Bilateral Disc Edema Research Articles

Non-Blast Phase Chronic Myelogenous Leukemia Presenting as Bilateral Infiltration of the Optic Nerve.

A 27-year-old woman presented with bilateral blurry vision for 2 weeks. She had a presumed diagnosis of idiopathic intracranial hyper-tension given her bilateral disc edema; however, bilateral disc and retinal infiltration was noted on ophthalmoscopy. This report presents a rare case of chronic myelogenous leukemia (CML) in the non-blast phase presenting as bilateral optic disc infiltration, indicating central nervous system involvement at the time of diagnosis. The patient had no other clinical symptoms at the time of diagnosis and was promptly treated with orbital radiation and a tyrosine kinase inhibitor, resulting in 20/20 vision in both eyes. [Ophthalmic Surg Lasers Imaging Retina 2024;55:XX-XX.].

Sudden visual loss after COVID-19 infection with diabetic ketoacidosis

We report a case of an atypical optic neuritis (AION) in an adolescent male, diagnosed with diabetic ketoacidosis in the setting of a coronavirus disease 2019 (COVID-19) infection. On the third day of admission, he developed sudden profound visual loss and ocular examination revealed bilateral pale disc edema. Examination and investigations were conclusive of bilateral optic neuritis. Optic neuritis in the setting of pale disc edema was unusual, and the pallor could be due to an episode of AION that was superadded by optic neuritis during the cytokine storm. The patient was given a trial of i.v. steroids. However, the vision loss could be recovered partially only. Thus, through this case report, we would like to highlight the importance of a close follow-up of patients following COVID-19 infection and a possible association of metabolic acidosis in precipitating the episode of optic neuritis.

Multimodal Imaging in Diagnosis of Vogt Koyanagi Harada Disease with Reference to Choroid and Retinal Pigment Epithelium

A 48 year dark skinned North Indian male presented first time to our outpatient with history of bilateral visual decline of 3 weeks duration with associated features of headache, myalgia and rhinitus of 4 weeks duration. A detailed examination confirmed bilateral active anterior granulomatous uveitis with bilateral disc oedema with serous retinal detachment. Multimodal imaging examination was carried out with nideks mirante. Diagnosis in favour of vogt koyanagi harada disease was confirmed. The posterior segment was assessed for various changes in acute and remission phase of vogt koyanagi harada disease with fundus fluorescein angiography, fundus autoflourescence, retroillumination and optical coherence tomography. Thickness and structural change related to central macula thickness, retinal pigment epithelium and choroid were assessed pre and post treatment with additional support of optical coherence tomography and retroillumination. Both these techniques were able to document a greater value change pre and post treatment in these structures. Hence we conclude the need to include these techniques in retinal pigment epithelium and choroidal assessment in vogt koyanagi harada disease.

Devic’s disease: Neuromyelitis optica spectrum disorder – A case series

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that mainly involves the optic nerve and the spinal cord. Purpose of this case series is to better understand the clinical profile of patients with NMOSD, its treatment, and prognosis and to differentiate them from patients having other demyelinating disorders. Five patients (one male and four females) aged between 18 and 65 years were studied. All of them presented with severe vision loss. Bilateral optic nerve involvement was seen in four patients and unilateral involvement in one patient. One patient presented with only optic nerve involvement, two presented with initial optic nerve involvement followed by neurologic symptoms, while two patients presented with neurologic symptoms initially followed by optic nerve involvement. One patient was Neuromyelitis optica antibody negative and was diagnosed based on magnetic resonance imaging features. Two patients had optic atrophy at presentation itself, one had bilateral disc edema, and two had retrobulbar optic neuritis. All were treated with corticosteroids and immunosuppressants.

The Increasing Burden of Emergency Department and Inpatient Consultations for "Papilledema".

