Atypical presentation of bilateral Epstein-Barr virus dacryoadenitis: a case report of corticosteroid resistant orbital inflammation.

Epstein-Barr virus is a known cause of dacryoadenitis that is typically sensitive to corticosteroid treatment. When affecting the orbit, particularly the lacrimal gland, Epstein-Barr virus may cause chronic proptosis and a bilateral lacrimal mass effect. We provide a case of bilateral Epstein-Barr virus associated dacryoadenitis initially resistant to corticosteroid treatment requiring biopsy and confirmation by polymerase chain reaction of lacrimal tissue. Herein, we discuss the presentation with associated magnetic resonance imaging and histopathologic images, diagnostic dilemma, and treatment of an atypical case.

Orbital extranodal marginal zone lymphoma arising during pregnancy: a case report.

A 37-year-old, previously healthy woman presented during her first trimester of pregnancy with a two-week history of rapidly progressive proptosis in the left eye. Clinical examination revealed limited left supraduction and diplopia in upward gaze. Orbital magnetic resonance imaging showed a medial orbital mass adjacent to the globe with secondary proptosis. Pathologic examination of a biopsied specimen of the orbital mass and subsequent immunophenotyping by flow cytometry revealed an extranodal marginal zone B-cell lymphoma. Clinical and histological features as well as a review of the literature are described.

Neurotrophic keratopathy: current challenges and future prospects.

Neurotrophic keratopathy (NK), or neurotrophic keratitis, is a degenerative condition that results from decreased innervation to the cornea. The cornea is innervated by the ophthalmic branch of the trigeminal nerve. Neurotrophic keratopathy is most commonly caused by herpes keratitis however, any condition that disrupts the normal corneal innervation can cause NK. Neurotrophic keratopathy is a clinical diagnosis and is classified into three stages based on the disease severity. Stage 1 has mild epithelial defects, such as punctate keratopathy, stage 2 disease has persistent epithelial defects, and stage 3 is defined by the presence of ulcers. Current treatment modalities consist of medical and surgical options. Stage 1 is treated with lubrication through artificial tears, eyelid taping, and punctal plug/cautery. Stage 2 treatment can involve therapeutic contact lenses, topical autologous or allogenic serum, tarsorrhaphy, botulinum toxin injections, and possibly anti-inflammatory medications. Stage 3 disease may require human nerve growth factor, amniotic membrane transplantation, conjunctival flap, or corneal neurotization. New therapies, such as matrix regenerating therapy, plasma rich in growth factors, Thymosin ß4, Substance P/Insulin like growth factor-1, and nicergoline represent exciting future options.KEY MESSAGESNeurotrophic keratopathy is a rare degenerative disease defined by decreased innervation to the cornea that is associated with significant morbidity.Treatment options range from lubrication alone to various medical and surgical treatments.Matrix regenerating therapy, plasma rich in growth factors, Thymosin ß4, Substance P/Insulin like growth factor-1, and nicergoline are exciting novel therapies that will influence how neurotrophic keratopathy is treated in the future.

Outcomes of corneal neurotisation using processed nerve allografts: a multicentre case series.

BACKGROUND: Corneal neurotisation is a rapidly evolving procedure treating neurotrophic keratopathy. The variety of surgical techniques used and corresponding outcomes after corneal neurotisation are not well understood. This study describes the techniques and outcomes in the largest case series of corneal neurotisation using processed nerve allografts to date. METHODS: This is a retrospective case series of patients who underwent corneal neurotisation with human cadaveric processed nerve allografts. All patients had preoperative and postoperative description of best corrected visual acuity and measurement of corneal sensation. Comparative studies after stratification of techniques were performed. RESULTS: A total of 17 patients were identified. The cause of corneal anaesthesia was prior infection in eight cases, trigeminal nerve palsy in eight cases and ocular trauma in one case. There were no intraoperative or postoperative complications. Following neurotisation surgery, the time to first gain of corneal sensation and maximal gain of sensation occurred at a mean of 3.7 months (range 1-8 months) and 6.6 months (range 3-15 months), respectively. The mean preoperative and postoperative corneal sensation as measured by Cochet-Bonnet aesthesiometry was 0.36 cm (range 0-3.2 cm) and 4.42 cm (range 0-6 cm), respectively (p<0.01). Visual acuity was unchanged after neurotisation. There were no statistical differences in outcomes based on end-to-end versus end-to-side coaptations, donor nerve selection or laterality of donor nerve. CONCLUSION: Corneal neurotisation with processed nerve allografts is a safe and effective procedure. This study provides further evidence for the use of processed nerve allografts for corneal neurotisation.

