Carotid artery dissection associated with Purtscher-like retinopathyPurtscher retinopathy was first described in 1910 by Otmar Purtscher in a man who suffered a traumatic head injury.1 Patients commonly present with a loss of visual acuity and a recent history of injury such as head trauma or chest compression.2,3 In the case of a nontraumatic injury, the terminology commonly used is “Purtscher-like retinopathy.” It is associated with various systemic diseases, including acute pancreatitis, pancreatic adenocarcinoma, thrombotic thrombocytopenic purpura, connective tissue disorders, and hemolytic uremic syndrome.
Severe chorioretinal atrophy in Boucher-Neuhauser syndromeA 51-year-old woman was seen in the neuro-ophthalmology clinic. She reported difficulties with night vision dating back to approximately age 10 and an abnormal gait since childhood, which was described as unsteady and hesitant with stiffness of both legs. Ten years previously she was noted to have nystagmus by her family practitioner and was referred to an ophthalmologist, who diagnosed her with probable retinitis pigmentosa (RP). At that time, visual evoked potentials showed delayed P100 responses bilaterally.
Wet age-related macular degeneration refractory to aflibercept but responsive to systemic everolimusA 69-year-old Caucasian woman with a history of metastatic breast cancer had been receiving injections of aflibercept every 4–8 weeks for wet age-related macular degeneration in her right eye for the past 4 years. Despite the regular frequency of the aflibercept injections, her optical coherence tomography had always demonstrated significant levels of intraretinal fluid (Fig. 1). It was decided in the past to maintain her schedule of aflibercept in order to maintain her baseline level of vision of 20/60.
Triple-hit lymphoma of the cavernous sinusDiffuse large B-cell lymphoma (DLBCL) is the most common non–Hodgkin-type lymphoma in adults, and the myriad of clinical presentations is well known to neuro-ophthalmologists.1 Traditionally, DLBCL was treated with standard chemotherapy, including R-CHOP (i.e., rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). Newer cytogenetic, fluorescent in situ hybridization (FISH), and next-generation sequencing (NGS) techniques, however, have shown the presence of genomic abnormalities that have significant treatment and prognostic implications.
Concurrent cerebral toxoplasmosis and cytomegalovirus retinitis in a patient with human immunodeficiency virusA 35-year-old previously healthy female recently emigrated from Honduras and presented with a five-day history of sudden onset decreased vision in her right eye, pain with extraocular movements, fevers, and a 3-day history of right upper and lower extremity weakness and numbness. On exam, she was afebrile, and mental status was normal. She had no light perception in the right eye, a right relative afferent pupillary defect, right lower facial weakness, 4/5 strength of proximal and distal right upper and lower extremities, and decreased sensation in the right face, arm, and leg.
Conjunctival Kaposi’s sarcoma with orbital extension in an HIV-negative manKaposi’s sarcoma (KS) is a vascular tumour whose development requires infection by human herpesvirus 8 (HHV-8). Although most commonly found in immunosuppressed patients, such as those AIDS, in the United States, classic KS is often found in older-aged men of European or Mediterranean ancestry without immunosuppression. Involvement of the skin of one’s extremities in classic KS is typically more common than that of viscera or mucocutaneous surfaces.
Unilateral transient vision loss associated with an opacified Onodi cellAn Onodi cell, also known as a sphenoethmoidal air cell, is a posterior ethmoidal air cell that has pneumatized laterally and rarely superiorly to the sphenoid sinus and is in close approximation to the optic nerve.1 When drainage of the cell is obstructed, visual disturbances can occur by physical compression, inflammation, or a combination of the 2. Nonetheless, optic neuropathy secondary to an isolated opacified Onodi cell is rare.2–4 Herein, we report a case of episodes of unilateral, transient, and painless vision loss caused by an opacified Onodi cell secondary to a focal sinusitis.
Unusual ocular presentation in a patient with lichen planusLichen planus is a relatively common mucocutaneous disorder of unknown etiology that is thought to be immunologically mediated. It is a chronic disorder characterized by periods of exacerbation and remission. Lichen planus has variable clinical manifestations, including the skin, oral mucosa, genital mucosa, nails, and scalp. The characteristic lesions have a well-established clinical appearance and histological features that help in establishing the diagnosis.1 Ocular involvement may occur in patients with lichen planus.
