Complete Bilateral Blindness Research Articles

Delayed reversal of methanol-induced blindness in an adult by a combination of erythropoietin and a high dose of methylprednisolone: a case report

BackgroundThe clinical manifestation following methanol toxicity accounts for a life-threatening problem that contributes to metabolic disorders, neurological complications, blindness, and even death. There is no completely effective treatment to retain the patient’s vision. Herein, we apply a new therapeutic strategy for the recovery of bilateral blindness in a patient who had ingested methanol.Case presentationA 27-year-old Iranian man with complete bilateral blindness was referred 3 days after accidental ingestion of methanol to the poisoning center at Jalil Hospital, Yasuj, Iran, in 2022. After taking his medical history, performing neurologic and ophthalmologic examinations, and routine laboratory tests, ordinary management was undertaken and counterpoisons were given for 4–5 days; however, the blindness did not reverse. Following the 4–5 days of unsuccessful standard management, he was given ten doses of subcutaneous erythropoietin 10,000 IU/12 hours twice daily, folinic acid 50 mg/12 hours, and methylprednisolone 250 mg/6 hours for 5 days. After five days, vision of both eyes recovered, reaching 1/10 in the left and 7/10 in the right eye. He remained under daily supervision until his release from the hospital, and he was discharged from the hospital 15 days post admission. In outpatient follow-up, his visual acuity was improved without having any side effects at 2 weeks after discharge.ConclusionA combination of erythropoietin and a high dose of methylprednisolone were useful for relieving the critical optic neuropathy and improved the optical neurological disorder following methanol toxicity.

Complete bilateral blindness associated with ruptured anterior communicating artery aneurysm: A literature review and illustrative case.

Anterior communicating artery (AcomA) aneurysms are considered one of the most common intracranial aneurysms, contributing to approximately 40% of the subarachnoid hemorrhages related to aneurysmal rupture. Aneurysms of the anterior circulation are commonly present with visual defects varying in their nature according to the aneurysmal site. However, complete bilateral vision loss associated with AcomA aneurysms is a significantly rare finding. We are reporting a case of complete bilateral blindness in a patient with a ruptured AcomA aneurysm with a literature review. We conducted a PubMed Medline database search by the following combined formula of subjects' headings: (((Intracranial Aneurysm [MeSH Terms]) AND (AcomA Aneurysm [Title/Abstract])) AND [(Vision loss OR Blindness [Text Word])) AND (case reports [Filter]). Additional articles were searched through the reference lists of the included articles. Our review yielded a total of five cases. All the present cases revealed unilateral blindness only, and their outcomes after treatment vary from recovery of vision to unchanged complete vision loss - none of the cases found in the literature presented with bilateral blindness. AcomA aneurysms can be associated with visual loss in some cases. However, usually, the defect is unilateral. Studies of the visual defects, including potential bilateral complete blindness associated with rupture inferiorly, directed AcomA aneurysm, should be highlighted.

Post-operative vision loss: analysis of 587 patients undergoing endoscopic surgery for pituitary macroadenoma

Purpose Vision loss following surgery for pituitary adenoma is poorly described in the literature and cannot be reliably predicted with current prognostic models. Detailed characterization of this population is warranted to further understand the factors that predispose a minority of patients to post-operative vision loss. Materials and methods The medical records of 587 patients who underwent endoscopic transsphenoidal surgery at the Mount Sinai Medical Centre between January 2013 and August 2018 were reviewed. Patients who experienced post-operative vision deterioration, defined by reduced visual acuity, worsened VFDs, or new onset of blurry vision, were identified and analysed. Results Eleven out of 587 patients who received endoscopic surgery for pituitary adenoma exhibited post-operative vision deterioration. All eleven patients presented with preoperative visual impairment (average duration of 13.1 months) and pre-operative optic chiasm compression. Seven patients experienced visual deterioration within 24 h of surgery. The remaining four patients experienced delayed vision loss within one month of surgery. Six patients had complete blindness in at least one eye, one patient had complete bilateral blindness. Four patients had reduced visual acuity compared with preoperative testing, and four patients reported new-onset blurriness that was not present before surgery. High rates of graft placement (10/11 patients) and opening of the diaphragma sellae (9/11 patients) were found in this series. Four patients had hematomas and four patients had another significant post-operative complication. Conclusions While most patients with pituitary adenoma experience favourable ophthalmological outcomes following endoscopic transsphenoidal surgery, a subset of patients exhibit post-operative vision deterioration. The present study reports surgical and disease features of this population to further our understanding of factors that may underlie vision loss following pituitary adenoma surgery. Graft placement and opening of the diaphragma sellae may be important risk factors in vision loss following ETS and should be an area of future investigation.