Ophthalmic changes in patients with hemifacial atrophy (Parry-Romberg syndrome).

PURPOSE: Parry-Romberg syndrome (PRS) is a rare condition characterized by progressive, unilateral facial atrophy. We hypothesize that patients with this condition may have involvement of the ocular structures. Here, we report our ophthalmic, clinical and anatomical findings in an observational study of six patients with long-standing PRS. METHODS: Patients diagnosed with PRS were invited to participate in a clinic visit during which the following tests were administered and data recorded: best-corrected vision, refractive error, Ishihara color plates, Hertel exophthalmometry, gonioscopy, complete slit-lamp and dilated fundus examination, Intra Ocular Lens Master measurements and keratometry. Two-sample T tests were used to compare data between affected and unaffected eyes, as well as affected eyes and a normative population. RESULTS: Six patients underwent complete eye examinations. The mean spherical equivalent of the affected eye was + 3.83 D, while that of the unaffected eye was + 0.13 D. The atrophic hemiface averaged 2.8 mm of enophthalmos on Hertel exophthalmometry. The axial length of the atrophic eye was 0.91 mm shorter than the unaffected eye. Compared to normative data, in patients with PRS, the difference between eyes was statistically different for each of the following variables: visual acuity, spherical equivalent, corneal diameter, axial length and flat and steep keratometry. CONCLUSIONS: This is one of the first quantitative, exploratory studies with ophthalmic measurements in patients with PRS. Our results suggest the globe may demonstrate atrophic changes similar to other soft tissues in the face known to be affected by this condition.

Posteriorly Displaced Orbital Implant Causing Intractable Anophthalmic Socket Pain.

Anophthalmic socket pain is a rare complication of enucleation. The authors present a patient presenting with intractable anophthalmic socket pain due to a posteriorly displaced orbital implant. The patient's pain localized to the V1 and V2 orbitofacial dermatomes, and we suspect compression of the frontal and zygomatic branches of the ophthalmic and maxillary nerves, respectively, as the underlying etiology of the patient's pain. Removal of the implant and placement of a dermis fat graft was effective at alleviating the patient's symptoms.

Topical Corticosteroid Use Associated With Increased Degree of Ptosis and Rate of Ptosis Repair Failure.

PURPOSE: To compare the degree of ptosis and the risk of ptosis repair failure among patients with and without a history of topical corticosteroid use. METHODS: Retrospective, case-controlled study examining topical corticosteroid use among adults with ptosis who underwent external levator advancement/resection (ELR) or Müller muscle conjunctival resection with at least 3 months postoperative follow-up. Comparative statistical analyses of surgical outcomes were performed amongst patients with and without history of topical corticosteroid use. RESULTS: A total of 240 patients (406 eyelids) met study criteria, of which 36 patients (44 eyelids) had history of topical corticosteroid use. Mean preoperative margin reflex distance was 0.20 mm and 0.58 mm for topical corticosteroid and non-corticosteroids users (p = 0.01). Mean preoperative levator function was 9.78 mm and 10.38 mm for topical corticosteroid and non-corticosteroid users (p = 0.02). The rate of ptosis repair failure was 30% and 16% in patients with and without a history of topical corticosteroid use (odds ratio 2.25, 95% confidence interval 1.10-4.55; p = 0.03). The rate of recurrence per surgical type in eyelids with and without history of topical corticosteroid use was: external levator advancement/resection 11/27 (41%) and 48/266 (18%) (odds ratio = 3.12, confidence interval 1.36-7.15 0; p = 0.01); Müller muscle conjunctival resection 2/17 (12%) and 9/96 (9%) (odds ratio 1.29, confidence interval 0.25-6.56; p = 0.76). CONCLUSIONS: Topical corticosteroid use is associated with more severe presenting ptosis and increased rates of ptosis repair failure. Compared to Müller muscle conjunctival resection, there is a significantly higher rate of ptosis repair failure in patients undergoing external levator advancement/resection.

Polytetrafluoroethylene frontalis suspension in blepharospasm with eyelid apraxia: an effective and well-tolerated adjunct to botulinum toxin therapy.

