Streff-Like Syndrome in an Adult: A Case Report.

Streff syndrome is a non-malingering visual disturbance commonly affecting near and color vision, which is prevalent in the younger population. This case report presents Streff-like syndrome in a middle-aged woman. A 47-year-old woman with underlying major depressive disorder presented with bilateral reduced vision and a constricted visual field for one week. These symptoms were accompanied by additional neurological complaints of headache, weakness, and numbness triggered after a stressful event that affected her work performance. Examinations revealed reduced vision more toward near, diminished red saturation, color vision deficiencies, and bilateral tubular visual fields. Notably, the relative afferent pupillary defect was negative, with both anterior and posterior segments normal. Neuroimaging and inflammatory workup results were within normal limits. An additional +1.00 lens improved her symptoms and visual acuities. Collaborative management involving psychiatry, neurology, and ophthalmology, including psychotherapy, led to significant symptom improvement. At the five-month follow-up, the patient experienced a complete resolution of her visual symptoms. Although Streff syndrome is a primary visual problem, additional psychogenic factors may add to variable cases. This case underscores the importance of recognizing stress-induced psychogenic manifestation, particularly in patients with underlying mental health conditions, and emphasizes an interdisciplinary management approach.

Critical Assessment of the Neurological Complications during High-Risk Anesthesia Procedures.

Damage to the peripheral and central nervous systems is frequently irreversible. Surgically induced neurological damage and anesthesia may result in catastrophic situations for patients and their families. The incidence of significant neurological complications during the perioperative period is examined in this article. In contrast to other organs like the kidney, heart, liver, lungs, and skeletal system, native neurological function cannot be replaced with artificial parts or devices soon. Ignoring brain function during the perioperative period has been a systemic problem in anesthesiology, even though the central and peripheral nervous systems are crucial. This bold claim is intended to draw attention to the fact that, unlike the circulatory and respiratory systems, which have been routinely monitored for decades, the brain and other neural structures do not have a standard monitoring during surgery and anesthesia. Given that the brain and spinal cord are the principal therapeutic targets of analgesics and anesthetics, this deficiency in clinical care is even more alarming. Organs that are notoriously hard to repair or replace after damage have, up until now, received comparatively little attention. In this article, a succinct overview of five neurological complications associated with surgery and anesthesia is presented. After critically reviewing the literature on the subject, the article is focused to common (delirium), controversial (postoperative cognitive decline), and potentially catastrophic (stroke, spinal cord ischemia, or postoperative visual loss) adverse events in the neurological surgery setting. The findings will increase awareness of major neurological complications to the involved surgical and anesthesia team and enhance preventive and treatment strategies during the perioperative period.

Permanent Visual Loss as a Complication of Decompressive Surgery for the Treatment of Posterior Fossa Tumours: A Report of Two Cases.

Posterior fossa tumours are one of the most common types of solid neoplasia in paediatric patients. Although impaired vision can occur at presentation, it usually stabilises or improves after decompressive surgery. However, cases of permanent and profound visual loss have been reported following successful tumour resection, despite receiving little attention from the medical community. In this paper, we present two cases of young patients who experienced severe and permanent visual loss following uncomplicated surgery for posterior fossa tumour removal. We discuss the possible mechanism involved in the visual loss and measures to prevent such a dreadful complication.

Optociliary shunt vessels in multiple sclerosis.

