Frozen overnight: Acute orbital apex syndrome caused by aspergillosis.

PURPOSE: to describe a rare case of orbital apex syndrome caused by aspergillosis with acute presentation. CASE DESCRIPTION: retrospective case report of a 70-year-old man who developed unilateral ophthalmoplegia overnight. He was initially given the diagnosis of suspect Tolosa-Hunt syndrome, but biopsy of the involved tissue showed aspergillosis. CONCLUSION: orbital apex syndrome caused by fungal disease is a life-threatening condition that should be promptly diagnosed and treated. It may present acutely and should not be misdiagnosed as Tolosa-Hunt syndrome. To our knowledge this is the first such case report in the English ophthalmic language Literature.

Optical Coherence Tomography Angiography in Chiasmitis.

A 29-year-old girl presented complaining of acute bilateral visual loss associated with mild headache and retrobulbar pain. She was diagnosed with chiasmal optic neuritis caused by multiple sclerosis. Her visual acuity and visual field defect promptly improved after steroid therapy. However optical coherence tomography angiography showed a progressive reduction of superficial capillary plexus density of the retina and optic nerve consistent with the progressive impairment of the retinal ganglionar cell layer. Contrary to chiasmal compression, in chiasmal optic neuritis, the superficial capillary plexus density reduction is diffuse and does not reflect the peculiar anatomy of the chiasm.

Topical Nerve Growth Factor for the Treatment of Neurotrophic Keratopathy Caused by Wallenberg Syndrome.

PURPOSE: The aim of this study was to report a case of central neurotrophic keratopathy (NK) in Wallenberg syndrome (WS) and its successful management with topical recombinant nerve growth factor (rNGF). METHODS: A 47-year-old man with WS caused by a stroke in the territory of the left vertebrobasilar artery complained of progressive visual loss in his left eye (OS). Examination showed corneal anesthesia associated with a corneal epithelial ulceration consistent with a diagnosis of NK grade 3 of central origin. Topical treatment with rNGF, 1 drop 6 times daily, was started for 8 weeks, and the patient was followed up for 1 year. RESULTS: Topical treatment with rNGF was successful in promoting complete epithelial corneal healing. No recurrence was seen at 1-year follow-up. CONCLUSIONS: Clinicians should be aware that visual loss can also occur from NK of central origin. To the best of our knowledge, this is the first case report of NK caused by WS successfully treated with rNGF reported in the literature.

Should "Retro-ocular Pain, Photophobia and Visual Acuity Loss" Be Recognised as a Distinct Entity? The ROPPVAL Syndrome.

Unilateral retro-ocular pain, photophobia and visual disturbance in patients suspected as having acute optic neuritis was described as a distinct clinical entity by Jefferis et al. in 2018. We hereby report a further four patients with the same clinical phenotype and propose the term ROPPVAL syndrome (Retro-Ocular Pain, Photophobia and Visual Acuity Loss). All of them had a previous (mis)diagnosis of optic neuritis. All of the patients had normal ocular and neurological examinations, no relative afferent pupillary defect and no objective structural abnormality was identified. We also discuss possible mechanisms, the role of cycloplegics that we found to be useful in reducing symptoms, and the importance of distinguishing this syndrome from optic neuritis.

Intraocular Tuberculosis: A Challenging Case Mimicking Wet Age-Related Macular Degeneration.

An otherwise healthy 72-year-old Chinese patient diagnosed with exudative age-related macular degeneration and decreased vision in left eye was fully investigated. The retrospective analysis of past multimodal imaging revealed bilateral severe choroidal neovascularization and choroiditis associated with a positive tuberculin skin testing and interferon-gamma release assay (QuantiFERON-TB Gold - Cellestis®, Chadstone, VIC, Australia) suggestive of latent ocular tuberculosis. The variable presentation and tests' results interpretation represent the greatest limitations in understanding and treating intraocular TB (IOTB). This may present without any other systemic symptoms, the intraocular tissues are of limited access to biopsies and other tests, including imaging and immunological tests, are of relative value. This case highlights how variable may be the presentation of IOTB, which can be easily misdiagnosed leading to a delayed treatment and worse prognosis.

Optical Coherence Tomography Angiography Findings in Optic Nerve Sheath Meningioma.

