Correspondence
Retro-orbital alveolar soft-part sarcoma in a 76-year-old female: case report and review of the literature
Alveolar soft part sarcoma (ASPS) is a rare mesenchymal neoplasm that represents less than 1% of all sarcomas.1 Orbital ASPS is extremely rare, representing only 5%–15% of all cases of ASPS.2 The average age at presentation of orbital ASPS is 13.5–17.8 years. A recent review of the literature suggests there are fewer than 70 documented cases of primary orbital ASPS.1Sebaceous cell carcinoma presenting as ocular Marjolin ulcer following immunosuppression for a chemical burn
Marjolin ulcer is a term used to describe malignancy that originates from chronically inflamed, ulcerated, or scarred tissue.1 The potential for malignant transformation of ocular surface chemical injuries to a Marjolin ulcer is not well recognized.2 In fact, this progression may be accelerated by the immunosuppressive treatments often used to control the postburn cicatrizing process. This report presents an ocular Marjolin ulcer following an alkali burn that was diagnosed histopathologically as sebaceous carcinoma.Secondary pseudotumour cerebri syndrome in children: clinical characteristics and long-term outcomes
Pseudotumour cerebri syndrome (PTSC) is characterized by elevated intracranial pressure (ICP) with the absence of an intracranial mass, hydrocephalus, or abnormalities in cerebrospinal fluid composition. PTSC can be primary (also known as idiopathic intracranial hypertension) or secondary. We recently published an article summarizing the clinical characteristics and visual outcomes for primary PTSC in children.1 The purpose of this correspondence is to report the clinical characteristics and long-term outcomes in a concomitant cohort of children with secondary PTSC from the same institution over the same time period.Evidence of nocturnal hypotension in an inpatient prior to an episode of classic NAION
Nonarteritic anterior ischemic optic neuropathy (NAION) is a known cause of acute, painless, monocular vision loss in patients with small cup-to-disc ratios and at least 1 vascular risk factor.1 The etiology has been debated, although Hayreh et al.2 have hypothesized that nocturnal hypotension impairs perfusion to the optic disc causing ischemia to the optic nerve. Despite evidence supporting this via retrospective monitoring of diurnal changes in blood pressure (BP) with Holter monitoring in patients with prior outpatient episodes of NAION, this BP change has not been documented at the time of the event.Preserved retinal sensitivity following spontaneous regression of soft drusen
Soft drusen are the clinical hallmark of intermediate age-related macular degeneration (AMD) and a major risk factor for late-stage disease. Soft drusen usually increase in size, area, and confluence with aging.1 Their natural life cycle may involve retinal pigment epithelium (RPE) changes, collapse, and macular atrophy or spontaneous regression.2 Pharmacologic and laser photocoagulation treatments intended to induce drusen regression have yet to show visual benefit.1,3 Early treatment of intermediate AMD before progression to macular atrophy and/or neovascular complications is an area undergoing intense study and would be a breakthrough.Late-onset diffuse lamellar keratitis after treatment with cenegermin
Cenegermin 0.002% ophthalmic solution (Oxervate, Dompe, Boston, Mass.) is a recombinant human nerve growth factor (NGF) that has been a promising novel topical treatment dosed 6 times daily for 2 months to treat neurotrophic keratitis (NK).1,2 NK is a degenerative ocular disease characterized by reduced or absent corneal sensation. One iatrogenic etiology of NK, for which cenegermin has garnered interest, is after laser in situ keratomileusis (LASIK).3Symptomatic iris varix with enlargement following argon laser
Iris varix is a rare iris lesion that may be mistaken for a malignant neoplasm.1 Thirty-six cases have been described since 1975, most treated by resection without recurrence, although spontaneous regression has been reported.2 Most varices have been described as large, lobulated, well-circumscribed black or red lesions of the iris that may be associated with spontaneous hemorrhage, although smaller varices have been described as prominent, elongated, and tortuous vessels.2,3 Although benign, iris varices may be of sufficiently suspicious appearance as to warrant a detailed work-up to exclude other etiologies such as hemangiomas or melanomas.Intraocular pressure fluctuations in a professional woodwind musician with advanced glaucoma