BACKGROUND/INTRODUCTION: Optociliary shunt vessels develop as a result of chronic retinal venous obstruction. Optic neuritis has never been reported as a causative influence. OBJECTIVE: To determine whether optic neuritis predisposes to the development of optociliary shunts in patients with multiple sclerosis. CASES: This case series follows two patients with multiple sclerosis from August 1st, 2019 to April 24th, 2024, who developed optociliary shunt vessels after attacks of optic neuritis. A 43-year-old female presented with left visual loss and bilateral superior optociliary shunt vessels. Perimetry showed bilateral peripheral visual field loss. Optical coherence tomography showed bilateral retinal thinning and ganglion cell complex loss. Optical coherence tomography angiography showed reduced capillary density bilaterally. We investigated her and eventually diagnosed her with multiple sclerosis. The second, a 49-year-old female, developed right-sided optociliary shunt vessels after an episode of neuroretinitis. Perimetry revealed bilateral central scotomata; optical coherence tomography showed disc and retinal nerve fiber layer edema, and serous retinal detachment; later, ganglion cell complex loss; and reduced capillary density on optical coherence tomography angiography. Neuroimaging revealed demyelination in both, leading to a diagnosis of multiple sclerosis, and therapy was instituted. CONCLUSIONS: We hypothesize, that demyelinating optic neuritis due to multiple sclerosis causes chronic retinal hypoperfusion, leading to subsequent optociliary shunt development in affected eyes. Our case series reveals that eyes with optic neuritis, both previous episodes and fresh cases, can contribute to sufficient retinal vein hypoperfusion to cause the development of optociliary shunts, which should be reported in the literature.

Does optic neuritis in multiple sclerosis cause optociliary shunt vessels? Our case study shows that optociliary shunt vessels have developed in eyes having previous as well as fresh optic neuritis in two multiple sclerosis patients, as demonstrated by examination and investigations. We hypothesize that multiple sclerosis causes decreased retinal perfusion predisposing to the development of optociliary shunts. This will guide neurologists and ophthalmologists in diagnosing this debilitating condition upon the visualization of optociliary shunts; heralding previous or recurrent attacks of optic neuritis. @SanaNadeemS.

Efficacy of supplemental oxygen in reducing the need for laser or intravitreal bevacizumab in preterm infants with stage 2 retinopathy of prematurity.

BACKGROUND: Retinopathy of prematurity (ROP) is a disease that affects preterm infants born younger than 30 weeks of gestation. The pathophysiology of ROP involves an initial vaso-obliterative phase followed by vaso-proliferative phase that leads to disease progression. The use of supplemental oxygen during the vaso-proliferative phase of ROP has been associated with reduced disease progression, but how this impacts the need for ROP treatment is unclear. The goal of this study was to compare the rate of laser or intravitreal bevacizumab after implementation of a new supplemental oxygen therapy protocol in preterm infants with stage 2 ROP. METHODS: This is a retrospective chart review of preterm infants diagnosed with stage 2 ROP at Riley Hospital for Children between 1/2017 and 12/2022. Patients diagnosed between 1/2017 and 6/2020 were classified as Cohort A, preprotocol implementation. Patients diagnosed from 8/2020 to 12/2022 were classified as Cohort B, postprotocol implementation. In Cohort A, oxygen saturation was kept at 91-95% through the entire hospitalization. In Cohort B, oxygen saturation was increased to 97-99% as soon as Stage 2 ROP was diagnosed. Statistical analyses were performed using chi-square and Student's T test, followed by multivariate analyses to determine the impact of the oxygen protocol on the need for ROP treatment. RESULTS: A total of 211 patients were diagnosed with stage 2 ROP between 1/2017 and 12/2022. Of those patients, 122 were before protocol implementation therapy (Cohort A), and 89 were after implementation of supplemental oxygen protocol (Cohort B). Gestational age was slightly higher in Cohort B (Cohort A 25.3 ± 1.9, Cohort B 25.8 ± 1.84, p = 0.04). There was no difference in birth weight, NEC, BPD, or survival. Cohort B had lesser need for invasive mechanical ventilation and higher days on CPAP during hospitalization. Notably, Cohort A had 67 (55%) patients treated with laser photocoagulation or intravitreal bevacizumab versus 20 (22%) patients in Cohort B (OR 0.19, 0.08-0.40). CONCLUSION: The need for laser photocoagulation or intravitreal bevacizumab was significantly decreased in high-risk patients treated with the supplemental oxygen protocol. This result supports the idea that targeted supplemental oxygen therapy to keep saturations between 97 and 99% can reduce disease progression in infants with stage 2 ROP and potentially decrease the burden of additional procedures.