Increasing incidence of idiopathic intracranial hypertension (IIH), overreported radiologic signs of intracranial hypertension, difficult access to outpatient neuro-ophthalmology services, poor insurance coverage, and medicolegal concerns have lowered the threshold for emergency department (ED) visits for "papilledema." Our objective was to examine referral patterns and outcomes of neuro-ophthalmology ED and inpatient consultations for concern for papilledema. At one university-based quaternary care center, all adults referred for "papilledema" over one year underwent a standardized ED "papilledema protocol." We collected patient demographics, final diagnoses, and referral patterns. Over 1 year, 153 consecutive patients were referred for concern for papilledema. After papilledema protocol, 89 of 153 patients (58%) had bilateral optic disc edema, among whom 89% (79/89) had papilledema (intracranial hypertension). Of the 38 of 153 (25%) consultations for suspected disorder of intracranial pressure without previous fundus examination (Group 1), 74% (28/38) did not have optic disc edema, 21% (8/38) had papilledema, and 5% (2/38) had other causes of bilateral disc edema. Of the 89 of 153 (58%) consultations for presumed papilledema seen on fundus examination (Group 2), 58% (66/89) had confirmed papilledema, 17% (15/89) had pseudopapilledema, and 9% (8/89) had other causes of bilateral optic disc edema. Of the 26 of 153 (17%) patients with known IIH (Group 3), 5 had papilledema and 4 required urgent intervention. The most common diagnosis was IIH (58/79). Compared with IIH, patients with secondary causes of intracranial hypertension were older (P = 0.002), men (P < 0.001), not obese (P < 0.001), and more likely to have neurologic symptoms (P = 0.002). Inpatient and ED consultations for "papilledema" are increasing. Of the 153 ED and inpatient neuro-ophthalmology consultations seen for "papilledema" over 1 year, one-third of patients with optic disc edema of unknown cause before presentation to our ED had new vision- or life-threatening disease, supporting the need for prompt identification and evaluation of optic disc edema in the ED. In the face of limited access to neuro-ophthalmologists, this study supports the need for emergency department access to expert eye-care evaluation or ocular fundus camera for prompt identification of optic disc edema and standardized evaluation for neurologic emergencies.

Diabetic papillopathy: A rare ocular manifestation of diabetes mellitus

Abstract Diabetic papillopathy (DP) is a rare ocular manifestation of diabetes mellitus. The exact etiology is not well understood, but in some cases, it may be associated with a rapid correction of the blood glucose level. There are limited case reports describing the use of intravitreal anti-vascular endothelial growth factor antibodies which have shown effectiveness in reducing disc swelling. In this case, we present the case of an 80-year-old diabetic woman diagnosed with DP during a diabetic retinopathy (DR) screening. Her visual acuity (VA) was counting fingers on her right eye and 20/160 on her left eye. At the fundoscopy, an exuberant bilateral hemorrhagic disc edema was observed, along with a nonproliferative DR. This edema was also evident in the fluorescein angiography and was documented through the optical coherence tomography. Brain computed tomography normal. She was treated with ranibizumab with significant improvement of the optic disc edema and the VA.

Bilateral uremic optic neuropathy in a patient with chronic kidney disease

A 62-year-old man with chronic kidney disease presented with complaints of sudden-onset painless loss of vision in both eyes for 4 days. The vision was reduced to hand movements close to the face bilaterally. A clinical examination revealed bilateral disc edema. Blood urea and serum creatinine levels were elevated. The patient was treated with systemic steroids and hemodialysis. One month following the presentation, best-corrected visual acuity (BCVA) had improved to 6/12, N12 in both eyes.

Bilateral optic neuritis with complete ophthalmoplegia: An extremely rare complication following COVID-19 (Covishield) vaccination

A 35-year-old healthy female presented with decreased vision, headache, painful and restricted ocular movement 5 days following the second dose of coronavirus disease 2019 (COVID-19) (Covishield) vaccination. Her best-corrected visual acuity was 6/36 in both eyes, and color vision was defective. Dilated fundus showed bilateral disc edema. She developed complete ophthalmoplegia 2 days following initial presentation. Imaging studies and systemic investigations were normal. She was treated with systemic corticosteroids. Complete recovery was noted 1 month following presentation. It is critical to raise awareness about the different complication following different COVID vaccinations, but equally important is a timely and justified treatment which would lead to complete recovery.