A 46-year-old female with a history of 5 months of progressive painless visual loss in the left eye was found to have an optic nerve sheath meningioma. Optical coherence tomography angiography showed a reduction in the superficial capillary plexus density consistent with her visual field defect and peripapillary retinal nerve fiber layer thinning. Moreover, abnormalities in the choriocapillaris were found in the affected eye compared to the fellow eye. Possible explanations for these findings are discussed. Further studies and a consistent number of cases are needed to correctly assess the impairment of ocular blood flow in optic nerve sheath meningioma.

Corneal Involvement in Wallenberg Syndrome: Case Report and Literature Review.

A 47-year-old man with Wallenberg syndrome after a stroke in the territory of the left vertebrobasilar artery and posterior inferior cerebellar artery, presented a week later complaining of visual loss in the left eye. Examination showed corneal anaesthesia associated with a neurotrophic corneal epithelial defect. Corneal involvement secondary to trigeminal neuropathy in Wallenberg syndrome is not frequently reported and may lead to epithelial erosion and neurotrophic keratopathy. Clinicians should be aware that visual loss can occur from a neurogenic basis (e.g., Wallenberg syndrome) due to neurotrophic keratopathy. The use of cocaine, a potential cause of neurotrophic keratopathy, should also be suspected in the appropriate clinical scenario.

Interface infectious keratitis after anterior and posterior lamellar keratoplasty. Clinical features and treatment strategies. A review.

Interface infectious keratitis (IIK) is a novel corneal infection that may develop after any type of lamellar keratoplasty. Onset of infection occurs in the virtual space between the graft and the host where it may remain localised until spreading with possible risk of endophthalmitis. A literature review identified 42 cases of IIK. Thirty-one of them occurred after endothelial keratoplasty and 12 after deep anterior lamellar keratoplasty. Fungi in the form of Candida species were the most common microorganisms involved, with donor to host transmission of infection documented in the majority of cases. Donor rim cultures were useful to address the infectious microorganisms within few days after surgery. Due to the sequestered site of infection, medical treatment, using both topical and systemic antimicrobials drugs, was ineffective on halting the progression of the infection. Injection of antifungals, right at the graft-host interface, was reported successful in some cases. Spreading of the infection with development of endophthalmitis occurred in five cases after Descemet stripping automated endothelial keratoplasty with severe sight loss in three cases. Early excisional penetrating keratoplasty showed to be the treatment with the highest therapeutic efficacy, lowest rate of complications and greater visual outcomes.

A Modified One-Stage Early Correction of Blepharophimosis Syndrome Using Tutopatch Slings.

PURPOSE: To investigate the efficacy of a one-stage early correction of blepharophimosis-ptosis-epicanthus inversus syndrome (BPES), using bovine pericardium derived membrane (TUTOPATCH(®)) for the frontalis suspension. METHODS: We prospectively studied 12 eyes from 6 patients (median age 14 months) affected by BPES with severe ptosis. All patients were submitted to a one-stage early correction of ptosis (frontalis suspension with TUTOPACH(®)) and telecanthus and epicanthus inversus. Upper margin reflex distance (MRD), nasal inner intercanthal distance (IICD), horizontal fissure length (HFL), and IICD/HFL ratio were evaluated using photographs. RESULTS: The Wilcoxon signed-rank test showed a statistically significant difference between pre- and post-operative MRD, IICD, HFL, and the IICD/HFL ratio. CONCLUSION: An early TUTOPATCH-assisted frontalis suspension, together with the correction of telecanthus and epicanthus inversus, is an effective procedure for BPES cases with severe ptosis.

Intraorbital injection of Rituximab in idiopathic orbital inflammatory syndrome: case reports.

