A 25-year-old woman presented with acute bilateral blurred vision and history of headache, dizziness, and syncope for three days. Her visual acuity was 20/60 in both eyes. Fundoscopy revealed multiple bilateral peripapillary yellow-white patches like cotton wool spots, intraretinal hemorrhages and macular edema. The patient was diagnosed with Purtscher-like retinopathy based on the retinal findings and lack of trauma history. She was urgently admitted to the nephrology clinic due to thrombotic microangiopathy findings (hemoglobinemia, thrombocytopenia, and acute renal failure). After excluding thrombotic microangiopathy, the patient was diagnosed with atypical hemolytic uremic syndrome (aHUS) with the clinical and laboratory findings. Eculizumab treatment was added to hemodialysis and plasmapheresis therapy. Three months after starting treatment, retinal lesions regressed and visual acuity increased to 20/20 in both eyes. To the best of our knowledge, this is the first reported case of Purtscher-like retinopathy associated with aHUS.

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Purpose: To evaluate the ophthalmic, systemic, and genetic characteristics of patients with Wolfram syndrome. Methods: In total, 13 patients with suspected or clinically diagnosed Wolfram syndrome underwent ophthalmic and systemic examinations and genetic analyses for Wolfram syndrome between August and October 2018. Results: The mean age of the subjects was 24.2 ± 7.1 years, of which 5 (38.5%) subjects were male and 8 (61.5%) were female. The mean best-corrected visual acuity ranged from counting fingers to 20/40, with a mean of 20/250 (1.10 ± 0.69 logarithm of the minimum angle of resolution). Dyschromatopsia was present in all patients (100%). There was a severe decrease in the average peripapillary retinal nerve fiber layer and macular ganglion cell–inner plexiform layer thicknesses (54.7 ± 6.5 and 51.9 ± 4.8 µm, respectively). Optical coherence tomography angiography showed significantly lower whole-image, inside disk, and peripapillary vessel densities in the patients with Wolfram syndrome than in the healthy controls (p < 0.001 for all). All patients who underwent genetic analyses had mutations in the WFS1 gene. Moreover, two novel mutations, p.Met623Trpfs*2 (c.1867delA) and p.Arg611Profs*9 (c.1832_11847del16) at exon 8, were detected. The frequency of systemic findings was as follows: optic atrophy (100%), diabetes mellitus (92.3%), central diabetes insipidus (38.5%), sensorineural hearing loss (38.5%), and presence of urological (30.8%), psychiatric (30.8%), and neurological (23.1%) diseases. Conclusion: Wolfram syndrome is a rare genetic disorder that can be associated with severe ophthalmic and systemic abnormalities. All patients who present with unexplained optic atrophy should be evaluated for Wolfram syndrome, even if they do not have diabetes mellitus because optic atrophy can sometimes manifest before diabetes mellitus.

AimTo report a critical case series of six patients with posterior microphthalmos (PM).MethodComplete ophthalmologic examinations of all patients were performed using best-corrected visual acuity (BCVA), cycloplegic refraction, applanation tonometry, slit lamp biomicroscopy of the anterior segment, fundoscopy, A and B mode ultrasonography (USG), keratometry, and optic coherence tomography (OCT).ResultsThe most significant clinical characteristics of male patients aged 10–25 years was the presence of shorter posterior segments (mean: 15.27–18.91 mm) accompanying high hyperopia (mean +9.00 – +18.50 diopter) despite the normal anterior segment findings. The BCVA ranged between 20/320 and 40/100. Retinal folds were detected bilaterally on the papillomacular band in all patients. Although neurosensory retina was included in the fold in OCT images, retinal pigment epithelium, choroid, and sclera were not included in the fold. Pigmentary retinopathy was detected in one patient.ConclusionDespite normal anterior segment, posterior microphthalmos is characterized with high hyperopia, and shorter axial length and bilateral papillomacular retinal fold. Refractive amblyopia, uveal effusion syndrome, retinal detachment and macular hole are complications that can be corrected. Posterior microphthalmos must be kept in mind in patients with a normal anterior segment, and high hyperopia.

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Methanol intoxication is detrimental to health, possibly resulting in blindness and occasionally death. In association with ocular signs and the other systemic and laboratory features, the ophthalmologist should be alert to the diagnosis of methanol intoxication in which visual loss may be the only symptom.

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