Ocular manifestation of hand, foot, and mouth disease : A case series.

Virus-related illnesses are a common phenomenon, especially in the colder months of the year. They usually manifest with cough, cold, and other flu-associated symptoms. They affect people of all ages and genders. In recent years, also virus-associated ocular symptoms have been documented repeatedly. One of the viruses known to cause these is the Coxsackievirus A, which causes hand, foot, and mouth disease. Three cases of these virus-associated macular changes are described below. In all three cases, the symptoms occurred unilaterally and with little time delay to the virus-typical general changes. Complete functional remission occurred in all cases, whereby permanent changes in the pigment epithelium were observed.

Sudden Visual Loss due to Arteritic Anterior Ischaemic Optic Neuropathy: A Rare Manifestation of Eosinophilic Granulomatosis with Polyangiitis.

Background: eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of various organs. While the eye is uncommonly affected in patients with EGPA, multiple ophthalmic manifestations have been reported, which can result in serious visual impairment without timely treatment. Case report: we report the case of a 79-year-old woman with a history of asthma and nasal polyps who presented with low-grade fever, mild alteration of mental status, and fatigue. Chest X-ray revealed bilateral interstitial infiltrates. Lab tests showed elevated C-reactive protein level and eosinophilia (eosinophil count, 4.6 x109 cells/l); blood cultures and parasitological examination of stools tested negative. Four days after presentation, the patient reported sudden and severe blurring of vision in her left eye. Ophthalmological examination revealed bilateral swollen optic disc and visual field loss, more severe in the left eye. A diagnosis of EGPA complicated by arteritic anterior ischaemic optic neuropathy (A-AION) was proposed, while an alternative or concurrent diagnosis of giant cell arteritis was ruled out based on clinical picture. Immunosuppressive treatment with high-dose intravenous glucocorticoids was promptly started. The patient's visual defect did not improve; however, two months later, no worsening was registered on ophthalmic reassessment. Conclusions: A-AION is an infrequent but severe manifestation of EGPA, requiring prompt diagnosis and emergency-level glucocorticoid therapy to prevent any further vision loss. Disease awareness and a multidisciplinary approach are crucial to expedite diagnostic work-up and effective management of EGPA-related ocular complications. LEARNING POINTS: Arteritic ischaemic optical neuropathy is a potential cause of sudden and severe visual loss in eosinophilic granulomatosis with polyangiitis (EGPA) patients.Visual loss due to arteritic ischaemic optical neuropathy is rarely reversible; however, a timely glucocorticoid treatment may prevent further progression of visual impairment.Multidisciplinary approach is crucial to expedite diagnostic work-up and effective management of EGPA patients with ocular complications.

A Case Report of Bilateral Exudative Retinal Detachment in Severe Pre-eclampsia.

We report a case of a 31-year-old gravida 2 para 1 female presenting to the optician with a two-week history of blurred vision and persistent headaches at 29 weeks gestation. Visual acuity on presentation was 6/100 in the right eye and 6/24 in the left eye. Fundoscopy of both eyes revealed serous retinal detachment in the absence of background retinal changes. On urgent admission to the maternity assessment unit, blood pressure was 189/126 mmHg and urine dipstick revealed 4+ proteinuria. Due to recurrent poor foetal heart rate variability on cardiotocography monitoring, an emergency caesarean was conducted. Sixteen hours following delivery, visual symptoms had improved, and clinical examination revealed normal blood pressure. An optical coherence tomography scan performed three months later was dry bilaterally with minor retinal pigment epithelium clumping. Serous retinal detachment involves the separation of the neurosensory retinal layer from the underlying retinal pigment epithelium. It is rare in pre-eclampsia but can be seen in patients with severe disease. The presentation of serous retinal detachment includes acute visual loss, reduced visual acuity, floaters, and flashing lights appearing in the vision. Although alarming on initial presentation, resolution is commonly seen within a couple of days postpartum. The pathogenic mechanism for serous retinal detachment development is widely discussed and thought to include changes to the choroidal circulation. Overall, although often self-resolving, a move to thorough antenatal care and vigilant monitoring in pre-eclamptic women is vital to prevent complications like this from occurring.

