spots on examination was 1 (range: 1-4). All the patients had a history of ocular disease, most frequently glaucoma (55.5%), and had lived in Puerto Rico for more than 40 years. A total of 33.3% of the patients were retired or unemployed at the time of their presentation. While the origin of these dots remains unclear, ongoing efforts to document and characterize WIPK and PR spots will persist, with the aim of enhancing our understanding of this clinical entity.

West Indian (Caribbean) Punctate Keratopathy in a Puerto Rican Girl.

We report on a case of West Indian (Caribbean) punctate keratopathy (WIPK) in a pediatric patient living in Puerto Rico, USA. A 9-year-old Hispanic girl presented for a routine ophthalmic follow-up. The patient had a history of juvenile idiopathic arthritis and chronic bilateral anterior uveitis. At the presentation, her visual acuity was 20/30 in the right eye and 20/20 in the left eye. An examination of the right eye was remarkable for one round, white subepithelial corneal opacity of approximately 0.1 mm in height by 0.1 mm in width, located slightly lateral to the center of the cornea, which was consistent with WIPK. This case highlights the importance of recognizing WIPK in children who have a history of living on any one or more of the Caribbean islands.

Tolosa Hunt Syndrome: MRI Findings.

Tolosa-Hunt syndrome (THS) is an idiopathic inflammatory condition involving the cavernous sinus and orbital apex with an incidence of 1 case per million per year. We report on a case of a 70-year-old male with atypical MRI findings, vision loss, and painless ophthalmoplegia.  Ophthalmic evaluation revealed his best-corrected visual acuity was 20/40 in the right eye and counting fingers at a 0.5-foot distance in the left eye. External examination of the left eye revealed limited ocular movement, proptosis, and a positive relative afferent pupillary defect. Complete blood count, inflammatory markers, and full biochemistry tests, including thyroid and liver function tests, were within the normal range. A magnetic resonance imaging of the orbits with and without contrast demonstrated a homogenously enhancing lesion at the posterior intraconal compartment of the left orbit, extending to the orbital apex with the involvement of the adjacent extraocular muscles. The patient was started on intravenous methylprednisolone 60 mg daily and later discharged on prednisone 5 mg daily with partial symptom improvement on follow-up. Resection and biopsy revealed a soft tissue lesion with mixed inflammatory infiltrate. The clinical, pathological, and imaging findings favored the diagnosis of THS.

Managing a Pigmented Corneal Ulcer in a 58-Year-Old Patient.

We report on a case study involving a 58-year-old male with a pigmented corneal ulcer. The patient presented with a two-month history of an unresolved corneal ulcer in the oculus sinister (OS), accompanied by increasing ocular discomfort. His best corrected visual acuity (BCVA) was 20/20 oculus dexter and hand motion OS. Examination of OS revealed mild conjunctival injection, diffuse corneal edema, and the presence of a central pigmented lesion. Microbiological analysis via culture identified the causative agent as Ochroconis fungi, belonging to the dematiaceous species. Subsequently, the patient's condition was managed through a comprehensive regimen that included multiple topical antifungal agents, a topical antibiotic, and povidone-iodine drops. After two months of treatment, the patient exhibited improvement in his condition. His BCVA improved to counting fingers at a distance of two feet OS.

Retinitis Pigmentosa Sine Pigmento in a Carrier of Usher Syndrome.

We report a carrier of Usher syndrome type I with retinitis pigmentosa sine pigmento. A 71-year-old male was referred for further evaluation of severe, progressive, painless vision loss in both eyes over the course of four years. He had bilateral sensorineural hearing loss. Upon a comprehensive examination, his best-corrected visual acuity was 20/100 in the right eye and 20/40 in the left eye. He had an unremarkable anterior segment examination and normal intraocular pressures in both eyes. Upon fundus examination, the patient had pale discs, optic disc cupping, and multiple scattered drusen in the macula and at the midperiphery of both eyes. Optical coherence tomography showed retinal nerve fiber layer thinning in all quadrants. The visual field was severely constricted in both eyes. A comprehensive workup for infectious and inflammatory causes, as well as a brain MRI, was unremarkable. Sequencing analysis showed that he carried a heterozygous pathogenic mutation, USH1C c.672C>A (p.Cys224*) variant. Usher syndrome is a rare genetic disease characterized by hearing loss and retinitis pigmentosa. Our case suggests that patients and carriers of Usher syndrome may have a phenotype compatible with retinitis pigmentosa sine pigmento.

Bilateral Acute Retinal Necrosis Treated With Antivirals and Corticosteroids.

