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A 29-year-old man presented with longstanding, stable, unilateral vision loss in the setting of a remote paintball injury. His examination was notable for a sensory exotropia as well as multiple foci of posterior synechiae, segments of white lenticular material and islands of lenticular cells within a grossly intact capsule, and severe zonular compromise in the affected eye. The majority of the nuclear lens material was absent. The patient was diagnosed with post-traumatic lens absorption and underwent synechialysis, capsulotomy, excision of remnant lenticular material, and placement of a sulcus lens, with significant improvement in visual acuity and ocular alignment following surgery. Our report uniquely highlights identification of a Soemmering's ring in an absorbed lens in the presence of an intact anterior and posterior capsule as well as successful refractive and sensorimotor outcomes following surgical repair despite delay in treatment of many years.

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Gelatinous drop-like corneal dystrophy (GDLD) is a rare autosomal recessive corneal dystrophy that has been associated with mutations in the TACSTD2 (M1S1) gene, which is normally expressed in corneal epithelial cells. GDLD is characterized by progressive deposition of amyloid in the corneal stroma with rapid recurrence in grafts after penetrating keratoplasty. We report of case of a patient with GDLD treated bilaterally with staged limbal stem cell transplantation and penetrating keratoplasty that resulted in long-term control of his disease. This case demonstrates that staged allogenic limbal stem cell transplantation, before or after penetrating keratoplasty, can be used to restore vision long-term in GDLD patients.

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Orbital penetrating injuries from pencils are rare. This report describes a case of penetrating orbital injury in a young child with a retained blue-colored pencil core foreign body, which led to rapid onset of orbital abscess requiring surgical drainage. Intraoperatively, orbital tissues were stained a bright-blue color. Histopathological study of specimen also highlighted bright blue aggregates of foreign material infiltrating the orbital tissues. Cultures grew Streptococcus mitis and Exophiala jeanselmei , which are rare causes of orbital abscess. Unique properties of retained colored pencil core as compared to graphite pencil core are herein discussed. Due to differences in composition, colored pencil core foreign bodies may require more timely surgical intervention compared to noncolored graphite pencil core.

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A 65-year-old male presented with headaches and painless episodes of unilateral vision loss. He had a history of renal cell carcinoma, in remission following surgery and immunotherapy with ipilimumab and nivolumab, discontinued 2 years and 3 months before presentation, respectively. MRI revealed an optic nerve sheath mass and perineuritis. After 1 month of corticosteroid therapy, there was a robust clinical and radiographic response, which relapsed dramatically following cessation. An optic nerve sheath biopsy showed chronic mild inflammation, and extensive work-up for alternative etiologies of orbital inflammation was negative. Following a prolonged taper of corticosteroids, he demonstrated complete response. In the setting of ocular immune privilege, ophthalmic immune-related adverse events (irAE) are rare, although multifarious. While on-treatment irAE are well-characterized, posttreatment irAE have become increasingly recognized across multiple organ systems. We report a case of a delayed-onset inflammatory optic nerve sheath mass and perineuritis after cessation of immunotherapy.

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Lacrimal sac squamous cell carcinoma is a rare but life-threatening disease that is often a delayed diagnosis secondary to difficulty in differentiating from other causes of dacrocystitis and acquired nasolacrimal duct obstruction. Chronic inflammation, including that of an underlying autoimmune disease, prior instrumentation, and poor wound healing, may be risk factors in the development to lacrimal sac squamous cell carcinoma. The authors present the first case of lacrimal sac squamous cell carcinoma associated with antineutrophil cytoplasmic antibody-associated vasculitis and immunoglobulin G4 positivity. Rather than an overlap syndrome between antineutrophil cytoplasmic antibody-associated vasculitis and immunoglobulin G4-related disease, high immunoglobulin G4 positivity may be considered an inflammatory marker of disease severity in the setting of antineutrophil cytoplasmic antibody-associated vasculitis and underlying malignancy. Inflammation-mediated tumorangiogenesis should be considered in the development of malignancy and red flags of chronic uncontrolled inflammation should warrant a lower threshold for further workup.

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Nodular fasciitis is a benign, idiopathic condition that can simulate both benign and malignant neoplasms. In adults, it generally occurs in the subcutaneous or superficial fascia of the trunk or upper extremities; occurrence in the periorbital region is far less common. We describe a case of a 16-year-old male with a 4-month history of a nodular, non-tender, progressively enlarging mass of the superotemporal periorbita. Histopathologic analysis of the excisional biopsy demonstrated nodular fasciitis, confirmed by molecular cytogenetic analysis that showed rearrangement of USP6.