Transient Monocular Visual Loss (Amaurosis Fugax): How Does Age Impact Diagnosis?

Transient monocular visual loss (TMVL), also known as transient monocular blindness or amaurosis fugax ("fleeting blindness"), is a temporary loss of vision often due to ischemia to the retina. While acute TMVL should be considered an emergency that further requires exhaustive investigation, there are some cases in which TMVL arises secondary to benign causes. Age has a major impact in the diagnosis of ischemia and although the differential diagnosis of TMVL can be broad, timely and appropriate history, examination, diagnostic testing, and treatment can be vision- or life-saving. We review the causes of TMVL and the impact of age on the differential diagnoses and management.

Consulting in the dark: Robert Hamilton (1749-1830) and the importance of 'tenderness' towards patients.

Robert Hamilton (1749-1830) was born in Coleraine, Ireland, attended medical school in Edinburgh, Scotland, served in the British army and practised in South-East England. In order to differentiate him from his contemporary and namesake, Hamilton is identified by having worked in Ipswich, Suffolk and Colchester, Essex. This submission considers Hamilton's biography, his 1787 book on the British regimental surgeon and his ideas therein about professionalism. Central to his concept of professionalism is 'tenderness', a notion that broadly equates to empathy. He notes that tenderness brings improvement in clinical outcome and he has the foresight to recognise nurses as key to such care. The authors explore the concept of 'consulting in the dark', i.e. without access to clinical investigations. This is exemplified by doctors of the eighteenth century and earlier. Today general practitioners must still be comfortable 'consulting in the dark', e.g. when attending a patient's home. Hamilton's biography offers a further example of 'consulting in the dark': In later life, he lost his vision but continued to practise successfully. Central to his gift of consulting 'in the dark' was likely to be 'tenderness' for his patients, expressed through language and gentle touch. Hamilton's entreaty for 'tenderness' contrasts with modern medical education where reliance upon clinical tests, technology and pharmacology risksblinding young doctors towards patients and their lives.

Retinal complications post posterior chamber phakic intraocular lens implantation at a tertiary eye hospital in the Eastern Province of Saudi Arabia.

PURPOSE: Phakic intraocular lenses treat higher degrees of myopia not possible previously with conventional refractive surgery. The aim of this study is to report the incidence and risk factors of retinal complications after posterior chamber PIOL implantation and assess the differences in biometric parameters between patients who developed such complications versus those who did not. METHODS: This retrospective study recruited 514 patients who underwent ICL implantation to correct myopia at a tertiary eye hospital center in the Eastern province of Saudi Arabia. Follow up period was at least one year. Medical records of the patients were reviewed to obtain the required data. Associations between respondents' characteristics and retinal complications were evaluated using the Chi-squared test. RESULTS: The mean (SD) age was 27.7 (± 6.5) years ranging from 18 to 47. Laser treatment was performed in 14 cases (2.7%). Retinal complications occurred in six cases (1.2%). The risk of retinal complication was significantly higher among patients with high axial length (OR = 1.3, 95% CI 1.2, 1.4) and patients with high pre-spherical equivalent before ICL (OR = 1.09, 95% CI 1.03, 1.4). CONCLUSION: Patients with higher axial length and higher pre-spherical equivalent before ICL implantation are at high risk of retinal complications.

Superselective Arterial Hyaluronidase Thrombolysis is an Effective Treatment for Hyaluronic Acid-Induced Retinal Artery Occlusion: Study in a Rabbit Model.

