Peripapillary Retinal Haemorrhages in Wernicke's Encephalopathy Following Bariatric Surgery in a Young Patient.

Wernicke's encephalopathy (WE) is an acute neuropsychiatric syndrome resulting from thiamine deficiency (vitamin B1). It is characterised classically by a triad of ophthalmoplegia, confusion, and ataxia. WE is classically associated with alcoholism but increasingly has been observed due to other causes, particularly in undernourished post-bariatric surgery patients. Herein, we describe a case of WE following laparoscopic sleeve gastrectomy in a young male patient who presented with binocular horizontal diplopia and was found to have preretinal peripapillary haemorrhages. This case raises the awareness that posterior segment findings can occur in WE but have been under-reported previously.

Aggressive Low-Grade Optic Nerve Glioma in Adults.

Primary optic nerve gliomas are most commonly benign pilocytic astrocytomas (World Health Organization [WHO] Grade I) occurring in childhood and following an indolent course. Malignant optic gliomas occur in adulthood and follow an extremely aggressive course, with rapid infiltration of the chiasm, blindness, and death typically within months. A third category of optic glioma, occurring in adulthood, histopathologically benign (WHO Grade I-II) but following an aggressive course, has been rarely reported. The authors describe clinical and histopathologic features of clinically aggressive but histopathologically benign optic nerve gliomas of adulthood. Retrospective review of cases of biopsy-proven optic nerve glioma in the neuro-ophthalmology division of the Jules Stein Eye Institute from 1990 to 2011 was carried out. Cases following an aggressive course were selected for review of clinical, neuroradiologic, and histopathologic features. Three cases were selected for detailed study. Ages ranged from 31 to 45 years. All were initially diagnosed with optic nerve inflammation or benign neoplasm based on clinical and neuroradiologic features, but all suffered neuroradiologic extension and rapid deterioration of vision in the affected eye to no light perception over 3-8 weeks. Optic nerve biopsies were undertaken for the suspicion of malignancy. Features ranged from WHO Grade I (pilocytic astrocytoma, ganglioglioma) in two cases, to WHO Grade II in one case (diffuse astrocytoma, histopathologically benign, but associated with aggressive features such as high p53 [13-21%] and Ki-67 [40%]). The diffuse astrocytoma case subsequently developed extensive intracranial extension suspicious for malignant transformation. These findings indicate that benign optic nerve glioma in adults may be initially misdiagnosed as inflammation, be clinically aggressive, and require excision to prevent further intracranial involvement.

Coexistence of Idiopathic Intracranial Hypertension with Unilateral Duane Retraction Syndrome Type 1 in an Adult Female.

Idiopathic intracranial hypertension (IIH) is a poorly understood condition, and its presentation can coexist with other diseases. Simultaneous IIH and Duane retraction syndrome (DRS) type 1 have never been reported to coexist in an adult patient. Herein, we report a 32-year-old obese female with a history of chronic renal failure who had a renal transplant rejection 6 years prior to presentation and was treated with oral steroids and immunosuppressive medications. She began to experience signs and symptoms of increased intracranial pressure (morning headache and binocular horizontal diplopia) and had limited abduction of one eye on examination. The case was later diagnosed as IIH with DRS type 1.

Bitemporal visual field defects in ethambutol-induced optic neuropathy.

BACKGROUND: Ethambutol-induced optic neuropathy is well documented and most frequently associated with central or cecocentral scotomas. We designed a study to characterize the subset of patients who exhibit bitemporal visual field defects. METHODS: A computer search was performed for patients evaluated in a university academic neuro-ophthalmology consultative practice to identify those with the diagnosis of ethambutol-induced optic neuropathy. Clinical features were tabulated, including dose and duration of ethambutol use, time to onset of visual loss, initial and follow-up visual acuities, automated perimetry, optic disc appearance, and MRI features. Assessments for bitemporal visual field defect with alignment on vertical midline and for visual improvement after discontinuing ethambutol were performed. RESULTS: Nineteen cases of ethambutol-induced optic neuropathy were identified; All but 2 eyes demonstrated visual field defects worse in the temporal fields, most with margination along the vertical midline with superimposed central or cecocentral scotomas. Six cases (12 eyes) showed bitemporal defects with such margination without superimposed scotomas. Median time to onset of visual loss was 6.0 months. Visual improvement occurred (of 17 cases with data available) by at least 3 Snellen lines in 17 of 34 eyes (50%); mean visual acuity improvement was 3.74 lines (median, 3.0). Visual improvement by at least 3.0 decibels (dB) mean deviation (MD) on automated perimetry occurred in 27 of 34 eyes (79%); mean improvement in MD was 7.82 dB (median, 7.86). Median follow-up was 8.0 months. None had MRI abnormality in the chiasmal region. CONCLUSION: Bitemporal visual field defects are common in ethambutol-induced optic neuropathy. The pattern may mimic chiasmal compression, and neuroimaging is required. It may reflect susceptibility to toxicity of chiasmal crossing fibers.