We report on the case of a Hispanic male with bilateral acute retinal necrosis (BARN), whose disease was managed with intravenous acyclovir, topical prednisolone, topical atropine, intravitreal ganciclovir, oral valacyclovir, and oral prednisone. The oral corticosteroid was added to his treatment regimen eight days after his initial presentation. The 55-year-old patient presented with a two-week history of bilateral blurred vision. His medical history was pertinent for remote varicella and herpes zoster (the latter limited to the forehead). His best-corrected visual acuity (BCVA) was counting fingers in both eyes (OU). His examination was remarkable in OU for grade 3+ cells, pharmacologically dilated pupils, and grade 2+ vitreous cells. The patient's fundus was remarkable in OU for optic-nerve swelling, vitreous condensation, ghost vessels, and retinitis patches. Given the clinical and ancillary testing results, an assessment of BARN was made. The patient received acyclovir treatment initially, and systemic steroids were introduced eight days later. He ultimately experienced significant clinical improvement.

Xanthogranuloma of the Eyelid: A Case Report.

We report on the case of a Hispanic woman with necrobiotic xanthogranuloma (NBX) whose disease was managed based on her symptoms. She underwent a diagnostic and debulking surgical intervention and surveillance for hematologic malignancy. This 56-year-old patient presented with a six-year history of enlarging masses and swelling around her eyes, with intermittent inflammation, associated pain, and occasional redness. Her past medical history was remarkable for asthma and nasal polyps. Upon external examination, she had severe fullness of the upper lids with yellow plaques and palpable masses along them, nontender palpation, the absence of visible erythema, and blepharoptosis in both eyes. The patient presented with bilateral visual field constriction due to mechanical obstruction. An orbital computed tomography scan revealed a dense diffuse lesion involving the pre- and postseptal tissues and invading the orbit of the right eye. A facial magnetic resonance imaging scan revealed infiltration of the postseptal spaces within both orbits. A skin and soft tissue biopsy from the bilateral periorbital regions of both eyes confirmed the diagnosis of NBX. A workup for underlying hematologic malignancies, including plasma cell dyscrasias and lymphoproliferative disorders, was unremarkable. The patient underwent diagnostic and debulking surgery in an attempt to improve her visual function. Subsequently, she was scheduled for ongoing monitoring of her disease progression.

Bilateral Acute Iris Transillumination Syndrome Following Oral Moxifloxacin Overdose.

We report a case of bilateral acute iris transillumination (BAIT) syndrome caused by an overdose of oral moxifloxacin in a Hispanic female patient with no previous respiratory viral infection. A 56-year-old Hispanic female with no history of ocular illness was referred to our glaucoma service to manage her microcystic edema, swelling, and refractory ocular hypertension. Her ocular and systemic symptoms, including progressively worsening bilateral ocular pain, severe photophobia, blurred vision, nausea, and vomiting, started 14 days after an accidental overdose of oral moxifloxacin. Moxifloxacin had been prescribed to treat a complicated urinary tract infection. A slit-lamp examination revealed bilateral microcystic corneal edema and transillumination in the right temporal iris, both consistent with a diagnosis of BAIT syndrome. The existing literature on BAIT syndrome is scarce, and its etiology remains unclear. This case provides clinical evidence supporting moxifloxacin toxicity as a possible cause of BAIT syndrome. We emphasize the importance of conducting extensive research to define the mechanisms involved in moxifloxacin-induced BAIT syndrome and to search for other potential etiologies of this condition.

Histocompatibility Leukocyte Antigen-A29-Associated Retinal Vasculitis without Choroidal Lesions: A Report of 4 Cases.

This study describes a cohort of patients presenting with histocompatibility leukocyte antigen (HLA)-A29-associated retinal vasculitis without choroidal lesions that may share clinical features with birdshot retinochoroiditis. The methods include a retrospective chart review of patients presenting with HLA-A29-associated retinal vasculitis without choroidal lesions. The data on the patients were entered retrospectively into a new database and analyzed. Four patients who had HLA-A29-associated retinal vasculitis without choroidal lesions were identified. The median age at presentation was 40 years (range: 14-71); 75% were female. At presentation, all four patients had a visual acuity of 20/50 or better in both eyes. All the eyes had mild vitritis, three eyes (37.5%) had cystoid macular edema, and two eyes (25%) had optic disc edema. All the patients required treatment with systemic steroids and immunosuppressive therapy. HLA-A29-associated retinal vasculitis without choroidal lesions appears to share many clinical features with birdshot chorioretinitis, including the need for systemic immunosuppressive therapy. Whether this entity represents an early form of birdshot retinochoroiditis or a more localized variant of the disease is a topic for additional studies.

Systemic Disease Associations in a Cohort of Hispanic Patients with Scleritis.