BACKGROUND: There are serious complications associated with hyaluronic acid (HA) facial injections, including vision impairment due to retinal artery ischemia. In this study, we put forth a clinically relevant model of retinal ischemia and reperfusion in rabbit. We used this to verify the efficacy of hyaluronidase intra-artery thrombolysis in the treatment of hyaluronic acid-induced retinal artery occlusion. METHODS: Retinal artery ischemia was induced by injecting HA into the ophthalmic artery (OA) of adult chinchilla rabbit, and reperfusion was achieved by intra-artery thrombolysis therapy with hyaluronidase following 60 min and 4 h of occlusion. Digital subtraction angiography (DSA) and fundus fluorescein angiography (FFA) were used to evaluate blood flow in the retina. Electroretinogram (ERG), hematoxylin and eosin staining and transmission electron microscope were used to evaluate the structure and function of the retina after ischemia and reperfusion following 60 min and 4 h of occlusion. RESULTS: DSA and FFA images confirmed occlusion of the ophthalmic and central retinal arteries, as well as reperfusion after hyaluronidase thrombolysis. ERG indicated retinal dysfunction following ischemia, and thrombolysis partially rescued its impairment following 4 h of occlusion. Hematoxylin and eosin staining and TUNEL staining revealed ischemia-induced histological damages in the retina at different time windows, and hyaluronidase thrombolysis partially mitigated these damages. CONCLUSIONS: We report a method to establish a HA-induced retinal artery occlusion animal model. Hyaluronidase intra-artery thrombolysis was used to recanalize the embolized OA at different time points. Using our method, we achieved retinal reperfusion, and an improvement was observed in the visual function of rabbits after hyaluronidase thrombolysis following 4 h of occlusion. We believe that hyaluronidase intra-artery thrombolysis is an effective method to treat HA-induced retinal artery occlusion in clinic. LEVEL OF EVIDENCE II: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Blinding Optic Neuropathy Associated With Carboplatin Therapy: A Case Report and Literature Review.

Various forms of cancer and chemotherapeutics are associated with optic neuropathy. Cisplatin is a platinum analogue chemotherapeutic commonly associated with ocular toxicity among many other serious adverse effects. Carboplatin is a more chemically stable platinum analogue that is generally better tolerated with a comparatively favorable side effect profile. There are very few reports of carboplatin precipitating optic neuropathy. This case report describes a rare occurrence of carboplatin-induced blinding optic neuropathy. We treated a patient receiving carboplatin for neuroendocrine bladder cancer who developed rapidly progressive bilateral optic neuropathy over the course of three days. Upon evaluation at our clinic, his visual acuity had declined to light perception only and 20/60 in his left and right eye, respectively. Carboplatin therapy was immediately held and steroids were initiated. Despite the intervention, the patient's visual deficits have not improved at the one-year follow-up. Although the mechanism by which carboplatin causes ocular toxicity remains speculative, arterial ischemia appears to be the likely mechanism given the irreversible nature of visual decline. As demonstrated by our patient's course, irreversible vision loss despite high-dose steroid intervention necessitates expeditious recognition and management of this rare adverse effect. ​​​​​.

A rare presentation of a common carotid artery occlusion.

Background: A common carotid artery occlusion (CCAO) is very rare and the clinical features of CCAO have rarely been described. Since the blood supply of the eye and orbit is derived from the internal carotid artery, a CCAO may present with various ophthalmological symptoms, ranging from incidental findings to complete visual loss but also other neuro-ophthalmological abnormalities. Case report: A 61-year-old woman presented with acute monocular vision loss and an elevation deficit of the right eye. Fluorescein angiography showed delayed filling of both the retinal and choroidal vasculature, without occlusion/embolisms of the retinal arteries. Vascular imaging showed a right CCAO. Conclusion: CCAO has a variable presentation. In patients with acute unilateral visual loss a CCAO should be considered, especially when ocular motility deficits are present. Fluorescein angiography examination can aid in the localization and diagnosis of the vascular insult. Urgent referral for a systemic work-up is essential.