Bilateral optic disc edema as the presenting sign of brain metastasis

To report a rare case of bilateral optic disc edema as a presentation of brain metastasis. Case report. A 51-year-old healthy woman presented with complaints of headache, diplopia, and distortion of peripheral vision for the past one month. Examination of both the eyes showed massive optic disc edema, multiple radial hemorrhages, hard exudates at the disc margin, and absent venous pulsation. Magnetic resonance imaging (MRI and MRA) showed leptomeningeal enhancement with raised intracranial pressure: a sign of metastasis. She had undergone mastectomy and had received eight cycles of systemic chemotherapy and radiation for breast carcinoma two years ago. She was urgently referred to an oncologist for further management. This case highlights the importance of neuroimaging in patients presenting with bilateral disc edema and prior history of breast carcinoma to rule out metastasis.

Bilateral disk edema with retinitis pigmentosa: Diagnostic dilemma

Bilateral disk edema in a case of retinitis pigmentosa can cause diagnostic confusion and delay in treatment. Existing literature shows few such cases where inflammatory pathology is postulated. A battery of tests may be required to reach a definitive diagnosis. Here, we will report a case of retinitis pigmentosa with bilateral disk edema due to malignant hypertension. Even in the presence of high systemic blood pressure, diagnostic dilemma can occur in such cases due to overlapping clinical features like arterial attenuation, decreased vision due to optic atrophy, and macular edema. Hence, other differential diagnoses like inflammatory causes and intracranial pathology should be carefully ruled out.

Gauging the pressure: The clinical dilemma with Chiari! (P11-4.004)

<h3>Objective:</h3> N/A <h3>Background:</h3> Differentiating between Chiari Malformation I (CMI) and acquired tonsillar herniation due to spontaneous or iatrogenic intracranial hypotension (IH) can be challenging. We describe a case of IH misdiagnosed as CM1 to emphasize several distinct clinical signs that can prevent misdiagnosis. <h3>Design/Methods:</h3> <h3>Case Description:</h3> A 36-year-old multigravida female with history of multiple epidural analgesia during vaginal deliveries presented with 6 months of constant, poorly localized headaches. Fundoscopic exam revealed bilateral mild inferior disc edema without vessel obscuration. Contrasted magnetic resonance imaging (MRI) showed diffuse pachymeningeal enhancement with a 7mm downward herniation of the cerebellar tonsils but no syrinx in the upper cervical cord. The patient was thought to have CM1, and she was treated with supportive care. After 2 years, the patient again presented with severe intractable headaches and vomiting 7 weeks following epidural for vaginal delivery. Repeat MRI brain showed a 23 mm downward herniation of the cerebellar tonsils associated with severe effacement of the basilar and prepontine cisterns and diffuse sulcal effacement. MRI cervical spine showed a large cervical syrinx at this time. MRI thoracic and lumbar spine showed an epidural cerebrospinal fluid (CSF) collection extending from T1 to L3 levels with diffuse cord edema, confirming CSF leak. Given high risk of complications, blood patch was not attempted, and the patient ultimately underwent suboccipital decompressive craniotomy. <h3>Results:</h3> N/A <h3>Conclusions:</h3> This case highlights the importance of considering acquired causes of cerebellar tonsillar herniation in symptomatic adults. Headache description, chronicity, and presence/absence of syrinx cannot differentiate CMI from IH. Pachymeningeal enhancement, subdural hygromas, pituitary enlargement, distention of cerebral venous sinuses, and effacement of subarachnoid spaces along with flattening of pons are more indicative of IH. Evidence of an extradural CSF collection in the spinal cord or a CSF leak on computerized tomography myelogram are diagnostic of IH. <b>Disclosure:</b> Miss Phillips has nothing to disclose. Dr. Mannyam has nothing to disclose. Dr. Sharma has nothing to disclose.

Ocular manifestations of DiGeorge syndrome

A 20-year-old woman with facial features of DiGeorge syndrome came for a regular ophthalmic examination. She had hypoparathyroidism with hypocalcemic tetany and severe anemia but no cardiac anomalies. On ophthalmic examination, the visual acuity was 6/6 OU. She had bilateral eyelid hooding, ptosis, esophoria, posterior embryotoxon, and dilated, tortuous vessels with disc edema in the fundus. Bilateral vascular tortuosity and disc edema could mimic established papilledema or plus disease, which has significant treatment implications. Hence knowledge of ocular and systemic features is essential in providing recommendations for DiGeorge syndrome.

