Efficacy and Side Effects of Topiramate in Treatment of Children With Pseudotumor Cerebri Syndrome.

BACKGROUND: Topiramate is often considered as a second-line medication for the treatment of pseudotumor cerebri syndrome (PTCS), but limited studies exist that evaluate its efficacy in children. METHODS: Retrospective study of patients aged <21 years with PTCS who were treated with topiramate alone or in combination with acetazolamide was performed. Data regarding clinical courses and visual outcomes were recorded. RESULTS: A total of 46 patients were identified. Three (6.5%) patients were treated with topiramate alone, 31 (67.4%) transitioned to topiramate from acetazolamide, and 12 (26.1%) took both topiramate and acetazolamide concurrently. The median time to resolution of papilledema on topiramate was 0.57 years (interquartile range 0.32 to 0.84). Among eyes with papilledema graded on the Frisen scale at topiramate initiation, 40 of 57 (70.2%) were grade 1, nine of 57 (15.8%) were grade 2, and eight of 57 (14.0%) were grade 3. Twenty-seven of 46 (58.7%) reported headache improvement after starting topiramate. The mean dose of topiramate was 1.3 ± 0.8 mg/kg/day. The most common side effect was patient report of cognitive slowing (10 of 46 [21.7%]). All patients on topiramate monotherapy who were compliant with treatment and follow-up had resolution of papilledema with no evidence of visual function loss. CONCLUSIONS: Topiramate can effectively treat PTCS in children with mild to moderate papilledema or in those unable to tolerate acetazolamide. More research is needed to assess the efficacy of topiramate for higher grade papilledema.

Idiopathic Intracranial Hypertension in Female-to-Male Transgender Patients on Exogenous Testosterone Therapy.

PURPOSE: To present four female-to-male (FTM) transgender patients on testosterone therapy diagnosed with idiopathic intracranial hypertension (IIH). METHODS: The authors report 4 consecutive FTM transgender patients on exogenous testosterone diagnosed with IIH at a single institution. RESULTS: Patient 1 presented with progressive blurred vision and a central scotoma 10 weeks after starting testosterone cypionate injections for hormonal gender transition. Bilateral grade 5 papilledema was present; the patient underwent bilateral optic nerve sheath fenestration with improved vision and resolution of edema. Patient 2 presented with transient vision loss, pulsatile tinnitus, and blurred vision 13 months after starting testosterone cypionate injections. The patient had grade 4 and 3 disc edema of the right and left eyes, respectively. Patient 3 presented with headaches and pulsatile tinnitus and was on testosterone injections at an unknown dose. The examination revealed grade 1 and 2 disc edema of the right and left eyes, respectively. Patient 4 presented with decreased vision, transient visual obscurations, and daily migraines while using topical testosterone gel every other day. Color vision was reduced, and lumbar puncture revealed elevated intracranial pressure. All patients had neuroimaging findings consistent with increased intracranial pressure. CONCLUSIONS: Testosterone therapy plays an essential role in FTM hormonal transitioning and may play a role in IIH. Patients undergoing testosterone therapy for gender transition should be informed of the possibility of developing IIH while on treatment, with obesity possibly increasing this risk. Comprehensive eye examinations should be considered in these patients before initiating hormone therapy.

A high-pressure situation.

An 8-year-old boy presented with acute visual loss in the right eye and nausea, vomiting, and diplopia. Imaging revealed a right orbital apex mass. Biopsy showed Langerhans cell histiocytosis (LCH), and the patient was diagnosed with isolated orbital LCH causing an orbital apex syndrome. A 12-month cytarabine chemotherapy course was begun, during which the patient developed bilateral optic disc edema. He was diagnosed with cytarabine-induced intracranial hypertension, which was successfully treated with acetazolamide. The cytarabine course was completed with complete resolution of the LCH lesion. The ophthalmologic relevance of this rare disorder is discussed.

Bilateral optic disc edema as a possible complication of cabozantinib use-a case report.