Purpose: To report the efficacy of polytetrafluoroethylene (PTFE) frontalis suspension for blepharospasm with eyelid apraxia and postoperative botulinum toxin requirements. Methods: Retrospective chart review of patients with blepharospasm and eyelid apraxia who underwent frontalis suspension. The primary outcome was a surgical success, defined by surgeon- and patient-reported success in postoperative eyelid opening. Comparative statistical analyses of botulinum toxin dosage and treatment intervals were performed amongst patients before and after frontalis suspension ptosis repair. Results: Five patients (10 eyelids) met the study criteria, of which 40% were female. Mean age was 63.2 years. All patients had successful surgical outcomes based on physician-reported and patient reported satisfaction with the postoperative eyelid opening. Average follow-up was 14 months. Preoperative botulinum toxin treatments averaged 80.4 units (range 32-110, SD 33.2) to the periocular region over an average of 9.6-week intervals. Postoperative botulinum toxin treatments averaged 61.4 units (range 24-110, SD 34.7) to the periocular region over an overage of 9.8-week intervals. No patients experienced postoperative exposure keratopathy, extrusion of the sling, or postoperative infection. Conclusions: Frontalis suspension using PTFE suture in the setting of blepharospasm with eyelid apraxia was found to be a safe and effective procedure. Frontalis suspension in this population is not a substitute for botulinum toxin treatment but may allow for reduced treatment dosage. Frontalis suspension appears to increase patient functionality with improved eyelid opening in patients with blepharospasm with eyelid apraxia.

Primary Lacrimal Gland Oncocytoma Associated With Hypervascularity: A Case Report With Imaging and Immunohistochemical Characterization.

Oncocytoma is a rare benign neoplasm with few cases reported to involve the orbit. When affecting the orbit, oncocytomas have very slow growth and often evade early diagnosis. The diagnostic evaluation of these orbital tumors is not fully understood. The authors present a case of a benign oncocytoma arising in the lacrimal gland associated with significant vascularity and intraoperative bleeding. The authors provide immunohistochemical analysis of this rare orbital tumor and insights in surgical planning.

Rapidly Enlarging Hidradenoma of the Eyelid.

Hidradenoma is a rare benign neoplasm, with few cases reported to involve the eyelid. When affecting the eyelid, hidradenomas may mimic other benign or malignant lesions. Rarely, a benign hidradenoma may transform into a malignant hidradenoma and metastasize. The authors present a case of a benign hidradenoma arising from the eyelid, presenting with rapid growth, ulceration, and bleeding, suggestive of a malignant lesion.

Outcomes After Müller Muscle Conjunctival Resection Versus External Levator Advancement in Severe Involutional Blepharoptosis.

PURPOSE: To compare outcomes between Müller muscle conjunctival resection (MMCR) ptosis repair and external levator resection (ELR) in patients with severe involutional blepharoptosis. DESIGN: Retrospective, interventional, comparative case series. METHODS: A retrospective review was performed of patients who underwent ptosis repair between 2012 and 2019. Inclusion criteria were patients who underwent MMCR or ELR ptosis repair, patients with complete documentation of preoperative eyelid measurements, and patients with documentation of postoperative outcome. The main outcome measure was surgical failure, defined as patient-reported or physician-reported dissatisfaction with postoperative eyelid height or postoperative upper margin reflex distance (MRD1) of less than 2 mm. Severe ptosis was described as an MRD1 of 0 or worse. Outcome analysis was also performed after stratification for concomitant blepharoplasty performed at the time of ptosis repair. RESULTS: A total of 231 patients (372 eyelids) met the study criteria, of which 142 eyelids had severe ptosis. Comparing outcomes of MMCR vs ELR in patients with severe ptosis, there was a statistically significant higher rate of success after MMCR (P = .0143). The rate of ptosis repair success in eyelids that underwent MMCR was 97.2% and 90.9% in patients with severe ptosis and mild/moderate ptosis, respectively (P = .42). In eyelids that underwent ELR, the rate of ptosis repair success was 77.4% and 85% in eyelids with severe ptosis and mild/moderate ptosis, respectively (P = .15). Concomitant blepharoplasty did not affect ptosis repair outcomes in any group. CONCLUSIONS: MMCR ptosis repair is an effective approach in treating patients with severe ptosis, and it may offer superior outcomes to ELR. In patients with good responses to phenylephrine, MMCR may offer an efficient and highly efficacious surgery regardless of presenting MRD1.