A rare case of posterior reversible encephalopathy syndrome in a patient with severe leptospirosis complicated with rhabdomyolysis and acute kidney injury; a case report

BackgroundLeptospirosis is a zoonotic spirochetal disease caused by Leptospira interrogans. The clinical presentation ranges from an asymptomatic state to a fatal multiorgan dysfunction. Neurological manifestations including aseptic meningitis, spinal cord and peripheral nerve involvement, cranial neuropathies and cerebellar syndrome are well recognized with varying frequencies among patients with this disease. Posterior reversible encephalopathy syndrome is a very rare occurrence in leptospirosis and only two cases are reported in the medical literature up to now. We report a case of posterior reversible encephalopathy syndrome in a patient with leptospirosis with rhabdomyolysis and acute kidney injury.Case presentationA 21 year-old male presented with fever and oliguric acute kidney injury with rhabdomyolysis. A diagnosis of leptospirosis was made and he was being managed according to the standard practice together with regular hemodialysis. The clinical condition was improving gradually. On day 8 of the illness, he developed headache and sudden painless complete bilateral vision loss followed by several brief generalized tonic clonic seizure attacks. Examination was significant for a Glasgow Coma Scale of 14/15, blood pressure of 150/90 mmHg and complete bilateral blindness. The findings of magnetic resonance imaging of the brain were compatible with posterior reversible encephalopathy syndrome. He was managed with blood pressure control and antiepileptics with supportive measures and standard treatment for leptospirosis and made a complete recovery.ConclusionPosterior reversible encephalopathy syndrome, though very rare with leptospirosis, should be considered as a differential diagnosis in a patient with new onset visual symptoms and seizures, especially during the immune phase. Optimal supportive care together with careful blood pressure control and seizure management would yield a favourable outcome in this reversible entity.

Predictors of Postoperative Visual Outcome After Surgical Intervention for Craniopharyngiomas

Because of involvement of the optic apparatus, craniopharyngiomas frequently present with visual deterioration. Although visual improvement is a primary goal of surgical intervention, prediction models are lacking. We retrospectively reviewed all patients undergoing craniopharyngioma surgery at a single institution (2014-2019). Preoperative, intraoperative, and postoperative variables of interest were collected. Visual acuity and visual fields (VFs) were standardized into Visual Impairment Scores (VISs), defined by the German Ophthalmological Society. VIS ranged from 0 (normal vision) to 100 (complete bilateral blindness). Visual improvement/deterioration was defined as a postsurgical decrease/increase of ≥5 VIS points, respectively. Complete ophthalmologic assessments were available for 61 operations, corresponding to 41 patients (age, 4-73 years). Vision improved after 28 operations (46%), remained stable after 27 (44%), and deteriorated after 6 (10%). In bivariate analysis, significant predictors of visual improvement included worse preoperative VIS (odds ratio [OR], 1.058; P < 0.001), worse preoperative VF mean deviation (OR, 1.107; P= 0.032), preoperative vision deficits presenting for longer than 1 month (OR, 6.050; P= 0.010), radiographic involvement of the anterior cerebral arteries (OR, 3.555; P= 0.019), and gross total resection (OR, 4.529; P= 0.022). The translaminar surgical approach was associated with visual deterioration (OR, 6.857; P= 0.035). In multivariate analysis, worse preoperative VIS remained significantly associated with postoperative visual improvement (OR, 1.060; P= 0.011). Simple linear correlation (R2=0.398; P < 0.001) suggests prediction of postoperative VIS improvement via preoperative VIS. Patients with reduced preoperative vision, specific radiographic vascular involvement, and gross total resection showed increased odds of visual improvement, whereas the translaminar approach was associated with visual deterioration. Such characteristics may facilitate patient-surgeon counseling and surgical decision making.