Full-field electroretinography - when do we need it?

Multimodal imaging and genetic testing allow sophisticated assessment of suspected inherited retinal disease. Given the availability of such technology, some question whether the full-field electrogram (ffERG) is needed anymore. In fact, a ffERG remains essential for certain clinical scenarios. The goal of this case-based review is to provide a clear understanding of what clinical situations warrant a ffERG. All practicing ophthalmologists should be familiar with this information.

Beyond Myelin Oligodendrocyte Glycoprotein and Aquaporin-4 Antibodies: Alternative Causes of Optic Neuritis.

Optic neuritis (ON) is the most common cause of vision loss in young adults. It manifests as acute or subacute vision loss, often accompanied by retrobulbar discomfort or pain during eye movements. Typical ON is associated with Multiple Sclerosis (MS) and is generally mild and steroid-responsive. Atypical forms are characterized by unusual features, such as prominent optic disc edema, poor treatment response, and bilateral involvement, and they are often associated with autoantibodies against aquaporin-4 (AQP4) or Myelin Oligodendrocyte Glycoprotein (MOG). However, in some cases, AQP4 and MOG antibodies will return as negative, plunging the clinician into a diagnostic conundrum. AQP4- and MOG-seronegative ON warrants a broad differential diagnosis, including autoantibody-associated, granulomatous, and systemic disorders. These rare forms need to be identified promptly, as their management and prognosis are greatly different. The aim of this review is to describe the possible rarer etiologies of non-MS-related and AQP4- and MOG-IgG-seronegative inflammatory ON and discuss their diagnoses and treatments.

Perioperative visual loss and consent for adult spine surgery: a national survey of the practice amongst spine surgeons and anaesthetists†.

BACKGROUND: Perioperative Visual Loss (POVL) is a devastating complication for patients undergoing spine surgery. Consent process for POVL amongst spine surgeons and anaesthetist remains variable. The aim of this study is to evaluate their practice and views about it. METHODS: Two similar questionnaires were distributed to members of the Society of British Neurological Surgeons (SBNS), British Association of Spine Surgeons (BASS), and Neuroanaesthsia and Critical Care Society (NACCS). RESULTS: A total of 271 responses were received (SBNS/BASS n = 149, NACCS n = 122). Fewer surgeons considered POVL as a material risk for patients compared to the anaesthetists (57.7 versus 79.7%). Outpatient/pre-assessment clinics were considered as the optimal setting for discussing POVL by the majority of the clinicians (81.2 and 93.4%). POVL should be discussed by both specialists according to 75% of the anaesthetists. Estimated incidence of POVL was considered to be higher by the anaesthetists (0.03-0.2% by 63% of the anaesthetist versus 0.0001-0.004% by 57% of the surgeons). Twenty-three surgeons and 10 anaesthetists had a patient who suffered from POVL, which led to a change of practice in most of them. This questionnaire will lead to a change in practice/consent to 18.1% of the surgeons and 23.5% of the anaesthetists. CONCLUSIONS: Most of the surgeons and anaesthetist feel that POVL is a material risk that ideally needs to be firstly discussed before the day of surgery, by both specialties. However, a significant number of clinicians have an opposite view. A national guidance from respective societies should encourage POVL to be discussed routinely.

Diffusion-weighted magnetic resonance imaging in early central retinal artery occlusion.

