Anaplastic spheno-orbital meningioma: rapid growth after extensive exenterationSpheno-orbital meningiomas (SOMs) arise from the sphenoid bone and extend into the orbit. These tumours account for approximately 2% of all orbital lesions.1 Presenting symptoms typically include proptosis, diplopia, or optic neuropathy. Most SOMs are CNS World Health Organization (WHO) grade 1 lesions. These are generally considered benign and slow growing. A small proportion of SOMs consists of CNS WHO grade 2 lesions, whereas CNS WHO grade 3 SOMs are very rarely described. These are often called malignant meningiomas.
Explantation of iris-sutured intraocular lensWe describe a simple technique for explantation of iris-sutured intraocular lens that aims to reduce the risk of posterior dislocation of the implant at the time of surgery and minimize iatrogenic iris trauma. This technique involves bimanual rotation of the intraocular lens haptic out of the iris stitch.
Primitive myxoid mesenchymal tumours of infancy: first case surrounding the optic nervePrimitive myxoid mesenchymal tumour of infancy is a rare form of sarcoma presenting in infancy.1 It was first described by Alaggio et al.2 in 2006 as a primitive mesenchymal soft tissue sarcoma without any distinctive lineage of differentiation. The tumours described initially occurred on the trunk, head, neck, extremities, chest, scalp, abdomen, back, or limbs.2,3 The tumours initially may be diagnosed as and placed under the broad umbrella of sarcomas, but immunohistochemical and genetic analyses have now categorized the tumour as a definite entity.
Ciliochoroidal effusion syndrome associated with mebendazole systemic treatmentMebendazole (Vermox; Johnson & Johnson Inc, New Brunswick, NJ) is an effective antiparasite drug used for the treatment of Oxyurus vermicularis infections (oxyuriasis).1 Although theoretically considered as a well-tolerated drug with few adverse effects, we report a unique case of transient ciliochoroidal effusion that occurred in temporal association with mebendazole therapy.
Updated review: optical coherence tomography findings of the pachychoroid disease spectrumThe ocular choroid consists of 5 distinct layers: innermost Bruch's membrane, capillaries of the choriocapillaris, Sattler's layer housing medium-sized blood vessels, Haller's layer containing larger-sized vessels, and the suprachoroidal space. Pachychoroid disease spectrum (PDS) was first described by Warrow et al.1 in 2013 as a group of chorioretinal disease entities characterized by a thickened choroid exceeding 300 µm and leading to exudative and neovascular complications. However, improved multimodal imaging, including extended depth imaging optical coherence tomography (EDI-OCT), has helped characterize PDS as a spectrum of ocular phenotypes all sharing 3 common characteristics: engorged Haller's layer vessels, Sattler's layer compression, and choriocapillaris attenuation.
Allergic conjunctivitis and contact dermatitis following silicone tube intubationLacrimal stenting systems have been used to maintain patency of the canaliculi after insults such as surgery, trauma, radiation, and chemotherapy. The first nasolacrimal stents were made of silver wire and used by Graue in 1932.1 Since then, other materials have been used, including silk, nylon, polyethylene, and polypropylene. Modern-day stents are most commonly made of silicone because this material is pliable, affordable, and widely available. Previously thought to be inert, there is evidence that silicone can elicit a foreign-body and inflammatory reaction.
Laser-induced maculopathy after iris depigmentation cosmetic treatmentA cosmetic procedure that changes the colour of the eyes using laser therapy against iris melanosomes is being performed in several countries around the world. Some ophthalmologists have expressed concern about this procedure potentially causing glaucoma or uveitis.1 Exposure to laser radiation also may cause visual impairment by retinal injury, sometimes resulting in irreversible damage to the patient.2 We present the first case of a light-induced maculopathy in a patient who underwent an iris depigmentation laser treatment for electively changing the colour of her eyes.
Audible blink in carotid-cavernous fistulaA carotid-cavernous fistula (CCF) is an abnormal arteriovenous communication between the carotid artery system and the cavernous sinus. Common ocular manifestations of CCFs include pulsating exophthalmos, conjunctival chemosis, periorbital bruit, diplopia, and ophthalmoplegia.1 We report a patient with atypical CCF who presented with audible blinks and describe the possible pathogenesis of this phenomenon.
Ocular findings of oculomotor apraxia/ataxia type 1Early-onset ataxia with oculomotor apraxia or oculomotor apraxia/ataxia type 1 (AOA-1) is a recessive progressive neurodegenerative disease that is clinically characterized by progressive diffuse ataxia, dysarthria, hand and head tremors, chorea, and dystonia.1 The onset may occur as growth retardation primarily between the ages of 1 and 20 years. Cognitive impairment, mental retardation, peripheral axonal neuropathy, distal atrophy, superficial and deep sensory insufficiency, and hypo-/areflexia are seen in the progression of the disease along with movement disorders.