Ocular hypertension and fluctuations in intraocular pressure (IOP) are critical modifiable risk factors for the development and progression of glaucoma. Transient IOP elevations while playing wind instruments have been observed in musicians with1 and without2,3 glaucoma. However, the comparative effect of trabeculectomy surgery on IOP fluctuations during wind instrument performance has not been previously described.Ischemic cranial nerve 6 palsy and lipemia retinalis in a patient with hypertriglyceridemia
Severe hypertriglyceridemia can occur as a primary (genetic) disorder or secondary to diabetes mellitus, hypothyroidism, alcohol use, or medications. Ophthalmic signs of hypertriglyceridemia include xanthomas, xanthelasmas, and corneal arcus.1 However, atypical presentations also can occur. Prior case reports describe presenting symptoms of intermittent vision loss due to associated hypertensive retinopathy or blurry vision from secondary central serous chorioretinopathy or branch retinal vein occlusion.Superior limbic keratoconjunctivitis following ptosis repair
Superior limbic keratoconjunctivitis (SLK) is an inflammatory condition of the superior bulbar conjunctiva, tarsal conjunctiva, and upper cornea of unknown etiology. The pathogenesis of SLK is unclear, although it has been linked to mechanical microtrauma, deficiencies in tear film quality, and immunologic dysregulation.1Meningococcal B vaccine-associated papillophlebitis and cilioretinal artery occlusion
Vaccine-induced optic neuritis is a known subtype of optic neuritis that has been reported following the administration of various types of vaccines, both viral and bacterial. Ocular symptoms vary significantly and can result in permanent vision loss. There has been only one case of optic neuritis following meningococcal B vaccine (Bexsero; GlaxoSmithKline, London, UK) administration that was reported on the Vaccine Adverse Event Reporting System (2016, VAERS ID 686394), but the details of the event are not thoroughly explained.Marjorin ulcer (squamous cell carcinoma) in a temporal artery biopsy wound
It is well recognized that postoperative and traumatic wounds and scars are susceptible to malignant degeneration, most commonly to squamous cell carcinoma (SCC).1 This phenomenon can occur months to decades after the inciting trauma.1 We describe a case arising from a temporal artery biopsy (TAB) site, demonstrating the importance of considering skin malignancy in any nonhealing incision. Informed consent for publication of this patient's case was obtained, adhering to the Declaration of Helsinki principles.Ultra-wide-field retinal imaging in tetralogy of Fallot before and after cardiac surgery
Congenital heart disease can be classified physiologically into 3 categories: volume overload, cyanotic, and obstructive. Cyanotic congenital heart disease (cCHD) includes lesions with right-to-left shunts or mixing abnormalities (e.g., transposition of the great vessels, persistent truncus arteriosus, tetralogy of Fallot).1 Tetralogy of Fallot (ToF) encompasses a variety of anatomic abnormalities, including a large and unrestrictive ventricular septal defect, right ventricular outflow tract obstruction, overriding of the aorta, and right ventricular hypertrophy.Traumatic direct carotid cavernous fistula following partial maxillectomy
Carotid cavernous fistula (CCF) is a rare traumatic complication that can occur following iatrogenic interventions.1 We report a case of direct CCF (type A) developing following partial maxillectomy with the Le Fort I approach. This case report adheres to the ethical principles outlined in the Declaration of Helsinki as amended in 2013.Acute retinal necrosis from herpes simplex virus type 2: a case series
Acute retinal necrosis (ARN) is a potentially blinding condition characterized by rapidly progressive peripheral retinal necrosis with occlusive vasculopathy and prominent inflammatory reaction.1,2 Identification of a viral etiology can be done with polymerase chain reaction (PCR) testing of intraocular fluid, and prompt management is necessary to preserve vision. ARN caused by herpes simplex virus 2 (HSV-2) is rare, and our understanding is based on a few small case series.3 Here we present three cases of HSV-2 ARN in immunocompetent hosts with a prolonged clinical course of systemic and intravitreal antivirals.Delayed-onset abducens nerve palsy following parafalcine meningioma complicated by subdural hematoma
Meningiomas are slow-growing, benign brain tumors accounting for approximately 20% of primary intracranial masses.1 They may remain asymptomatic for long periods of time and can present with headaches, seizures, and other more site-specific symptoms. Although they are highly vascular structures, meningiomas do not typically produce subdural hematoma (SDH).2 Only 38 cases of meningioma associated with acute SDH have been reported in the English literature.1 We report a delayed-onset abducens nerve palsy due to an SDH associated with a parafalcine meningioma.Migratory orbital inflammation in rheumatoid arthritis