Glycine induced acute transient postoperative visual loss.

INTRODUCTION Hysteroscopic surgeries, which require use of irrigating fluids like glycine can rarely be associated with significant complications.[12] We report a case of transient postoperative blindness following use of large quantities of glycine delivered through a rotatory pump set at an inappropriately high pressure. CASE REPORT A 27-year-old nulliparous female patient, American Society of Anesthesiologists grade I physical status with a history of menorrhagia and severe progressive dysmenorrhea, resistant to medical management, was scheduled for a hysteroscopic myomectomy under general anaesthesia, after ultrasound study revealed multiple submucous fibroids. Routine preoperative blood investigations were normal. Premedication included intramuscular glycopyrrolate 0.2 mg given half an hour before surgery, intravenous ranitidine 50 mg and intravenous ondansetron 4 mg. Anaesthesia was induced with intravenous thiopentone 250 mg, intravenous fentanyl 100 μg and intravenous atracurium 25 mg. After endotracheal intubation, 60% nitrous oxide in oxygen and isoflurane 0.8–1% was used for maintenance of anaesthesia with controlled ventilation. A 22F Karl Storz™ (Germany) resectoscope was used for hysteroscopy. To facilitate distension of the uterine cavity and better visualization during hysteroscopy, glycine 1.5% was infused at 200 mmHg pressure through the side port of Karl Storz endomat roller pump. 33 L glycine was infused and the outflow container collected 27 L. The procedure lasted for approximately 40 min. Haemodynamic parameters stayed within 20% of baseline values throughout the procedure. Neuromuscular blockade was reversed and the patient had an uneventful recovery from anaesthesia. On awakening, the patient complained of visual loss which was initially attributed to either residual effects of anaesthetic agents or the eye ointment. An hour after recovery in the postanaesthesia care unit (PACU), she was fully awake but complained of nausea, vomiting and had complete bilateral blindness. There were no other neurological deficits. On ophthalmic examination, the patient had normal eye movements, no light perception, mildly dilated pupils, normal intraocular pressures and absent direct and indirect light reflexes. Fundal examination revealed normal vasculature with no signs of the optic disc or macular oedema. A presumptive clinical diagnosis of glycine toxicity was made, and the patient was reassured that the blindness was a transient phenomenon. In the PACU, serum electrolytes, osmolality, urea, glucose, and ammonia were checked an hour after the procedure. Serum osmolality (282 mOsm/kg), serum potassium (4.3 mmol/L) and glucose were within normal limits. Serum ammonia was 137 μg/dl (reference range 27–102 μg/dl) and serum sodium was 127 mmol/L. Serum creatinine, liver function tests and blood urea nitrogen were within normal limits. 8 h after the procedure, serum ammonia was 234 μg/dl, serum sodium was 130 mmol/L and serum potassium was 2.3 mmol/L. Her vision showed signs of improvement and returned to normal within 20 h of the procedure. Serum ammonia level, checked 26 h after the procedure, was normal (52 μg/dl). Her remaining hospital stay was uneventful. She was discharged on the 3rd day after the surgery. DISCUSSION Glycine, a low-viscosity fluid, is favoured for uterine distension during hysteroscopies for its good optical image, poor electrical conduction, and minimal haemolysis.[3] Visual disturbances correlate with plasma glycine concentration of 5–8 mmol/L when glycine, an inhibitory neurotransmitter in the retina, slows down the transmission of impulses from the retina to the cerebral cortex resulting in prolonged visual evoked potentials and absent oscillatory potentials on the electroretinogram.[45] Transient blindness, which resolves within 24 h, following glycine absorption, has been reported.[6] Though blood ammonia (an intermediate product in glycine metabolism) concentration of more than 100 μmol/L (normal range 10–35) is associated with neurological symptoms,[7] inter-individual variability is great and patients may show neurological symptoms with normal blood ammonia concentration after absorbing large amounts of 1.5% glycine. There is a vague correlation between hyperammonaemia and visual disturbances.[8] There have been reports of transient blindness with high levels of ammonia after hysteroscopy, one case of temporary complete blindness, and several cases where visual acuity was temporarily reduced to perception of light after transurethral resection of prostate using glycine as the irrigation fluid.[9] In our patient, the increase in blood ammonia level corresponded with the period of blindness. For a better view, gynaecologists prefer a well-distended uterus. In our case, this was achieved by increasing the pressure (200 mmHg) to infuse glycine, which was higher than the safe standards suggested by Vulgaropulos et al.[10] (35–75 mmHg) which would have contributed to rapid absorption resulting in toxicity. CONCLUSION Large quantities of glycine, when used under higher than recommended pressures can result in excessive absorption and toxicity. With the increasing numbers of hysteroscopic procedures performed on a more routine basis, a more judicious use of irrigating solutions like glycine is necessary to avoid complications.