Mydriasis due to Opcon-A: An indication to avoid pharmacologic testing for anisocoriaA 35-year-old woman presented to the Emergency Department with an acute, painless, dilated left pupil. Her medical history was notable for myopia and truncal herpes zoster infection that had completely resolved 2 years ago. Ocular history was significant for bilateral contact lens wear, and her preferred wetting and cleaning solution was Opcon-A (naphazoline and pheniramine). Her surgical, social, and family history and a complete review of systems were unremarkable. On examination, the visual acuity was 20/20 OU.
Unilateral isolated foveal hypoplasiaA 9-year-old male was referred to the Pediatric Ophthalmology service for a suspected epiretinal membrane at the left macula. The patient was asymptomatic. His ocular, systemic medical, birth, and developmental history were all noncontributory, and he was not on any prescription medication.
Conjunctival-limbal allografts in graft-versus-host disease using same HLA-identical bone marrow transplantation donorHematopoietic stem cell transplantation (HSCT) allows for the transplantation of multipotent hematopoietic stem cells for treatment of hematologic, immunologic, metabolic, and neoplastic diseases. Graft-versus-host disease (GVHD) is a unique complication of allogeneic HSCT in which the donor cells mount an immune response against the host cells.1 Ocular GVHD affects 60%–90% of patients with chronic systemic GVHD.2
Bilateral isolated choroidal melanocytosis with isoautofluorescenceOcular melanocytosis is a congenital hyperplasia of the melanocytes in the ocular tissues: episclera, iris, ciliary body, and choroid. It is usually unilateral but can be bilateral. It can affect the surrounding eyelids, as in oculodermal melanocytosis, or be isolated to the choroid, which is referred to as isolated choroidal melanocytosis. Isolated choroidal melanocytosis is an uncommon condition that has been reported unilaterally in a number of cases,1,2 but bilaterally in only 5 other cases in the literature.
Late-onset Leber hereditary optic neuropathy presenting after intraocular surgeryLeber hereditary optic neuropathy (LHON) is a retinal ganglion cell degeneration characterized by bilateral, typically sequential, acute/subacute central visual loss. This maternally inherited condition arises from point mitochondrial DNA (mtDNA) mutations, and more than 90% of affected individuals harbour 1 of 3 primary mtDNA variants, m.11778G>A, m.14484T>C, or m.3460G>A.1 Intriguingly, (i) the majority of subjects harbouring mtDNA mutations do not develop visual symptoms, (ii) a striking number of LHON patients have no family history, and (iii) there is significant male predominance (male:female, ~3:1).
Mantle cell lymphoma: conjunctival mass in a female patientMantle cell lymphoma (MCL) is a rare neoplasm in the ocular adnexa, accounting for 3%–5% of conjunctival B-cell non-Hodgkin lymphomas (NHL).1,2 Conjunctival MCL typically presents in male patients in the eighth decade and is often advanced at the time of presentation.3 Treatment typically consists of chemotherapy or radiation, although the prognosis is poor.4 The authors present a rare case of conjunctival MCL in a female patient. Collection and evaluation of protected patient health information complied with the Health Insurance Portability and Accountability Act.
Unilateral retinitis pigmentosa: clinical and electrophysiological diagnosisRetinitis pigmentosa (RP) is a group of hereditary disorders characterized by gradual atrophy and cell death of the photoreceptors and adjacent cell layers of the retina, the common feature of which is progressive deterioration in vision. Initial presenting symptoms of the disease include night blindness, with gradual deterioration of the light-sensitive cells of the retina, causing diminution of vision in daylight in the later stages. Presentation is bilateral and symmetrical.
Transscleral fixation of a toric intraocular lens by a slipable suture techniqueIn keratoplasty, astigmatic correction is crucial for achieving a favourable visual outcome. Several procedures have been introduced for astigmatic correction, including astigmatic keratotomy,1 wedge resection,2 and excimer laser refractive surgery.3 Each of these procedures have limitations, including unpredictable refractive outcomes, technical difficulty, expenses, and potential complications.4
Bilateral simultaneous artificial iris implantation for post-traumatic aniridia: a case reportA blunt ocular trauma may lead to sequelae of aniridia and aphakia. Such presentations may need complex reconstructive surgery. Aniridic patients frequently experience symptoms of decreased visual acuity, diplopia, glare, photophobia, and cosmetic discrepancy. A reconstructive procedure may prove to be challenging when there is loss of capsular support or when minimal iris remnant is present. Oltra et al.1 reported one case of bilateral traumatic aniridia for which coloured contact lenses were offered because patient was asymptomatic.
