Navigation-guided nasal endoscopic surgery for acute vision loss caused by fibrous dysplasia: a case report and review of literatures

Background Bone fibrous dysplasia is a benign disease of bone tissue dysplasia. Vision impairment is the commonest neurological complication of craniofacial fibrous dysplasia. Most of the vision loss caused by craniofacial fibrous dysplasia is usually a gradual process. Very few present with acute visual impairment as described in our case. Case presentation We report a patient with fibrous dysplasia presenting rapidly progressive visual loss in the left eye secondary to bone cyst formation. Transnasal endoscopic surgery guided by navigation with drainage and curettage of this bone cyst and orbital decompression resulted in progressive improvement in visual acuity that returned to normal 1 month post-operatively. Conclusions In cases with acute visual loss due to fibrous dysplasia, emergency surgical treatment should be considered to preserve vision. In the surgical approach, navigation-guided nasal endoscopic surgery may be preferred because of its advantages.

Acute visual loss as the first ocular symptom in a Sjögren’s syndrome patient with bilateral common carotid artery occlusion: a case report

Background Sjögren’s syndrome may be a risk factor for carotid artery stenosis. Bilateral common carotid artery occlusion (BCCAO) in a patient with Sjögren’s syndrome was not reported before. In this report, we describe a female with Sjögren’s syndrome who had acute visual loss due to ocular ischemic syndrome (OIS) with BCCAO. Case presentation A 50-year-old female with Sjögren’s syndrome visited our clinic with acute visual loss in the left eye. The best corrected visual acuity (BCVA) was 2/100 in the left eye, and the intraocular pressure (IOP) was normal in both eyes. Ocular ischemic change was observed during the ophthalmic examination. Aortography and computed tomography angiography (CTA) showed nearly total occlusion of the bilateral CCA. Thus, OIS with BCCAO was diagnosed. The vision in the left eye improved to 30/100 after carotid artery stenting for the left common carotid artery. Conclusions BCCAO may be present in patients with Sjögren’s syndrome. Large vessel abnormalities should be considered when acute visual loss is found in a patient with Sjögren’s syndrome.

Retrobulbar neuritis associated with empty sella syndrome: A diagnostic challenge

A 35-year-old female presented with sudden diminution of vision to finger counting at half meters in right eye (RE) since 15 days. RE showed grade III RAPD. Rest of the ocular examination was normal. She had no significant past medical history. Neuroimaging showed empty sella. We suspected retrobulbar neuritis (RBN) as a cause of loss of vision since there was no evidence of acute change in morphology of sella turcica like hemorrhage, trauma or ischemia, furthermore there were no pathological findings in RE and visual cortex to explain acute visual loss. Therefore, we prescribed intravenous steroids followed by oral steroids. Vision in RE improved to 6/12 after 15 days. We found that RBN can be a cause of sudden vision loss in cases with empty sella syndrome (ESS) and can pose diagnostic challenge that whether the vision loss is due to ESS or RBN. RBN can be a cause of acute vision loss in patients with ESS and can create diagnostic confusion.

Ocular ischemic manifestations in fibromuscular dysplasia

Purpose: To report visual, clinical and radiological outcomes of three patients with fibromuscular dysplasia (FMD) that presented different ocular ischemic events, supported by different multimodal imaging. Methods: A retrospective study including patients who attended a tertiary referral hospital from July 2016 to June 2019 referring acute visual loss and later diagnosed of FMD. Ophthalmological examination included best corrected visual acuity (BCVA), biomicroscopy, fundoscopy, fluorescein angiography (FA), and macular and retinal nerve fiber layer spectral domain optic coherence tomography (SD-OCT). Patients were admitted by the Neurology service, completing the study with blood and cerebrospinal fluid (CSF) test, serology and cultures, cerebral magnetic resonance angiography (angioMRI), and arteriography. Results: Three patients were included; one male and second females. All referred unilateral acute visual loss, with BCVA of counting fingers or hand-motion. Relative afferent pupilar defect was present in all patients. Two patients associated neurological focal signs and one had chronic hypertension. Fundoscopy revealed hemorrhages and exudates in all patients, and the typical sign of “string-of-beads” in one. FA showed dye filling delay and macular and peripheral hypofluorescence. SD-OCT revealed acute and chronic retinal ischemic signs. Blood and CSF tests, serologies and cultures resulted negative. All presented cerebral vascular involvement, objectified in the cerebral angioMRI or arteriography. Conclusions: FMD should be considered in cases with unilateral sudden visual loss associated to neurological focal deficits in young patients. The typical “string-of-beads” image is rare in small arteries such as the retinal vasculature. When suspected, a complete ophthalmological examination and cerebral vascular imaging is essential to confirm the diagnosis.