(1) Purpose: A patient with scleritis may have an associated systemic disease, which is often autoimmunological and seldom infectious in origin. The data regarding such associations in Hispanic populations are scarce. Therefore, we evaluated the clinical characteristics and systemic-disease associations of a cohort of Hispanic patients with scleritis. (2) Methods: A retrospective review of the medical records (January 1990-July 2021) of two private uveitis practices in Puerto Rico was performed. Clinical characteristics and systemic-disease associations observed either at presentation or diagnosed as a consequence of the initial workup were recorded. (3) Results: A total of 178 eyes of 141 patients diagnosed with scleritis were identified. An associated autoimmune disease was present in 33.3% of the patients (rheumatoid arthritis, 22.7%; Sjögren's syndrome, 3.5%; relapsing polychondritis, 2.8%; sarcoidosis, 1.4%; systemic lupus erythematosus, 1.4%; and systemic vasculitis, 0.7%). An associated infectious disease was present in 5.7% of the patients (2.13%, syphilis; 1.41%, herpes simplex; 1.14%, herpes zoster; and 0.71%, Lyme disease). One patient had all-trans retinoic-acid-associated scleritis. Statistical analysis revealed that patients with nodular anterior scleritis were less likely to have an associated immune-mediated disease (OR: 0.21; p = 0.011). (4) Conclusion: Rheumatoid arthritis was the most common systemic autoimmune disease association, while syphilis was the most common infectious disease associated with scleritis patients. Our study suggests that patients with nodular scleritis have a lower risk of having an associated immune-mediated disease.

Aflibercept as a Treatment for Secondary Central Serous Chorioretinopathy in a Patient With Myasthenia Gravis.

We report on a case of central serous chorioretinopathy (CSCR) secondary to chronic steroid use that showed sustained improvement when treated with an aflibercept intravitreal injection. A 44-year-old woman presented with decreased visual acuity of the left eye (OS). The patient had a recent history of myasthenia gravis and was being treated with systemic corticosteroids and immunosuppressants. At presentation, her visual acuity was 20/80 OS; an examination (using fluorescein angiography) of the left fundus revealed a serous retinal detachment of the posterior pole that extended to the mid-periphery and multiple areas of leakage, which findings were consistent with CSCR. The patient also had a history of unresolved strabismic amblyopia in her right eye. The patient's CSCR was managed with one injection of intravitreal aflibercept (2 mg/0.05 mL). One month following treatment, her visual acuity improved to 20/20 OS, and the serous retinal detachment had resolved. Ten months following treatment, an examination revealed a sustained improvement, with a visual acuity of 20/20 OS. Concomitantly, the patient's amblyopic eye revealed an improved visual acuity of 20/20. Our case suggests that some cases of secondary CSCR may respond to treatment with intravitreal aflibercept. This case also suggests that the CSCR imposed a unique form of occlusion therapy that helped improve the amblyopia of the contralateral eye in this adult patient.

Contralateral Recurrences of Post-vaccination Multiple Evanescent White Dot Syndrome.

We report on a case of multiple evanescent white dot syndrome (MEWDS) following the simultaneous administration of the human papillomavirus and meningococcal (conjugate) vaccines and two recurrences of MEWDS following the administration of the second dose of the human papillomavirus (HPV) vaccine and the COVID-19 vaccine and COVID-19 viral infection. A 17-year-old Hispanic female presented with a one-week history of photopsia and blurred vision in her left eye following the simultaneous administration of the human papillomavirus and meningococcal (conjugate) vaccines. Upon a comprehensive examination, her best-corrected visual acuity was 20/20 in the right eye and 20/100 in the left eye. A left fundus examination revealed multiple white dots in the macula and nasal periphery, consistent with a diagnosis of MEWDS. Ancillary testing, including fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and optical coherence tomography, supported the diagnosis. One month following her initial diagnosis, the patient's symptoms had resolved without any therapy, and a fundus examination revealed multiple relatively ill-defined brown-colored subretinal lesions in the nasal midperiphery, corresponding to the location of the previous MEWDS lesions. Subsequently, she received the second dose of the HPV vaccine and then developed a mild COVID-19 infection. Four months after the initial presentation, she received the first dose of the BNT162b2 COVID-19 vaccine, followed by the second dose a month later. Eight months following her initial presentation, she presented with photopsia in the right eye. Her visual acuity remained 20/20 in the right eye and improved to 20/20 in the left eye, and white dots were identified nasal to the disk and surrounding the peripapillary region; the contralateral MEWDS diagnosis was confirmed by the previously mentioned ancillary tests. At her one-month follow-up, she presented new onset photopsia of the right eye. Her visual acuity remained 20/20 in both eyes, and a fundus examination revealed white lesions suggestive of active MEWDS temporal to the macula and brown-colored spots nasal to the disk, suggestive of recovering MEWDS, nasally. The aforementioned testing confirmed the coexistence of new and resolving lesions; nonetheless, the patient's symptoms resolved without any therapy, and she received the third dose of the BNT162b2 COVID-19 vaccine 11 months after her initial presentation. Our case suggests that vaccines may serve as immunological triggers of MEWDS. Recurrent MEWDS may occur when an individual is exposed to a powerful immune challenge, such as receiving a wide array of vaccinations in a short period of time. We believe this case constitutes a previously undescribed finding of multiple relatively ill-defined brown-colored subretinal lesions present in late MEWDS.