Clinical evaluation of a novel surgical technique (large cross incision) for conjunctival cystsAn acquired conjunctival cyst may occur spontaneously or after ocular surgery or trauma.1 Chief complaints are foreign-body sensation or cosmetic issues. Although simple needle aspiration might be an easy choice for the treatment of conjunctival cysts, such an approach may be associated with frequent recurrence of the cysts. Typically, a simple resection can resolve the problem; however, incomplete resection may also result in recurrence of the lesion. Moreover, identification of the cyst margin can be difficult because of poor visualization of the cyst capsule.
Choroidal metastasis arising from esophageal adenocarcinomaThe majority of choroidal metastasis in the eye arises from primary tumours of the breast, lung, prostate, and gastrointestinal tract primary sites but rarely from the esophagus.1–4 Recently, a few cases have been reported that may coincide with the increasing incidence of Barrett’s esophagus and associated esophageal adenocarcinoma.1,2,5 We present a case of esophageal adenocarcinoma that initially presented with signs and symptoms of choroidal metastasis.
Orbital metastatic small cell carcinoma of the pancreas with optic nerve compressionSmall-cell carcinoma accounts for 18%–20% of all primary lung malignancies but has also been described as arising from the bladder, prostate, stomach, colon, and pancreas.1 Small-cell cancer of the pancreas (SCCP) is an extremely rare malignancy and represents only 0.2%–1% of all pancreatic malignancies.1,2 Here, we describe a unique case of an SCCP presenting as a metastatic lesion to an extraocular muscle causing optic nerve compression.
Structural and functional degeneration of retinal nerves in sibling carriers of a Leber’s hereditary optic neuropathy mutationLeber’s hereditary optic neuropathy (LHON)1 presents with painless bilateral or sequential vision loss caused by degeneration of the papillomacular retinal nerve fibre bundle.2 Optical coherence tomography (OCT) has demonstrated thickening of the retinal nerve fibre layer (RNFL) before and during visual decline, followed by long-term axonal degeneration.3–5 Unaffected male carriers of LHON mutations may experience mild temporal RNFL swelling.4 OCT can further detect ganglion cell complex (GCC) degeneration during disease and preceding symptom onset.
Photodynamic therapy for choroidal carcinoid metastasisCarcinoid tumours are derived from highly differentiated neuroendocrine cells that often produce biogenic amines or polypeptide hormones. They arise from various primary sites along the gastrointestinal, bronchopulmonary, and urogenital tracts and metastasize to the lymph node, liver, or bone, and only rarely to the eye and orbit. Uveal metastasis is the most common intraocular malignancy and mostly arises from breast cancer in women and lung cancer in men. Uveal metastasis from carcinoid tumours constitutes only 2.2% of all uveal metastasis.
Added value of newer optical coherence tomography technologies in hyperphosphatemic familial tumoural calcinosisIn contrast to dystrophic calcification seen in several rare genetic disorders, such as pseudoxanthoma elasticum, generalized arterial calcification of infancy, and Keutel syndrome, hyperphosphatemic familial tumoural calcinosis (HFTC) is a rare disorder of phosphate metabolism characterized by hyperphosphatemia and primary ectopic or metastatic calcifications in various locations.1
Case of Ab Interno Trabeculectomy in Juvenile Open-angle Glaucoma with 5-year Follow-upJuvenile open-angle glaucoma (JOAG) is a subset of primary open-angle glaucoma (POAG) affecting those between 3 and 40 years of age and often with a more progressive course compared to POAG.1,2 Transmission is typically autosomal dominant with mutations identified in the myocilin (MYOC) and CYP1B1 genes.2
Deep anterior lamellar keratoplasty for an intrastromal epithelial corneal cyst: a case reportCorneal cysts, usually caused by the displacement of epithelium into the substance of the cornea, were first described in 1853.1 Congenital or traumatic factors may contribute to the formation of corneal cysts.