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PURPOSE: To determine whether unilateral multifocal uveal melanomas (UM) in the setting of ocular melanosis (melanosis oculi) represent genetically independent tumors. DESIGN: Clinical case series. METHODS: Two patients with unilateral multifocal UM in the setting of melanosis oculi were included. Tumors were evaluated for gene expression profile (GEP) and next generation sequencing (NGS) for uveal melanoma-associated mutations. Histopathologic analysis of enucleated specimens was also performed when available. RESULTS: Patients were both female, ages 73 and 83 years. In Patient #1, the tumors both exhibited Class 2 GEP but each harbored a unique BAP1 mutation. In Patient #2, one tumor was Class 1 and harbored an SF3B1 mutation, whereas the other tumor was Class 2 and harbored a BAP1 mutation. CONCLUSIONS: Unilateral multifocal UM in the setting of melanosis oculi can arise due to the development of genetically independent primary tumors, which is detectable based on the mutation profile of each tumor. This is the first report of genetically-confirmed independent primary tumors in the setting of unilateral multifocal UM.

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PURPOSE: To report the histological findings and clinical course of 2 patients with microcyst-like epithelial keratopathy (MEK) associated with antibody-drug conjugate, depatuxizumab mafodotin. METHODS: Case series. RESULTS: Two patients with glioblastoma multiforme participating in a phase 3 clinical trial of the antibody-drug conjugate, depatuxizumab mafodotin, presented with bilateral MEK. Confocal imaging showed multiple large, round, hyperreflective lesions in the epithelium. Epithelial debridement was performed for symptomatic relief in both patients. Along with aggressive lubrication, bandage contact lenses, and reduction in the chemotherapeutic dose to maintenance levels, both patients experienced symptomatic improvement. However, MEK lesions recurred after re-epithelialization. Immunohistochemistry of the diseased epithelium showed immunoglobulin (Ig)G-positive granular cytoplasmic inclusions and increased cell apoptosis. CONCLUSIONS: Depatuxizumab mafodotin accumulates in the basal corneal epithelium resulting in MEK because of increased apoptosis. Frequent lubrication and bandage contact lenses can provide symptom relief.

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We report the clinical history and histopathology of the longest known postoperative course of a myopic cornea lenticule implanted via epikeratoplasty that also had been subjected to excimer laser and mitomycin. Despite the mechanical and photochemical processes the allogenic cornea stroma had sustained, it is notable that it retained structural integrity and clarity. This report affirms the potential long-term clarity and resilience of allogenic stromal cornea and supports its use as a potential stabilizing option in cornea allogenic intrastromal ring segments for keratoconus and variations thereof for other cornea disorders.

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The authors describe a rare case of recurrent ectopic meningioma associated with sphenoid encephalocele in the medial anterior orbit of a 52-year-old man with a history of a resected intracranial meningioma. Typical features of ectopic meningioma are reviewed as well as potential etiologies of this very rare recurrence of intracranial meningioma in the orbit. Treatment is typically surgical excision.The authors report a rare case of recurrent orbital ectopic meningioma in the right medial canthus of a 52-year-old man.

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PURPOSE: To report a case of Nocardia cyriacigeorgica endophthalmitis. METHODS: Case report. RESULTS: A 71-year-old man on long-term steroid therapy for poor respiratory function from lung carcinoma and presumed recent liver metastases presented with a four week history of blurry vision in the left eye. Ophthalmic examination revealed a white elevated mass in the macula with hemorrhage, concerning for metastasis. The patient was treated for presumed ocular metastases but had poor response to radiotherapy and was diagnosed four weeks later with N. cyriacigeorgica by retinal biopsy. Despite intravitreal and systemic antibiotics, the patient had progression of disease and died of respiratory involvement. CONCLUSION: Nocardia endogenous endophthalmitis can present as a mass retinal lesion in immunosuppressed patients with metastatic disease. Early vitreous and retinal biopsy may be required for definitive diagnosis and treatment. A pulmonary source of infection should be suspected and monitored closely.

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Subconjunctival granulomata developed in 2 patients after use of GenTeal gel to lubricate the cornea during strabismus repair. We report the clinical findings and histopathology of the subconjunctival nodules. These lesions are similar to those previously reported following use of GenTeal gel in retinal surgery and use of similar large polymers in nonocular surgeries. Copious irrigation should be considered when conjunctival wounds are exposed to GenTeal gel, and alternative corneal wetting agents should be considered for use during strabismus surgery.

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PURPOSE: Acanthamoeba is a protozoa that can lead to severe ocular disease and sequelae. Although intraocular Acanthamoeba infection is rare, the following case demonstrates an unusual presentation of recurrent Acanthamoeba infection in a 30 year old contact lens wearing male. OBSERVATIONS: After presenting with recurrent Acanthamoeba keratitis and undergoing various treatments, the patient developed nodular scleritis, which evolved into panophthalmitis, and ultimately, required enucleation. Eight months post-operatively, the patient developed orbital implant exposure secondary to persistent Acanthamoeba infection and underwent removal of the implant and aggressive, systemic treatment involving a multispecialty care team. He then underwent placement of a dermis fat graft and had no signs of persistent infection at the time of last follow-up, which was 24 months after placement of the dermis fat graft. CONCLUSIONS: and Importance: To the authors' knowledge, this is the first known case of Acanthamoeba infection causing orbital implant exposure. Persistent infection should be considered in Acanthamoeba patients who have undergone enucleation and have orbital implant exposure. Better knowledge regarding the pathogenesis and extracorneal complications of this challenging disease may improve patient care and outcomes.

