OBJECTIVE: To report a rare case of unilateral central retinal artery occlusion (CRAO) following spinal surgery.
METHODS: Observational case report.
RESULTS: A 15-year-old female patient underwent scoliosis surgery under general anesthesia in a prone position, her head being supported by a horseshoe headrest for approximately four hours, with stable vitals and without significant blood loss during surgery. Upon waking up from general anesthesia, the patient immediately reported severe visual loss in her right eye (RE), associated to marked periocular ecchymosis and chemosis. Visual acuity was limited to light perception. Fundus examination showed normal optic disc appearance with diffuse retinal pallor and a macular cherry red spot. Optical coherence tomography (OCT) showed increased reflectivity in the inner retina, consistent with ischemic maculopathy in the RE. Brain and neck magnetic resonance imaging angiograms were unremarkable. Further investigations ruled out collagen vascular disease, Behcet disease, syphilis, sickle cell disease and hypercoagulable states.
CONCLUSIONS: Central retinal artery occlusion is rarely observed following spinal surgery. The cause was presumed to be compression of the orbit by a horseshoe headrest in a prone position due to an accidental shift in position during surgery. This catastrophic complication, albeit rare, is usually irreversible and thus must be prevented. Proper positioning and vigilance by both the surgeon and the anesthesiologist during surgery are fundamental to ensure that the orbits are not under pressure.

UNASSIGNED: We performed a systematic review and meta-analysis of the association between retinal imaging parameters and Alzheimer\'s disease (AD).
UNASSIGNED: PubMed, EMBASE, and Scopus were systematically searched for prospective and observational studies. Included studies had AD case definition based on brain amyloid beta (Aβ) status. Study quality assessment was performed. Random-effects meta-analyses of standardized mean difference, correlation, and diagnostic accuracy were conducted.
UNASSIGNED: Thirty-eight studies were included. There was weak evidence of peripapillary retinal nerve fiber layer thinning on optical coherence tomography (OCT) (p = 0.14, 11 studies, n = 828), increased foveal avascular zone area on OCT-angiography (p = 0.18, four studies, n = 207), and reduced arteriole and venule vessel fractal dimension on fundus photography (p < 0.001 and p = 0.08, respectively, three studies, n = 297) among AD cases.
UNASSIGNED: Retinal imaging parameters appear to be associated with AD. Small study sizes and heterogeneity in imaging methods and reporting make it difficult to determine utility of these changes as AD biomarkers.
UNASSIGNED: We performed a systematic review on retinal imaging and Alzheimer\'s disease (AD).We only included studies in which cases were based on brain amyloid beta status.Several retinal biomarkers were associated with AD but clinical utility is uncertain.Studies should focus on biomarker-defined AD and use standardized imaging methods.

Sjögren-Larsson syndrome (SLS) is a rare, autosomal recessive neurocutaneous disorder. It is caused by the inheritance of sequence variants in the ALDH3A2 gene, which codes for fatty aldehyde dehydrogenase (FALDH). Universal signs of the condition are congenital ichthyosis, spastic paresis of the lower and upper limbs, and reduced intellectual ability. In addition to this clinical triad, patients with SLS experience dry eyes and decreased visual acuity caused by a progressive retinal degeneration. Examination of the retina in patients with SLS often reveals glistening yellow crystal-like deposits surrounding the fovea. This crystalline retinopathy often develops in childhood and is considered pathognomonic for the disease. The metabolic disorder typically shortens lifespan to half that of the unaffected population. However, now that patients with SLS live longer, it becomes increasingly important to understand the natural course of the disease. Our case describes a 58-year-old woman with advanced SLS whose ophthalmic examination illustrates the end-stage of the retinal degeneration. Optical coherence tomography (OCT) and fluorescein angiography confirm the disease is restricted to the neural retina with dramatic thinning of the macula. This case is unique since it is among the most advanced both in terms of chronological age and severity of retinal disease. While the accumulation of fatty aldehydes, alcohols, and other precursor molecules is the probable cause of retinal toxicity, a more complete understanding of the course of retinal degeneration may aid in the development of future treatments. The aim of our presentation of this case is to increase awareness of the disease and to foster interest in therapeutic research which may benefit patients with this rare condition.
Eye issues in Sjogren-Larsson Syndrome Sjögren-Larsson syndrome (SLS) is a rare, inherited condition that affects the skin and nervous system. It is caused by variations in a gene that controls the way fats are broken down in the body. The three key signs of the disease are (1) peeling, dry skin; (2) muscle stiffness and impaired movement of the arms and legs; and (3) reduced intellectual ability. Most signs of the condition appear shortly after birth. Genetic testing and counseling services can help patients and their families to understand what to expect with SLS. Caring for people with SLS requires teamwork by specialists like neurologists and physical therapists. Because eye problems are common, the early consultation of an eye doctor is also important. An eye examination can also confirm the diagnosis of SLS. SLS often causes the eyes to appear red, feel dry, or become irritated. This can make it hard to see in bright light. Decreased vision at night is also common. This is caused by the progressive loss of the central part of the retina which is needed to see fine details. Because SLS shortens lifespan, it is rare for anyone to reach the final stages of the disease. As patients with SLS are living longer, as illustrated by the individual in our case study, it becomes important to understand how the disease progresses. Unfortunately, treatments to restore vision are not yet available. Nevertheless, some protective measures can be taken. Eye examinations in early childhood are important for preventing damage to the eyes. Wearing glasses can improve vision, as well as protect eyes from accidental injury or falls. Eye drops can provide relief from dry eyes, and sunglasses can reduce glare and sensitivity to light. In the future, gene therapy may be used to treat SLS.