Orbital inflammatory disease is associated with several etiologies.1 Ocular manifestations of rheumatoid arthritis (RA), such as keratoconjunctivitis sicca, have previously been noted in the literature;2,3 however, migratory orbital inflammation is rare.Use of ripasudil for rapid resolution of acute hydrops in keratoconus
Acute corneal hydrops (CH) is characterized by marked stromal edema from the influx of aqueous humour through a tear in Descemet membrane.1 It is predominantly seen in patients with corneal ectasia and reported in up to 3% of patients with keratoconus.1,2 CH presents with an acute decrease in visual acuity, photophobia, and pain.1 Conservative management of CH includes topical hypertonic saline solution, cycloplegics, topical corticosteroids, topical antibiotics, and a bandage soft contact lens if needed for comfort.Unilateral vision loss in a patient with essential thrombocythemia
Essential thrombocythemia is a chronic myeloproliferative neoplasm that develops when megakaryocytes in the bone marrow produce an excess number of platelets.1 This chronic hematologic disorder is associated with a number of thrombotic and embolic complications, and prior cases of vision loss have been documented.1–6 We describe a rather unique case of essential thrombocythemia presenting with unilateral vision loss in a patient.Cotton-wool spots in patients with migraine
Cotton-wool spots (CWSs) refer to localized accumulations of axoplasmic debris in the retinal nerve fibre layer that result from interruption of ganglion cell axon organelle transport.1 The histologic hallmark is the cytoid body, which represents the terminal swelling of a disrupted ganglion cell axon that has expanded and accumulated mitochondria and other organelles and subcellular material.1 In an otherwise healthy person, even a single CWS is considered abnormal, and it is recommended that investigations for underlying systemic disease be initiated.Treatment of an orbital pseudomeningocele through an eyelid incision
Pseudomeningoceles (PMs) occur from extravasation of cerebrospinal fluid (CSF) into soft tissue secondary to a dural tear.1–3 Unlike a true meningocele, which is lined by arachnoid tissue, PMs are associated with the formation of a fibrous capsule.2 Although PMs can form due to surgical insults to the dural covering of the brain or spine, they can also occur secondary to trauma or congenital abnormalities.1,2 Clinical symptoms usually arise when there is mass effect on the surrounding structures.Experiences from a national webinar with recently matched Canadian ophthalmology residents for medical students
Canadian medical students interested in ophthalmology must participate in the Canadian Residency Matching Service (CaRMS) R-1 match,1 where they are assessed based on their academic and extracurricular profile (such as medical school performance record, custom résumés, volunteerism, leadership, research, reference letters, personal letters, and interviews) before they enter residency training. The most recent residency application cycle, completed in April 2021, brought medical students unique challenges secondary to the coronavirus disease 2019 (COVID-19) pandemic, such as canceled visiting electives, virtual interviews, limited clinical exposure, and limited in-person interaction with program faculty and residents.Use of topical allogenic fresh-frozen plasma drops in the treatment of ligneous conjunctivitis
Ligneous conjunctivitis is a rare inflammatory condition characterized by chronic and recurrent deposition of fibrin-rich pseudomembranes on the palpebral conjunctiva.1 Firm membranes with a characteristic ‘woody’ induration usually occur bilaterally presenting since childhood.1,2 The formation of fibrinous pseudomembranes is caused by a mutation in the PLG gene, leading to deficient levels of type 1 plasminogen.1 Plasminogen is a precursor of plasmin, a molecule involved in the degradation of fibrin.Varicella-zoster virus-induced orbital apex syndrome with superior ophthalmic vein occlusion and malignant intraocular pressure rise
Orbital apex syndrome from varicella-zoster virus (VZV) is extremely rare, and approximately 20 patients worldwide have been reported with this condition.1 We report a patient with VZV-associated orbital apex syndrome and unusual malignant intraocular pressure rise from superior ophthalmic vein (SOV) thrombosis.Intravenous tocilizumab in the treatment of resistant optic perineuritis
Optic perineuritis (OPN) is a rare orbital inflammatory disease that mainly involves the optic nerve sheath and surrounding tissues. It is accompanied by eye pain and various levels of optic nerve dysfunction, such as decreased vision, visual field defect, and positive afferent pupillary defect. In contrast to optic neuritis, OPN is not a self-limited disease and always requires treatment.1