To analyze the clinical and histopathological effects of low doses of intraorbital and intralesional Rituximab (RTX) in three patients affected by idiopathic orbital inflammatory syndrome (IOIS). Three patients with IOIS were enrolled, all of whom underwent lesion biopsy to confirm the diagnosis, complete blood examinations (thyroid function tests, complete blood cell count, fasting blood glucose, liver and renal function tests, erythrocyte sedimentation rate, serum ACE, C-reactive protein, rheumatoid factor, antinuclear antibody, antineutrophil cytoplasmic antibody, serum IGg4 level tests) and magnetic resonance imaging (MRI). Patients received the planned treatment schedule, consisting of a complete cycle of intraorbital injections of RTX (MabThera(®); Roche, Basel, Switzerland, 100 mg/10 ml): 10 mg, once a week for 1 month (four injections/month), in two patients repeated. The clinical and imaging follow-ups were at an average of 17.6 months (range 14-24 months) after treatment. A post-treatment bioptic procedure was performed in one patient. All patients showed a significant MRI reduction of the orbital lesion and a stable clinical improvement for the follow-up time of observation. The post-treatment histopathological specimen showed a disappearance of inflammatory cells. Low doses of intralesional RTX, which are safe, efficacious and used in other B cell-mediated disorders, are a useful treatment in IOIS, with decreased risks of generalized immunosuppression and fewer side effects than are afforded by systemic high doses of glucocorticoids and RTX. The result is very quick, effective and prolonged on the inflammatory component of the disease and seems to be related to the histologic reduction of infiltrating CD20+ lymphocytes.

Intraorbital injection of rituximab versus high dose of systemic glucocorticoids in the treatment of thyroid-associated orbitopathy.

The aim of the study was to compare, in a randomized prospective study, the efficacy and safety of intraorbital administration of low doses of RTX versus intravenous glucocorticoids (GCs) to treat patients affected by moderately severe thyroid-associated active orbitopathy. Twenty patients with active, moderately severe TAO, whose mean age was 56.7 years±10.2 SD participated in the study. Patients were randomly selected and treated with intraorbital injections of RTX or with i.v. GCs. Disease activity and severity were assessed by the Clinical Activity Score (CAS) and the NOSPECS. Computed tomography or magnetic resonance scans were performed in all patients. In the RTX group, full blood cell count and flow cytometric analysis on peripheral blood lymphocytes were done. The patients were followed for 20 months. In both groups, CAS and NOSPECS indexes were significantly reduced (p<0.005). In particular, CAS reduction was evident since the first follow-up with both treatments. Proptosis decreased significantly only in group B and diplopia showed no significant changes during follow-up times in both groups. Neither of the treatments affected the peripheral TRab. In group A, 5 weeks after the first injection, the CD20+ peripheral lymphocytes value was nearly zero. One patient treated with rituximab progressed to severe TAO (optic neuropathy) following the second injection so the treatment was discontinued. The data confirm the therapeutic efficacy of RTX in active TAO, even in low doses and locally administered. The efficacy on the inflammatory component of the disease is comparable to that of steroids and seems to be related with the reduction of peripheral CD20+ lymphocytes. Caution should be given to an accurate patient selection.

Femtosecond laser-assisted lamellar keratoplasty: early results.

PURPOSE: To evaluate the outcomes and safety of lamellar keratoplasty (LK) assisted by a femtosecond laser. METHODS: Twenty-one eyes of 21 patients affected by different corneal pathologies (5 posttraumatic corneal scar, 3 postkeratitis corneal leucoma, and 13 keratoconus) underwent LK procedures by using a femtosecond laser. The mean thinnest corneal thickness, evaluated with ultrasound corneal pachymetry and with confocal microscopy, was 434.19 +/- 62.60 (SD) microm (range, 333-548 microm). Mean preoperative uncorrected visual acuity was 0.09 +/- 0.28 SD and mean preoperative best spectacle-corrected visual acuity was 0.28 +/- 0.15 SD. A femtosecond laser was used to perform corneal cuts on both donor and recipient corneas. The donor corneal lamella diameters were 0.20-mm larger and thicker than the recipient to restore a physiologic corneal thickness and shape: mean donor diameter was 8.34 +/- 0.28 mm (range, 8.2-8.7 mm) and mean thickness was 352 +/- 40.27 microm (range, 220-400 microm). Mean follow-up was 20.86 +/- 5.76 months (range, 12-30 months). RESULTS: Early postoperative evaluation showed a clear graft in all cases. A normal corneal pattern topography and a physiologic thickness (mean corneal pachymetry, 542.48 +/- 33.20 microm) and transparency were restored. Twelve months after surgery, the mean postoperative uncorrected visual acuity was 0.45 +/- 0.34 SD, and the mean best spectacle-corrected visual acuity was 0.63 +/- 0.16 SD. CONCLUSIONS: Although the numbers in our study are small, our early results indicate that femtosecond laser-assisted lamellar keratoplasty shows promise as a safe and effective surgical choice in the treatment of various corneal pathologies.