INTRODUCTION: Cabozantinib, which was approved by the Food and Drug Administration (FDA) in 2012, is a tyrosine kinase inhibitor widely used in the treatment of metastatic renal cell carcinoma (RCC) and medullary thyroid carcinoma. To date, no ocular adverse events have been reported by the FDA or on the package label. Here, we described a patient with metastatic RCC who developed bilateral optic disc edema after a 4-month course of cabozantinib. CASE DESCRIPTION: A 55-year-old ethnic Chinese male with RCC with multiple metastases presented to our department with progressive blurred vision in both eyes for 1 month. He started taking cabozantinib 60 mg once daily 5 months prior to this presentation. Poor visual acuity and bilateral disc edema were then noted. Cabozantinib was discontinued after that, and 3-day pulse steroid therapy with methylprednisolone 1 g/day was given. The optic disc edema subsided gradually with limited improvement in visual acuity. CONCLUSION: Bilateral optic edema should be considered as a complication associated with cabozantinib. We propose discontinuation of the treatment in cases such as that, and pulse steroid therapy should be considered if there is no contraindication.

Silent progressive bilateral papillitis after COVID-19 vaccination: A case report.

RATIONALE: We report the case of a patient who developed asymptomatic bilateral papillitis after coronavirus disease 2019 (COVID-19) vaccination. PATIENT CONCERNS: A 61-year-old man presented to our tertiary clinic with bilateral optic disc edema, which was incidentally detected during his visit to a primary ophthalmology clinic. He had received an adenovirus-vectored COVID-19 vaccine 2 weeks before the optic disc edema was detected and had experienced no ocular discomfort, except for a floater in his right eye. Although his visual acuity was normal and he had no color vision deficiencies or marked visual field defects, the optic disc edema worsened over several days. Orbital magnetic resonance imaging showed no optic tract enhancement, and lumbar puncture revealed normal cerebrospinal fluid pressure. The patient tested negative for aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies and Leber hereditary optic neuropathy-associated gene mutations. DIAGNOSIS: The patient was diagnosed with bilateral papillitis, possibly induced by the COVID-19 vaccination. INTERVENTIONS: The patient received steroid pulse therapy with methylprednisolone (500 mg/day) for 3 days followed by an oral prednisolone taper for 3 weeks. OUTCOMES: The patient's papillitis started to subside 3 weeks after he received systemic steroid therapy and completely resolved without any sequelae 2 months later. A year after the diagnosis, the fundus remained stable without disease recurrence or optic disc atrophy. LESSONS: Healthy individuals receiving COVID-19 vaccines may present with various manifestations of optic neuritis. In the present case, the patient presented with asymptomatic progressive bilateral optic disc edema and had a favorable long-term course after receiving steroid therapy.

Papillitis and uveitis complicating Bacillus Calmette-Guérin immunotherapy.

Intravesical Bacillus Calmette-Guérin (BCG) administration was used to treat bladder carcinoma in a woman in her 60s. Severe bilateral non-granulomatous anterior uveitis and gross papillitis developed subsequently. The severe BCG-induced bilateral uveitis and papillitis were treated with high dose oral corticosteroids, with topical steroids and cycloplegics. Resolution of her ocular symptoms and signs eventuated. On lumbar puncture, no evidence of systemic spread of the BCG was found. Visual acuity returned to 6/9 in each eye with subsequent resolution of papillitis. Repeat cystoscopy demonstrated no evidence of recurrent bladder tumour.Hypersensitivity reactions are well recognised with Tubercle bacilli While both hypersensitivity reactions and dissemination of BCG throughout the body have been previously documented, the literature demonstrates that this case is the first example in which papillitis and bilateral uveitis were the prominent ophthalmological features.

Non-arteritic Anterior Ischemic Optic Neuropathy with Macular Star following COVID-19 Vaccination.

PURPOSE: To report a case of non-arteritic anterior ischemic optic neuropathy (NAAION) with macular star after receiving the second dose of SARS-CoV-2 vaccination. METHOD: Case report. OBSERVATION: A 51-year-old male presented with acute visual disturbances one day after the second dose of BNT162b2 mRNA SARS-CoV-2 vaccination. At presentation, best corrected visual acuity (BCVA) was 20/25 right eye (OD) and counting fingers at 3 feet left eye (OS). Anterior segment examination was normal in both eyes. Dilated fundoscopy was unremarkable OD, however, it disclosed optic nerve swelling and subretinal fluid OS. Patient was treated with a gradual tapering dose of oral prednisone over 1 month. At the five-week follow-up visit, optic disc swelling and subretinal fluid resolved with minimal improvement in BCVA to 20/400 OS. CONCLUSION: It is unclear whether COVID-19 vaccination was the triggering agent to the NAAION or just a coincidence, yet ophthalmologists should be aware of such a possible association.