Clinical findings and outcomes of uveitis associated with multiple sclerosis.

PURPOSE: To describe the clinical findings and outcomes in patients who presented with uveitis associated with multiple sclerosis. METHODS: Retrospective review of 20 patients (38 eyes). RESULTS: The most frequent ocular finding was multifocal elongated retinal perivenous "sheathing" with focal vascular leakage on fundus fluorescein angiography in 29 eyes followed by vitreous snowballs and debris in 26 eyes, anterior chamber inflammation in 15 eyes, mutton-fat keratic precipitates in 14 eyes, posterior synechiae in 13 eyes, cystoid macular edema in 9 eyes, iris nodules in 4 eyes, and optic neuritis in 3 eyes. Patients with cystoid macular edema were treated successfully with systemic corticosteroids combined with mycophenolate mofetil. Ocular complications were cataract in 6 eyes, glaucoma in 2 eyes and vitreous hemorrhage in 1 eye. CONCLUSIONS: Multifocal elongated retinal perivenous "sheathing" with focal vascular leakage on fundus fluorescein angiography is the most frequent finding in uveitis associated with multiple sclerosis.

Ophthalmologic abnormalities in a de novo terminal 6q deletion.

PURPOSE: To correlate the clinical phenotype with the genotype of a boy with a terminal deletion of chromosome 6q and to compare these observations to previous reports of 6q deletions and review of the literature. METHODS: Careful clinical evaluation, conventional cytogenetic analysis on GTG-banded chromosomes and 244K array CGH analysis. RESULTS: This 14 year old Saudi boy had modest mental retardation, seizures, microcephaly, cortical dysplasia, a non-comitant esotropia, impersistent eccentric gaze, congenital nystagmus, thick corneas, and substantial myopia. He had a de novo 10.79 Mb deletion on chromosome 6 from 6q25.3 to 6qter. The deleted region extended from nucleotide 159929512 to 170723629 and encompassed 87 genes. Eleven genes remained within the proband's deleted region after excluding genes located in deleted areas reported in phenotypically normal individuals. Among those 11 genes, only the TBP (TATA box binding protein) gene has been associated with any symptom or sign observed in our patient. CONCLUSIONS: This boy had clinical features similar to patients reported with the 6q terminal deletion syndrome. In addition, he had an unusual ocular motility pattern and thick corneas, features that may be more common than previously recognized. Deleted genes in this area of chromosome 6 may contribute to ophthalmic abnormalities in addition to mental retardation.

Capsular phimosis with complete occlusion of the anterior capsular opening after intact continuous curvilinear capsulorrhexis.

Shrinkage and whitening of the anterior capsule opening - capsular contraction syndrome - is a well-known complication after continuous curvilinear capsulorrhexis. A 72-year-old women underwent continuous curvilinear capsulorrhexis, phacoemulsification, and implantation of posterior chamber intraocular lens with polymethylmethacrylate haptics. Four months postoperatively, the patient reported deterioration in visual acuity that was resulted due to complete occlusion of anterior capsular opening by fibrotic tissue. The fibrous membrane was excised surgically in capsulorrhexis fashion.

Deep anterior lamellar Keratoplasty.

Keratoconus is a disease causing increased steepening of the cornea resulted in irregular astigmatism. Treatment options are Glasses, Hard contact lenses, Cross linking, Intracorneal Segments insertion, Refractive surgery (Gilda et al., 2008), or Keratoplasty. Lamellar Keratoplasty (LKP) can be a better choice to manage cases of moderate and some cases of severe Keratoconus without deep scarring and severe thinning, also in cases of corneal scarring not involving the deeper layers of the cornea. LKP is a corneal graft technique consisting of transplantation of partial-thickness donor tissue, devoid of endothelium, Descemet membrane (DM), and rear stroma into a recipient healthy stromal bed after dissection of pathologic anterior stroma. However, deep lamellar Keratoplasty (DLKP) is a surgical method that completely removes pathologic corneal stroma tissue down to the DM, followed by transplantation of donor cornea without endothelium over the host bed. DLKP has a number of advantages over penetrating Keratoplasty (PKP). Because it does not violate the intraocular structures of the eye, it diminishes or eliminates the chance of postoperative glaucoma, cataract formation, retinal detachment, cystoids macular edema, expulsive choroidal hemorrhage and epithelial ingrowths. Furthermore, this procedure avoids the replacement of host endothelium with donor endothelium and thus precludes endothelial graft rejection, with comparable visual outcomes and low rate of chronic endothelial cell loss compared to PKP.