Vitreous Hemorrhage from Papilledema After Neck Massage

A 41-year-old woman presented with left eye floaters after receiving a head and neck massage while prone. The patient had a history of idiopathic intracranial hypertension, treated with acetazolamide. Examination revealed a left inferior optic disc hemorrhage and bilateral optic disc edema (A-B). OCT of the left optic nerve depicted retinal nerve fiber edema with hyperreflectivity in the preretinal space extending into the vitreous (C). OCT retinal nerve fiber layer confirmed bilateral disc edema (D). This case represents active disc hemorrhage observed on OCT imaging secondary to idiopathic intracranial hypertension and exacerbated by pressure from massage, which induced venous rupture on the optic disc (Magnified version of Figure A-D is available online at www.aaojournal.org).

Prospective Study of Clinical Characteristics and Visual Outcome in Vogt–Koyanagi–Harada Syndrome in a Tertiary Eye Hospital in South India

Purpose: To describe the demographic pattern, clinical characteristics, treatment modalities, and visual outcome in Vogt–Koyanagi–Harada (VKH) disease patients observed at a tertiary eye care centre in South India. Methods: A prospective chart analysis of 38 patients with VKH disease. Results: Of the 38 VKH patients, 31 (81.6%) were female, the mean age was 39.8 years, and the median duration of symptoms was 10 days (range: 2 to 180 days). The most common ocular complaint was a defective vision (70 eyes or 92.1%). Extraocular manifestations were headache (13 patients or 34.2%) and tinnitus (4 patients or 10.5%). Twenty-four patients (63.2%) presented with bilateral panuveitis, and 12 patients (31.6%) had posterior uveitis. The most common posterior segment presentation included bilateral serous retinal detachment (100%), disc oedema (37 eyes or 48.7%), and vitritis (31 eyes or 40.8%). All patients (100%) were treated with oral and topical prednisolone, based on the ocular inflammation status, 9 patients (23.7%) required dexamethasone (intramuscular), 39 eyes (51.3%) required posterior subtenon triamcinolone, 16 patients (28.9%) required methotrexate, 1 (2.6%) required cyclophosphamide, 1 (2.6%) required azathioprine. At the final visit, the majority of the patients had statistically significant good visual outcomes. Conclusion: Prompt diagnosis and early initiation of treatment with systemic, local steroid and immunosuppressive therapy showed faster recovery with good visual outcome.

A Case of Idiopathic Intracranial Hypertension with Papilledema Secondary to Recombinant Human Growth Hormone Treatment

Purpose: To report a child with idiopathic intracranial hypertension secondary to recombinant human growth hormone treatment.Case summary: An 11-year-old girl presented with blurred vision in both eyes starting 6 weeks earlier. She did not have any underlying disease and her body mass index was normal. She had started recombinant human growth hormone injections for idiopathic short stature 3 months earlier. The best corrected visual acuity was 20/25 in both eyes. Fundoscopy revealed bilateral disc edema with peripapillary hemorrhage and increased tortuosity of the retinal vessels. Bilateral enlargement of the blind spot was found on automated visual field examination. Magnetic resonance imaging and venography of the brain showed no evidence of structural or vascular lesions related to increased intracranial pressure. A lumbar puncture showed an elevated opening pressure of 26 cmH2O with normal cerebrospinal fluid constituents. She was diagnosed with growth hormone-related idiopathic intracranial hypertension and it was recommended that the growth hormone injection be discontinued and oral acetazolamide started. After 4 weeks of treatment, the optic disc edema and visual field defect improved. At the 2-year follow-up, she had a normal visual field with a normal optic nerve in both eyes.Conclusions: Ophthalmologists should be aware of the clinical features and treatment of idiopathic intracranial hypertension secondary to recombinant human growth hormone treatment, which is a very rare, vision-threatening complication. Complete neuro-ophthalmological examinations should be performed immediately in children complaining of visual disturbances or headache during treatment with recombinant human growth hormone.