Papilledema and idiopathic intracranial hypertension due to the possible potentiation of ATRA by posaconazole in a case of acute promyelocytic leukemia.

INTRODUCTION: Idiopathic intracranial hypertension (IIH) (pseudotumor cerebri) is a rare side effect of all-trans retinoic acid (ATRA). IIH cases have been observed after the concomitant use of ATRA with azole group antimicrobials such as fluconazole and voriconazole. Here, we discuss about the diagnosis and treatment process of the IIH emerging in a young acute promyelocytic leukemia (APL) case with the ATRA impact, which can be increased by posaconazole. CASE: A 19-year-old male patient was diagnosed with APL. Headache and blurred vision were developed on the 12th day of the AIDA (ATRA, 45 mg/m2/day, oral and idarubicin 12 mg/m2, on days 2, 4, 6, 8, intravenous) protocol and posaconazole proflaxis. He was diagnosed IIH along with the existing eye findings and imagings. MANAGEMENT & OUTCOME: ATRA treatment and posaconazole were interrupted. Systemic acetazolamide and dexamethasone treatment were initiated. After significant clinical response was observed, ATRA treatment was resumed without posaconazole and a similar clinical condition did not recur. DISCUSSION: The combined use of ATRA and azole group drugs increases the risk of developing IIH. Patients with APL who developed IIH during the concomitant use of ATRA and fluconazole or voriconazole have been reported. To the best of our knowledge, our case is the first APL case with a IIH who treated with ATRA-based therapy and used posaconazole. In case of development of side effects, drugs should be interrupted and this combination should be avoided if possible after appropriate approach and clinical improvement.

BILATERAL SEROUS RETINAL DETACHMENT AND UVEITIS ASSOCIATED WITH PEMBROLIZUMAB TREATMENT IN METASTATIC MELANOMA.

PURPOSE: To describe the pathological features, treatment, and resolution of pembrolizumab-associated retinal detachment. METHODS: A case report with a brief review of the literature and details of patient presentation, physical examination, systemic workup, fluorescein angiography, and indocyanine angiography. RESULTS: A 25-year-old white woman was diagnosed with unresectable metastatic melanoma of the skin with a BRAF V600E mutation. The patient was treated with pembrolizumab injections every 3 weeks, upon which quick remission was seen of the metastases. After five injections, visual acuity of the patient deteriorated to 20/32 in the right eye. Ocular examination revealed bilateral panuveitis, papillitis, and serous retinal detachments. Treatment consisted of an oral prednisone taper schedule, topical prednisolone drops, and cessation of the pembrolizumab therapy, after which complete resolution of the subretinal fluid was seen. CONCLUSION: Pembrolizumab therapy may cause the development of panuveitis, papillitis, and serous retinal detachment, symptoms which are able to be controlled with lengthy steroid therapy.

Growth Hormone Treatment and Papilledema: A Prospective Pilot Study

Objective: To investigate the incidence of pseudotumor cerebri syndrome (PTCS) in children treated with growth hormone (GH) in a paediatric hospital and to identify risk factors for this complication. Methods: Prospective pilot study of paediatric patients treated with recombinant human GH, prescribed by the Paediatric Endocrinology Department, between February 2013 and September 2017. In all these patients, a fundus examination was performed before starting treatment and 3-4 months later. Results: Two hundred and eighty-nine patients were included, of whom 244 (84.4%) had GH deficiency, 36 (12.5%) had short stature associated with small for gestational age, six (2.1%) had a mutation in the SHOX gene and three (1.0%) had Prader-Willi syndrome. Five (1.7%) developed papilledema, all were asymptomatic and had GH deficiency due to craniopharyngioma (n=1), polymalformative syndrome associated with hypothalamic-pituitary axis anomalies (n=2), a non-specified genetic disease with hippocampal inversion (n=1) and one with normal magnetic resonance imaging who had developed a primary PTCS years before. Conclusion: GH treatment is a cause of PTCS. In our series, at risk patients had GH deficiency and hypothalamic-pituitary anatomic anomalies or genetic or chromosomal diseases. Fundus examination should be systematically screened in all patients in this at-risk group, irrespective of the presence or not of symptoms.