Presumed clomiphene-induced optic neuropathy: A case report

Background: Clomiphene citrate is an estrogen receptor ligand with mixed agonistic–antagonistic properties used for the treatment of female and male infertility. Various visual disturbances and several irreversible visual outcomes have been associated with clomiphene citrate. In this report, we present a patient with presumed clomiphene-induced optic neuropathy. Case: A 33-yr-old man with acute visual loss of the right eye was referred to Amiralmomenin Hospital, Rasht, Iran in November 2018. His only medication was clomiphene citrate 100 mg daily, taken for 2 wk for fertility issues. The patient presented with a sudden decrease of visual acuity in the right eye on the 14th day of starting the treatment and subsequently developed complete loss of inferior visual field within a few days. On examination, the visual acuity was 6/20 in the right and 20/20 in the left eyes, with a right relative afferent pupillary defect and decreased red color saturation. The fundus examination revealed optic disc swelling with venous dilation in the right eye and a normal left fundus with a crowded disc (disc-at-risk). The patient was evaluated for systemic disorders, all of which were normal. Findings were suggestive of non-arteritic anterior ischemic optic neuropathy most likely due to clomiphene. Conclusion: As clomiphene may increase blood viscosity, it is hypothesized that reduced flow in a posterior ciliary artery in conjunction with the disc-at-risk contributes to the anterior ischemic optic neuropathy. It is advised that patients with disc-at-risk be aware of the possible non-arteritic anterior ischemic optic neuropathy and those experiencing visual symptoms while taking clomiphene be examined promptly for evidence of optic nerve injury. Key words: Clomiphene citrate, Optic neuropathy, Visual acuity, Ischemia.

Acute disseminated encephalomyelitis with delayed onset and feasibility of the Miethke shunt and sensor reservoir system: a case report

Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating central nervous system disorder with predilection for early childhood. Delayed onset of ADEM is rare, and herein we present a previously healthy 5-year-old boy, with an unusual clinical course of ADEM with high intracranial pressure (ICP) and acute visual loss that was at first diagnosed as idiopathic intracranial hypertension without papilledema (IIHWOP). The boy underwent acute neurosurgical intervention with ventriculoperitoneal (VP) shunt using Miethke valve and sensor reservoir system and received high-dose steroid treatment with symptom relieve within days. This is the first case report using this system in such a young child, and we find it feasible and valuable also in younger children when VP shunt with ICP measurement is indicated.

Doubly Blinded: An Uncommon Cause of Acute Visual Loss Due to Orbital Compartment Syndrome

A 68-year-old female patient with a past medical history of atrial fibrillation on anticoagulation regimen with Apixaban and Clopidogrel presented for her scheduled Watchman device implantation. The device was indicated as patient was high risk for falling. Successful implantation of the left atrial appendage device was carried out, and the patient was sent to the floor. One hour after the procedure, the patient started having left-sided diplopia along with severe eye pain. An immediate CT scan of the head showed left superior orbital mass, concerning for hematoma. Urgent left canthotomy with cantholysis was conducted bedside. However, despite early interventions, the patient’s vision was lost.

Paraneoplastic optic neuropathy secondary to adenocarcinoma of the lung

We describe an uncommon cause of paraneoplastic optic neuropathy in adenocarcinoma of the lung. A 45-year-old healthy woman presented with seizure and encephalitis, followed by an acute visual loss in both eyes for 1 week. Her visual acuity was no perception of light in the right eye and hand movement in the left eye. There was a generalised restriction of extraocular muscle movements in both eyes. Funduscopy showed a bilateral pale optic disc. A paraneoplastic antigen autoimmune profile showed a positive anti-CV2/CRMP-5 antibody. CT of the thorax revealed the presence of right apical lung mass, confirmed to be adenocarcinoma through a biopsy. She was scheduled for lung lobectomy and chemotherapy. Unfortunately, her health deteriorated and she passed away eventually.