Neuromyelitis optica of very late onset: one more case

Dear editors, We read with extreme interest the paper by Souza et al.1 This study describes one case of a 75-year-old woman with neuromyelitis optica (NMO)1. Including this case, only six cases of neuromyelitis optica developing after the age of 70 have been reported. We think that it is important to describe our recent experience. A 72-year-old Caucasian woman sought our service complaining of a rapidly progressive visual loss in the right eye. After a further week, the other eye was affected, culminating in complete bilateral blindness in less than 15 days. The patient also developed paraparesis, becoming paraplegic in this period. On physical examination she had a T4 sensory level associated with significant dysesthesia. Her magnetic resonance image showed lesions in three contiguous spinal cord levels (T2-T4) and absence of any brain lesions suggestive of multiple sclerosis. Computed tomography scans of the chest, abdomen and pelvis were normal. A CSF analysis showed a cell count of 11 cells/mm3 (36% lymphocytes, 60% monocytes), glucose 75 mg% and proteins 68 mg%. Tests for syphilis, bacteria, fungi, herpes simplex, herpes zoster, cytomegalovirus, HTLV and HIV were negative. There were no oligoclonal bands. Serum assays on anti-DNA antibodies, anti-nuclear factor, anti-neutrophil cytoplasmic antibodies (p-ANCA and c-ANCA), anti-Ro (SSA), anti-La (SSB), anti-SM and rheumatoid factor, together with the serological test for the human immunodeficiency virus (HIV), were negative. Although she had not been tested for NMO-IgG, our patient fulfilled the diagnostic criteria for neuromyelitis optica2. The patient underwent pulse therapy with methylprednisolone for five days3. Her visual deficit showed some improvement. Immunoglobulin tests and plasmapheresis were not carried out owing to infectious complications that developed later. Our patient was released from the hospital with oral corticosteroids prescribed. Further evaluation is still necessary to establish whether the patient has shown any further improvement or relapses.

Migraine With Binocular Blindness: A Clinic-Based Study

To describe the syndrome of migraine with binocular blindness. Rarely do migraine patients complain of losing vision in both eyes during an attack of headache. There are no large clinic-based studies looking at the prevalence of binocular blindness in migraine sufferers and no information about patient demographics, neuroimaging, and laboratory testing. Over a 14-month time period, 383 new patients with a diagnosis of migraine were seen at the Geisinger Headache Center. All patients were asked if they ever experienced an episode of complete bilateral blindness along with their headaches. Those with a positive history had coagulopathy testing as well as brain magnetic resonance imaging and magnetic resonance angiography of the intracranial circulation. A total of 6 patients or only 1.6% of the new migraine patients had episodes of binocular blindness with their headaches. All were female and each had a history of migraine for at least 3 years. Five patients had a diagnosis of migraine without aura, while one had a diagnosis of basilar-type migraine. In all patients the blindness episodes occurred in isolation during a migraine headache. In all but one patient the blindness was instantaneous and not a slow evolution. In 2 patients the blindness episode only occurred 1 time; in 3 patients episodes occurred more than once but were rare, while 1 patient had blindness with 50% of her headaches. In regard to duration, in 2 patients blindness lasted only several seconds, 2 patients between 2 and 10 minutes, 1 patient 30 minutes and 1 patient 60-120 minutes. Neuroimaging was normal in all. Three patients had a history of smoking and 3 never smoked. Coagulopathy testing was abnormal in all patients. Two patients were homozygous for methylenetetrahydrofolate reductase (MTHFR) 677TT polymorphism, but both had normal homocysteine levels; 3 patients were heterozygous for MTHFR 677CT polymorphism and 1 had elevated homocysteine levels and 1 patient had a positive lupus anticoagulant (had the most frequent episodes of blindness). Binocular blindness with migraine headache is a very rare occurrence at least in a headache specialty clinic population. It is a female-predominant event and occurs mostly in migraine patients who do not have a history of aura. Blindness episodes can be very brief or prolonged and many do not fit the typical duration of a migraine aura. They are typically infrequent events and may occur only 1 time without recurrence. Migraine with binocular blindness may reflect an underlying clotting disorder. A possible etiology outside of a coagulopathy-related event is retinal spreading depression.