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PURPOSE: To report the first histological characterization of microcyst-like epithelial keratopathy (MEK) associated with depatuxizumab mafodotin (ABT-414). METHODS: Case report. RESULTS: A 35-year-old man with glioblastoma multiforme participating in a phase III trial of the antibody-drug conjugate ABT-414 developed a large corneal abrasion from complications of MEK. Confocal imaging showed multiple large, round, hyperreflective lesions. Epithelial debridement was performed. Immunohistochemistry of the diseased epithelium showed IgG-positive granular cytoplasmic inclusions and increased cell apoptosis. With discontinuation of topical steroids, frequent lubrication, bandage contact lenses, and reduction in dose to maintenance therapy, the patient experienced symptomatic improvement. However, the MEK lesions recurred after debridement. CONCLUSIONS: ABT-414 accumulates in the basal corneal epithelium resulting in MEK due to increased apoptosis. Frequent lubrication and bandage contact lenses can provide symptom relief.

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BACKGROUND: Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans histiocytosis with cutaneous manifestations, most commonly of the periorbital skin, and is often associated with hematologic disorders such as monoclonal gammopathy. Treatment of NXG is notoriously difficult, and fraught with recurrence and progression. CASE PRESENTATION: The authors describe a case of NXG with periorbital involvement in a patient with a complex autoimmune and hematologic medical history. The biopsy of this rare lesion prompted subsequent evaluation for an underlying disorder, which led to the diagnosis of multiple myeloma. Her NXG lesions demonstrated remarkable clinical improvement after treatment with intravenous immunoglobulin (IVIG). CONCLUSIONS: This case demonstrates the ophthalmologist's critical role in the diagnosis and management of NXG, as early detection cannot only prevent ophthalmic consequences such as ocular perforation and blindness, but also prompt further investigation that may reveal an underlying disorder or systemic involvement, including hematologic malignancy as in this case. NXG has been effectively treated with IVIG in a handful of reported cases. To the author's knowledge, this is the third case of periorbital NXG successfully treated with IVIG, and the first in the ophthalmic literature.

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PURPOSE: To analyze 3 unusual mesenchymal transformations within the eye: adipose or osseous metaplasia of the lens and adipose tissue in the vitreous cavity. DESIGN: Observational case series. METHODS: Reevaluation of clinicopathologic diagnoses and histopathologic findings in sections stained with hematoxylin-eosin, periodic acid-Schiff (PAS) reaction, and Masson trichrome method. RESULTS: The 3 cases of mesenchymal transformation occurred in microphthalmic eyes with persistent hyperplastic primary vitreous (more recently termed persistent fetal vasculature). In 1 case there was total lens replacement with lamellar bone; in another, total replacement of the crystalline lens by adipose tissue; and in a third, an anomalous pocket of adipose tissue in the central vitreous. Multifocal remnants of the lens capsule were seen in the osseous case but were absent from the adipocytic cases. The vitreous adipose tissue was surrounded by an elaborate capillary plexus with an empty, collapsed PAS-positive lens capsule in the pupillary region. Anterior pigmented neuroectodermal disorganization, dysgenesis of angle structures, and a hypoplastic or disorganized iris were also observed in the 3 cases. CONCLUSIONS: After review of the literature, it appears that lenticular osseous replacement occurs more often than adipocytic. In addition to vascularization of the lens through a capsular dehiscence, other causes are explored, including direct epithelial-mesenchymal transformations of the lens epithelium or, less likely, of the disorganized adjacent neuroectoderm. The focus of vitreous adipose tissue may represent a transformed luxated lens extruded from its capsule, which was left behind in the pupillary zone.

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We describe a case of breast cancer metastatic to the superior oblique, in a male. The patient was a 66-year-old Caucasian male with a history of stage IIIB rectal adenocarcinoma and stage IIA left breast carcinoma diagnosed 12 years and 5 years prior, respectively, who presented with headaches and intermittent diplopia. He underwent left total mastectomy with sentinel lymph node biopsy 6 years prior, which showed ER/PR+, HER2/neu-, moderately-differentiated, infiltrating ductal carcinoma with 3/14 positive nodes. He completed adjuvant doxorubicin/cyclophosphamide and oral tamoxifen, and prior routine surveillance imaging had found no evidence of recurrent disease. MRI of the orbit revealed a 0.7 × 1.4 × 1.9 cm mass in the superomedial right orbit in the region of the superior oblique. Transcaruncular orbitotomy with biopsy of the superior oblique revealed metastatic breast carcinoma. Unfortunately, he developed new metastases. Post-operatively, he continues to have good vision with minimal diplopia.

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