OBJECTIVE: To report a case of presumed sympathetic ophthalmia (SO) following scleral buckling (SB) surgery and to discuss the possible pathogenesis of this condition by reviewing the current evidence on this subject.
METHODS: Case report and narrative review of the literature; our case was imaged with spectral-domain optical coherence tomography (SD-OCT) and optical coherence tomography angiography (OCTA), fundus autofluorescence (FAF), fluorescein angiography (FFA) and indocyanine green angiography (ICGA).
RESULTS: A 55-year-old man presented with a macula on rhegmatogenous retinal detachment which was treated with 360° SB surgery, subretinal fluid drain (SRFD), cryopexy and pneumoretinopexy. Due to failure of the primary surgery, a second procedure was performed the day after with the explant of the prior buckle and the implant of a wider circumferential element. At three months from surgery, the patient complained of severe bilateral vision loss. Multimodal imaging revealed bilateral, multi-focal exudative retinal detachments and choroidal swelling. A diagnosis of presumed SO was made and the patient was treated with a combination of steroid and immunosuppressive drugs. The clinical picture completely resolved at postoperative month 12.
CONCLUSIONS: SO may be a rare complication of SB surgery. In our case, early recognition and prompt immunosuppressive treatment achieved good long-term clinical results.

BACKGROUND: Bietti crystalline dystrophy is primarily a retinal dystrophy caused by a CYP4V2 mutation and typically presents with crystalline retinal deposits in the posterior fundus.
METHODS: We present the case of an otherwise healthy 39-year-old Iranian woman with no family history of ocular disease who suffered with progressive vision loss that had started 2 years prior to presentation. Ocular examination revealed blurry optic nerve head margin and diffuse retinal crystalline deposit in both eyes. Spectral domain optical coherence tomography images showed retinal crystals, located mostly in outer retinal layers, with some areas of outer retinal tubulation and attenuation of outer retinal layers. Crystalline deposits were better visualized on near-infrared images as hyperreflective spots. Fundus autofluorescence images showed hyperautofluorescence areas on optic nerve head consistent with optic nerve head drusen and large hypoautofluorescence areas in posterior retina consistent with retinal pigment epithelium atrophy. Cystinosis was ruled out by blood testing.
CONCLUSIONS: Bietti crystalline dystrophy may be associated with optic nerve head drusen.

OBJECTIVE: To report the use of non-invasive multi-spectral imaging of a female choroideremia (CHM) carrier with mild visual symptoms and extensive fundus mottling.
METHODS: This was an observational case report study. A symptomatic 42-year-old female with a history of binocular CHM presented for routine ocular examination and underwent review of her clinical and photographic records, optical coherence tomography (OCT), intravenous fluorescein angiography (IVFA) and multi-spectral imaging (MSI). Dilated fundus examination and photography revealed similar outcomes of diffuse mottling with normal looking vessels. IVFA showed large irregular and confluent patches of RPE atrophy in the peripapillary and parapapillary areas as well as the midperiphery, corresponding to the OCT findings. The entire range of MSI imaging (520-940 nm) clearly illustrated the anomalies of the fundus including retinal pigment epithelium (RPE) mottling with melanin clumping not readily seen with the other imaging modalities. MSI fundus autofluorescence (MSI-FAF) showed a spotty hypo and hyperautofluorescent appearance of the fundus, consistent with the observations seen on IVFA and OCT images.
CONCLUSIONS: MSI significantly improves visualization of the retinal pigment epithelium in choroideremia. The non-invasive nature of MSI technique is a valuable tool in monitoring the effect of retinal and choroidal presentation in patients with CHM.