Orbital Involvement in Bing-Neel Syndrome

We read the article “Orbital Involvement in Bing-Neel Syndrome” by Stacy et al (1) with great interest and commend the authors for their excellent discussion of the manifestations of Waldenström macroglobulinemia (WM) in the orbit and brain. We had the opportunity recently to see a similar patient with WM and diffuse orbital involvement whose clinical course demonstrates that the visual outcome in these patients may be poor despite aggressive therapy. A 57-year-old man was referred to a retina specialist with a 3-week history of progressive, painless, bilateral visual loss. His medical history was significant for WM diagnosed 9 years previously, for which he was treated with 5 cycles of cyclophosphamide, rituximab, and prednisone, most recently 1 year prior to presentation. On examination, visual acuity was 20/160, right eye, and 20/32, left eye. Anterior segments were quiet, eye movements were full, and there was no proptosis. Dilated fundus examination revealed bilateral optic disc edema greater in the right eye than in the left eye, with a normal appearance of the retinal vessels, macula, and periphery. Fluorescein angiography showed leakage and staining of the optic discs. Infiltrative optic neuropathy was suspected, and he was started on oral prednisone 80 mg daily and referred immediately to his oncologist. MRI of the orbits revealed enhancement of the orbital fat and optic nerves (Fig. 1). Serum IgM was elevated at 1,350 mg/dL (range, 40-230 mg/dL). Chemotherapy with cyclophosphamide, vincristine, prednisone, and rituximab was initiated. However, vision declined to hand motions, right eye and 20/50, left eye, within 3 weeks of initial presentation. A right orbital biopsy was performed, and histopathologic examination revealed fibroadipose tissue with patchy aggregates of small lymphocytes, scattered plasma cells, and histiocytes. Immunoperoxidase staining of the lymphocytes was positive for CD20, CD79a, and CD138 and negative for CD23, CD5, CD10, and BCL-1. These matched markers from the patient's previous bone marrow biopsies, confirming orbital infiltration of WM.FIG. 1: Contrast-enhanced axial (A) and coronal (B) fat-suppressed T1 orbital MRI scans show patchy enhancement of orbital fat, enlargement of the extraocular muscles, and thickening and enhancement of the orbital segments of the optic nerves.The patient was hospitalized for intravenous infusion of high-dose methotrexate and intrathecal cytarabine. A lumbar puncture yielded lymphocytes in insufficient number for flow cytometry. The patient received external beam orbital radiation therapy in 18 fractions for a total dose of 30.6 Gy. Despite treatment, the patient's vision declined to no light perception in each eye. Stacy et al (1) noted that other than slight resistance to retropulsion, their patient's external examination gave no hint of the diffuse infiltration of the orbital fat and optic nerves by malignant cells found on neuroimaging and confirmed with orbital biopsy. They suggested the absence of proptosis could be explained by an almost equal replacement of fat by tumor. Similarly, except for bilateral optic disc edema and progressive visual loss, our patient's clinical examination offered no clues to the extent of the orbital and optic nerve involvement found on MRI. Our case differed somewhat in that severe visual loss occurred early and progressed rapidly to complete bilateral blindness despite aggressive therapy. In addition, our patient's MRI demonstrated involvement of the extraocular muscles. That such diffuse infiltration of orbital tissue can occur without proptosis or orbital congestion, and with preservation of eye movements, is striking. Rishi R. Doshi, MD Rona Z. Silkiss, MD, FACS Richard K. Imes, MD California Pacific Medical Center, San Francisco, California [email protected]