Painful vision loss in Leber hereditary optic neuropathy with novel ND1 variant mimicking optic neuritisMore than 90% of Leber hereditary optic neuropathy (LHON) cases are caused by 1 of 3 primary mitochondrial DNA mutations.1 They are MT-ND4 (m.11778G>A, p.Arg340His), which accounts for ∼70% of all cases; MT-ND6 (m.14484T>C, p.Met64Val); and MT-ND1 (m.3460G>A, p.Ala52Thr) found in genes coding protein subunits of NADH dehydrogenase (complex I) of the electron transport chain, resulting in defective energy production.2 Most patients with LHON presents with sudden unilateral painless vision loss followed by fellow-eye involvement within weeks to months.
Determination of the size of the stripping area using trypan blue in Descemet's stripping onlyIn recent years, corneal endothelial transplantation has become a standard procedure in the surgical management of Fuchs endothelial corneal dystrophy (FECD).1 Despite the recent development of effective surgical techniques, corneal endothelial transplantation is still associated with many complications, including transplant rejection, graft dysfunction, postoperative complications, and the scarcity of donor grafts.
Follicular lymphoma isolated to the superior oblique muscleA 76-year-old female presented with bilateral peripheral visual field constriction. She had a past medical history of left common iliac vein stenting secondary to compression from the iliac artery, left lower extremity deep vein thrombosis requiring angioplasty, bilateral lower extremity lipodermatosclerosis, hyperlipidemia, vitamin D deficiency, diverticulitis, and generalized anxiety disorder. Her regular medications included apixaban, clopidogrel, venlafaxine, and montelukast. She denied smoking or alcohol use.
Surgical management of a recurrent hereditary benign intraepithelial dyskeratosis lesion involving a Boston keratoprosthesisThe Boston keratoprosthesis (Kpro) can provide vision in high-risk keratoplasty eyes (i.e., neovascularization, limbal stem cell deficiency [LCSD]), where normal keratoplasty invokes a high likelihood of failure. Because of increased risks of melts, keratitis, and endophthalmitis, Kpro eyes need close monitoring with close inspection of the front plate optical stem–keratoplasty junction. One disease entity in which management via Kpro has not been described previously is hereditary benign intraepithelial dyskeratosis (HBID).
Double anterior chamber following deep anterior lamellar keratoplasty with endothelium-on donor tissueDeep anterior lamellar keratoplasty (DALK) is a well-established technique for corneal transplantation because of its lower risk of postoperative graft rejection than penetrating keratoplasty.1 One possible complication of DALK is the formation of a double anterior chamber (AC). During surgery, a microperforation of the Descemet's membrane (DM) may create a channel that allows aqueous fluid to enter the host–donor interface, forming a pseudo–anterior chamber. Double ACs are typically observed in the immediate postoperative period.
Acute suppurative bacterial dacryoadenitis with abscess formation in a childAcute suppurative bacterial dacryoadenitis (ASBD) is an uncommon ophthalmic condition that is rare in the pediatric population. Rarely, ASBD may progress to the formation of a lacrimal gland abscess that, in the absence of timely and effective management, poses a risk of ophthalmic and neurologic complications. This typically involves systemic antibiotics, and particularly in paediatric cases, definitive management may involve surgical incision and drainage.
Metastasis of endometrial carcinoma to the ocular adnexaA 61-year-old female was referred by her oncologist for a rapidly enlarging, painful erythematous mass in the region of the left medial canthus, first noticed 1 month prior. She reported no change in vision, no epiphora, and no discharge from the eye. She had been using warm compresses and gentle massage to no effect. She had no past ocular history. Her past medical history was notable for metastatic endometrial carcinoma with metastases to lung and femur, for which she had completed numerous rounds of chemotherapy and radiation as well as total abdominal hysterectomy with bilateral salpingo-oophrectomy years prior.
Bilateral primary squamous cell carcinoma of the conjunctivaSquamous cell carcinomas (SCCs) of the conjunctiva are rare, with an estimated incidence of 1–2.8 per 100 000 people per year.1 SCC is the most frequently occurring malignant epithelial tumour of the conjunctiva.2 Here we present a case of a patient presenting with 2 distinct primary carcinomas of the conjunctivae over a 3-year period.
Retro-orbital alveolar soft-part sarcoma in a 76-year-old female: case report and review of the literatureAlveolar 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.1
Sebaceous cell carcinoma presenting as ocular Marjolin ulcer following immunosuppression for a chemical burnMarjolin 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 outcomesPseudotumour 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 NAIONNonarteritic 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 drusenSoft 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 cenegerminCenegermin 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).3
Symptomatic iris varix with enlargement following argon laserIris 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 glaucomaOcular 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.