Pseudotumor Cerebri in the Setting of Differentiation Syndrome in a Patient with Acute Promyelocytic Leukemia Treated with All-trans Retinoic Acid During Induction Therapy

Introduction: Differentiation syndrome, a well-known complication of all-trans retinoic acid (ATRA) in patients with acute promyelocytic leukemia (APML), can very rarely have ophthalmic manifestations. Pseudotumor cerebri (PC) in the setting of differentiation syndrome (DS) in patients undergoing induction with all-trans retinoic acid has rarely been reported elsewhere. We herein report one such case. Case Presentation: A 28-year-old, non-obese female diagnosed as acute promyelocytic leukemia underwent induction with all- trans retinoic acid and Idarubicin. On day 4 of the treatment, she developed high grade fever (104 – 105 F), dry cough, hypotension, tachycardia, and tinnitus. Chest X ray showed floppy shadows in bilateral lungs. On physical examination, bilateral lower limb edema was noted. She also experienced sudden weight gain of 5 kilogram in 48 hours. After careful exclusion of systemic infection, she was suspected as having DS. She also noticed a reduction in vision in right eye. On eye examination, her best-corrected visual acuity (VA) was 6/60 in the right eye (RE) and 6/6 in the left eye (LE). Fundus evaluation revealed bilateral disc edema with peripapillary hemorrhages along with slight tortuosity of vessels and a yellowish lesion over the fovea in RE. Suspecting DS, she was treated with injection dexamethasone 10 mg twice daily and all- trans retinoic acid was temporarily discontinued. Immediately after its discontinuation, her headache lessened and vision improved gradually. After 2 weeks, her VA was 6/12 in RE and 6/6 in LE which improved to 6/6 in both eyes at 3 months. The patient was also receiving oral voriconazole for fungal prophylaxis and the potentiation effect of all- trans retinoic acid could thus be explained. Conclusion: Pseudotumor cerebri associated with all-trans retinoic acid treatment in acute promyelocytic leukemia, even though frequently reported in pediatric patients, is rare in adults. Ophthalmological evaluation is mandatory in all these patients under all-trans retinoic acid therapy.

Rare ocular manifestation of papilledema and bilateral choroidal tubercles in disseminated miliary tuberculosis

An 11-year-old female on treatment for miliary tuberculosis presented with decreased vision. Examination revealed bilateral disc edema, granulomatous uveitis, and multiple choroidal tubercles. Oral steroids were added. She was lost to follow-up, and 3 months later she had further visual loss due to optic atrophy. Repeat imaging of the CNS showed regression of the brain parenchymal lesions. This is a rare presentation of ocular tuberculosis in the setting of disseminated TB. While the mainstay of therapy is antitubercular therapy, this case highlights the importance of systemic corticosteroids to decrease both ocular and CNS inflammation and halt further vision loss.

Bilateral Optic Disc Edema in Multisystem Inflammatory Syndrome in Children Associated With COVID-19.

Bilateral Optic Disc Edema in Multisystem Inflammatory Syndrome in Children Associated With COVID-19.

Ultrasound monitoring for minocyclineinduced idiopathic intracranial hypertension

We report a rare case of idiopathic intracranial hypertension following oral minocycline therapy for the treatment of acne. A 29-year-old, non-obese female, with a history of minocycline use for 1 month for treatment of acne presented with headache and transient blurred vision for 3 weeks. She was found to have bilateral disc edema with normal visual acuity and color vision. Magnetic resonance imaging of the brain was normal with partially empty sella features and enlarged tortuous optic nerve in both eyes. Cerebrospinal fluid opening pressure was high. Ultrasound B-scan was done to serially monitor the optic nerve sheath diameter. She improved significantly after stopping the minocycline and following intracranial pressure lowering measures. Idiosyncratic reaction of intracranial hypertension with minocycline can be symptomatic as early as 1 week. Consultants should be aware of this as early consult with ophthalmologists/neurologists can prevent visual loss. A simple ultrasound B-scan can prove to be a vital non-invasive tool in monitoring these patients.

Transient bilateral disc edema with spontaneous resolution following spine surgery

A 49-year-old man presented with flashes and transient obscuration of vision in both eyes 1 week after spine surgery for disc prolapse. Best-corrected visual acuity was 20/20 in both eyes. Fundoscopy showed bilateral disc edema. All investigations were normal. There was spontaneous resolution of bilateral disc edema 2 months following presentation.