Clinical-pathology review: Supratentorial cerebral arteriovenous fistula

Objective We present the first report in the literature on the bilateral tentorial herniation after the endovascular embolization of a large brain arteriovenous fistula. Clinical presentation A 7-year-old boy who had chronic headaches underwent computed tomography (CT), which disclosed an abnormal intracranial mass in the right cerebral hemisphere. Magnetic resonance imaging (MRI) scan shows multiple tortuous signal void lesions suggesting vascular lesions. Cerebral angiography revealed a cerebral arteriovenous fistula with a very large venous pouch. The patient was referred to Beijing Tiantan Hospital for further treatment. Intervention Partial embolization of the AVF from the arterial side was initially attempted successfully resulting in a reduction of the shunt flow. The patient developed swelling of his right hemisphere and caused bilateral tentorial herniation. Although the decision was made to attempt resection of the thrombosed varix, there was yet any recovery of blindness caused by herniation. Conclusion Our study suggests that tentorial herniation can cause compression of optic tracts leading to complete blindness with conscious clarity. Clinicopathologic considerations of the complete bilateral blindness produced from the above events are discussed in detail. Prevention is the only therapeutic approach to this type of visual catastrophe. The outcome of radical surgical intervention for this kind of patients is still unclear and further investigation should be given.

Bilateral amaurosis in 11 patients with giant cell arteritis confirmed by arterial biopsy

Even in the times of corticosteroids giant cell arteritis may lead to complete bilateral blindness. To assess the frequency of complete irreversible blindness in giant cell arteritis. Among all 218 patients with the diagnosis of giant cell arteritis confirmed by arterial biopsy between 1980 and 2000, clinical data of patients with bilateral amaurosis were further investigated. The main interest was focussed on the kind of ocular manifestation, the interval between first symptoms and therapy and the interval between involvement of the first and second eye. In 11 patients (9 women, 2 men, mean age: 79 years) giant cell arteritis led to complete bilateral blindness. Morphological ocular changes were anterior ischemic optic neuropathy (15 eyes), optic atrophy (4 eyes), posterior ischemic optic neuropathy (2 eyes), and central artery occlusion (1 eye). The median interval between involvement of the first eye and initiation of therapy was 4 days (1/2 day to 8 weeks). The median interval between visual loss in the first and second eye measured 4 days (simultaneously to 30 days). In 2 patients visual loss occurred 1 and 2 days after initiation of treatment (500 mg methylprednisolone/daily), respectively. Treatment with corticosteroids (100 - 1000 mg) did not result in visual improvement in any patient. Complete bilateral blindness occurred in 5 % of patients with giant cell arteritis, up to 2 days after initiation of treatment with corticosteroids. This number can only be further reduced by immediate therapy after clinical suspicion of giant cell arteritis.

Quinnine Amblyopia

The case of quinine amblyopia to be reported is considered of interest for several reasons. First, since the amblyopia occurred during a period of hospitalization for another condition, the opportunity arose for immediate, as well as continued, close ocular observation. Second, corticotropin (adrenocorticotropic hormone) was employed in the treatment of the quinine amblyopia. REPORT OF CASE A 48-year-old white man was admitted to the surgical service of the Veterans Administration Hospital, Jefferson Barracks, Mo., on Oct. 5, 1953, for treatment of a peripheral vascular disorder of the lower extremities, manifested by intermittent claudication and deficient pedal pulses. The patient was first seen by members of the ophthalmology department four days later, within 45 minutes after he developed sudden complete bilateral blindness, associated with dizziness, flushing, vomiting, tinnitus, and partial bilateral deafness. Review of the medication revealed that the patient had received a total of 0.8 gm. of oral quinine sulfate