Intravitreal Steroid Treatment for Uveitis Associated with Dabrafenib and Trametinib for Metastatic Cutaneous Melanoma.

Purpose: To report a case of bilateral retinal inflammation under long-term therapy with dabrafenib/trametinib for metastatic cutaneous melanoma.Methods: Retrospective chart review.Results: A 59-year-old patient with metastatic cutaneous melanoma diagnosed in 2004 under treatment with dabrafenib/trametinib since 2014 presented to our department with intraretinal hemorrhage and extrafoveal macula edema on the right eye and optic disc swelling on the left eye. The patient did not report visual complaints. After cessation of dabrafenib/trametinib and subconjunctival and intravitreal corticosteroid injections, optic disc swelling on the left eye recovered after 6 months. The macula edema on the right eye was treated with one intravitreal anti-VEGF (vascular endothelial growth factor) injection after encroaching upon the fovea 10 months after initial presentation. The final visual acuity was 20/20 on both eyes.Conclusion: Even after years of treatment with low dose dabrafenib/trametinib, ocular toxicity can develop. Such cases can respond well to intravitreal corticosteroids.

Ocular Adverse Effects of Amiodarone: A Systematic Review of Case Reports.

SIGNIFICANCE: Amiodarone is an excellent antiarrhythmic medication; however, it has numerous systemic and ocular adverse effects. PURPOSE: We aimed to improve our understanding of amiodarone and its ocular adverse effects by performing a systematic review and meta-analysis of published case reports. METHODS: This systematic review was reported in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. We used the MEDLINE database, primarily through PubMed, and used keywords (amiodarone, eye, eye diseases, visual/ocular adverse effects/manifestations) to identify case reports of ocular adverse effects after amiodarone use. The initial search resulted in 92 total case reports. However, after excluding nonrelevant case reports, 25 cases were selected for the final analysis. RESULTS: Among the patients in the 25 case reports, 18 were male (72%), and the median age was 66 ± 9.9 years. In 15 cases (60%), the patients reported halos around light and/or decrease in vision after amiodarone use. The most common ophthalmic examination findings were cornea verticillata/vortex keratopathy in 19 cases (76%), followed by different patterns of papilledema and retinal hemorrhages in 5 cases (20%). Discontinuation of amiodarone was the most common intervention, followed by application of topical heparin. Outcomes among case reports were variable. CONCLUSIONS: Cornea verticillata/vortex keratopathy was the most common ocular adverse effect in cases where amiodarone was administered. Early recognition of amiodarone-induced ocular adverse effects is imperative to prevent worsening keratopathy or uncommon adverse effects. Collaboration between physicians prescribing amiodarone-to recognize the ocular symptoms-and referral to eye care physicians are important.

Resolution of papilledema associated with cyclosporine use after change to tacrolimus.

Use of medications including vitamin A derivatives and tetracyclines have been associated with papilledema and raised intracranial pressure. A 46-year-old woman was referred to neuro-ophthalmology for bilateral optic disc oedema and had a 7-year history of cyclosporine use after renal transplantation. She had preserved visual function and moderate bilateral optic disc oedema. Magnetic resonance imaging and magnetic resonance venography of the brain were normal apart from signs of raised intracranial pressure. Lumbar puncture revealed an elevated opening pressure of 40 cm of water with normal cerebrospinal fluid contents. Nephrology was consulted and cyclosporine was switched to tacrolimus and she was treated with acetazolamide. The papilledema resolved within 1 month of her initial visit. It is important to recognise the role that cyclosporine plays in raising intracranial pressure, especially in patients requiring immunosuppression, such as transplant patients. Tacrolimus is a suitable alternative in these cases.