INTRODUCTION: Restricted retinal diffusion (RDR) has recently been recognized as a frequent finding on standard diffusion-weighted imaging (DWI) in central retinal artery occlusion (CRAO). However, data on early DWI signal evolution are missing. PATIENTS AND METHODS: Consecutive CRAO patients with DWI performed within 24 h after onset of visual impairment were included in a bicentric, retrospective cross-sectional study. Two blinded neuroradiologists assessed randomized DWI scans for the presence of retinal ischemia. RDR detection rates, false positive ratings, and interrater agreement were evaluated for predefined time groups. RESULTS: Sixty eight CRAO patients (68.4 ± 16.8 years; 25 female) with 72 DWI scans (76.4% 3 T, 23.6% 1.5 T) were included. Mean time-delay between onset of CRAO and DWI acquisition was 13.4 ± 7.0 h. Overall RDR detection rates ranged from 52.8% to 62.5% with false positive ratings in 4.2%-8.3% of cases. RDR detection rates were higher in DWI performed 12-24 h after onset, when compared with DWI acquired within the first 12 h (79.5%vs 39.3%, p < 0.001). The share of false positive ratings was highest for DWI performed within the first 6 h of symptom onset (up to 14.3%). Interrater reliability was "moderate" for DWI performed within the first 18 h (κ = 0.57-0.58), but improved for DWI acquired between 18 and 24 h (κ = 0.94). CONCLUSION: DWI-based detection of retinal ischemia in early CRAO is likely to be time-dependent with superior diagnostic accuracy for DWI performed 12-24 h after onset of visual impairment.

Sudden Unilateral Visual Loss Due to an Onodi Cell Mucocele.

In a patient whose sudden vision loss is suspected on the side of a previously operated sphenoid or ethmoid sinus, an ischemic insult to the optic nerve may occur due to a compressing mucocele, especially when there is bony dehiscence of the orbital apex. We report a case of a 41-year-old healthy male patient who had a history of previous sinus surgery, and visited the emergency department for an abrupt left ocular pain following visual loss of the affected eye. The patient's left eye's best corrected visual acuity (BCVA) was hand motions with a reduced light reflex. A sphenoethmoidal sinus cell (Onodi cell) mucocele with bone dehiscence in the left orbital apex was seen on computed tomography. Upon suspicion of compressive ischemic optic neuropathy, urgent endoscopy-assisted endonasal marsupialization of the Onodi cell mucocele with high-dose pulse intravenous dexamethasone was performed. The pathology showed an inflamed nasal mucosa, confirming a mucocele. On the second postoperative day, his BCVA slightly improved in counting fingers at 30 cm. However, even though the nasalized Onodi cell remained intact, his eyesight did not show further improvement. The optic nerve may be directly insulted when a mucocele in the Onodi cell takes place, especially when there is bony dehiscence in the orbital apex. Despite accelerated surgical marsupialization and high-dose steroids, the chance of visual recovery remains very low, as demonstrated in our case.

A Stepwise Progression of Acute Bilateral Visual Loss Due to Onodi Cell Sinusitis.

Acute visual loss in an immunocompromised patient may be caused by acute invasive fungal sinusitis (AIFS), even if symptoms include only mild headache and computed tomography (CT) shows only mild sinusitis, especially of the Onodi cell. Herein, we report a case of a 71-year-old man with a medical history of dermatomyositis and type 2 diabetes mellitus who presented with a stepwise progression of acute bilateral visual loss, mild headache, and altered consciousness. Initially, as the plain cranial CT showed only mild fluid retention in the posterior ethmoid sinus without bone destruction, the sinusitis was considered unrelated to the visual loss. Afterward, however, contrast-enhanced cranial magnetic resonance imaging (MRI) showed mucosal thickening, fluid retention in the posterior ethmoid sinus, and spread of the contrast medium over the dura around the right posterior ethmoid sinus and bilateral optic nerve tracts. Aspergillus fumigatus was identified from endoscopic drainage of the sinus. The patient was diagnosed with AIFS and treated with amphotericin B 350 mg/day. The altered sensorium and headache rapidly improved, and his left visual acuity improved to counting fingers. Although AIFS is rare, it can cause severe sequela or death due to vascular or direct intracranial invasion. Therefore, immediate drainage of the sinus and intravenous antifungal therapy are essential for AIFS. Our findings will help physicians make accurate and rapid diagnoses of AIFS in future cases.