Ophthalmologic Exam Research Articles

A Randomized, Double-Blind, Placebo-Controlled Phase 3 Study to Evaluate the Safety and Efficacy of XEN1101 as an Adjunctive Therapy in the Treatment of Primary Generalized Tonic-Clonic Seizures (P11-1.012)

A Randomized, Double-Blind, Placebo-Controlled Phase 3 Study to Evaluate the Safety and Efficacy of XEN1101 as an Adjunctive Therapy in the Treatment of Primary Generalized Tonic-Clonic Seizures (P11-1.012)

From (Cat) Scratch: A Unique Presentation of CNS Bartonella Infection (P2-10.003)

<h3>Objective:</h3> NA <h3>Background:</h3> Central Nervous System (CNS) Bartonella infection is rare. Diagnosis is usually performed with serology and confirmed with polymerase chain reaction (PCR). We describe a unique case of disseminated necrotizing brain abscess in an immunocompromised patient caused by Bartonella. <h3>Design/Methods:</h3> A 28-year-old woman with medical history of Systemic Lupus Erythematosus (SLE) on chronic immunosuppression presented with four months of progressive, bilateral, painless vision loss. Two months before presenting to ED, her ophthalmologist increased her prednisone regimen to 80 mg daily due to concern for lupus retinitis. On admission, her ophthalmologic exam was pertinent for decreased visual acuity (20/50 in the right eye and 20/400 in the left eye), chorio-retinal inflammatory lesions and macular edema. MRI of the brain revealed numerous contrast-enhancing lesions throughout the brainstem, cerebral and cerebellar hemispheres. She was started on empiric therapy with ceftriaxone, ganciclovir, voriconazole, pyrimethamine, and sulfadiazine. Infectious etiologies, including HSV, EBV, HIV, mycobacteria, Histoplasma, Blastomyces, Cryptococcus, Toxoplasma were ruled out. CSF showed 1 WBC, 35 Protein, 76 Glucose, flow cytometry and cytology were negative for CNS lymphoma. She was tested for Bartonella henslae and quintana her IgM was negative; IgG was 1:64 (equivocal) and 1:256 (positive), respectively. Due to the lack of clinical improvement, the patient underwent biopsy of the brain lesions with PCR confirming Bartonella infection. She was started on doxycycline and rifampin and discharged home. Brain MRI at 5 weeks post-discharge showed decreased number and size of lesions. <h3>Results:</h3> NA <h3>Conclusions:</h3> We emphasize the consideration of CNS Bartonella infection as an etiology of multiple brain lesions in immunocompromised patients. We encourage evaluation for Bartonella infection if standard infectious workup is negative and there is no improvement with empiric treatment. Prompt diagnosis is important as treatment can improve morbidity and mortality. <b>Disclosure:</b> Dr. Rodriguez has nothing to disclose. Ms. Fedorova has nothing to disclose. Dr. Rukmangadachar has nothing to disclose.

Cocaine Orbitopathy Complicated by Orbital Wall Defects, Bilateral Naso-Orbital Fistulas, Phthisis, and Bilateral Vision Loss.

A 64-year-old male with a 40-year history of daily intranasal cocaine use presented with painless, gradually worsening binocular diplopia. External examination showed a severe saddle-nose deformity. Ophthalmologic exam was notable for visual acuity of 20/40 in OU, with restrictive strabismus, esotropia, and hypotropia in the OD. Neuroimaging revealed extensive nasal septal destruction with erosion of bilateral medial and orbital floors. The patient was lost to follow-up and presented 3 years later with progression of vision loss, now light perception vision OD and count fingers vision OS. There was now complete absence of the nasal bridge and medial orbital walls with large naso-orbital fistulas bilaterally, and phthisis of the right globe with complete corneal neovascularization. Laboratory work-up, including perinuclear anti-neutrophil cytoplasmic antibodies, cytoplasmic anti-neutrophil cytoplasmic antibody, erythrocyte sedementation rate, c-reactive protein, and chest X-ray, were unremarkable. The patient continually refused drug testing. Repeat neuroimaging revealed progression of the nasal midline destruction and further loss of the medial and orbital floors (panels A and B). These findings were consistent with anatomic destruction of the orbital bony walls and subsequent ocular necrosis, perforation, and ischemia related to long-term cocaine use (panel C). Cocaine use can result in tissue damage through its alpha agonist and inflammatory properties leading to a prothrombotic state, vasoconstriction, and subsequent ischemia. The authors present a severe case of presumed cocaine orbitopathy resulting in extensive midline structural loss, destruction of the medial and inferior orbital walls, naso-orbital fistulas, and subsequent phthisis bulbi resulting in vision loss.

Insights into the genotype–phenotype relationship of ocular manifestations in Kabuki syndrome

We aim to assess if genotype-phenotype correlations are present within ocular manifestations of Kabuki syndrome (KS) among a large multicenter cohort.We conducted a retrospective, medical record review including clinical history and comprehensive ophthalmological examinations of a total of 47 individuals with molecularly confirmed KS and ocular manifestations at Boston Children's Hospital and Cincinnati Children's Hospital Medical Center. We assessed information regarding ocular structural, functional, and adnexal elements as well as pertinent associated phenotypic features associated with KS. For both type 1 KS (KS1) and type 2 KS (KS2), we observed more severe eye pathology in nonsense variants towards the C-terminus of each gene, KMT2D and KDM6A, respectively. Furthermore, frameshift variants appeared to be not associated with structural ocular elements. Between both types of KS, ocular structural elements were more frequently identified in KS1 compared with KS2, which only involved the optic disc in our cohort. These results reinforce the need for a comprehensive ophthalmologic exam upon diagnosis of KS and regular follow-up exams. The specific genotype may allow risk stratification of the severity of the ophthalmologic manifestation. However, additional studies involving larger cohorts are needed to replicate our observations and conduct powered analyses to more formally risk-stratify based on genotype, highlighting the importance of multicenter collaborations in rare disease research.

A Rare Report of the Coexistence of Sickle Cell Disease, Neurofibromatosis Type 1, and Intracranial Hypertension in a Pediatric Patient.

A pediatric female with sickle cell disease (SCD) and neurofibromatosis type 1 was noted to have incidental papilledema, with subsequent workup showing an elevated opening pressure. She was diagnosed with intracranial hypertension and began treatment with acetazolamide. Hydroxyurea was also discontinued. Acetazolamide was tapered off, and hydroxyurea was restarted with no worsening in her ophthalmologic exam. We report this case due to the rare occurrence of all 3 conditions, and while intracranial hypertension has been reported in SCD, the diagnostic workup for papilledema in hemoglobinopathies is not well defined. This case helps delineate the presentation and diagnostic workup of papilledema in SCD.

Posterior Staphyloma as Determining Factor for Myopic Maculopathy

The aim of this study was to determine the influence of posterior staphyloma (PS) on the development of myopic maculopathy. Cross-sectional study. 467 highly myopic eyes (axial length (AL) ≥26 mm) of 246 patients were included. Patients underwent a complete ophthalmological exam, including multimodal imaging. Presence of PS was defined as the main variable analysing between groups (PS vs. non-PS): age, AL, best-corrected visual acuity (BCVA), Atrophy/Traction/Neovascularization (ATN) components and presence of severe pathologic myopia (PM). Two different cohorts were studied (age-matched and AL-matched) comparing PS vs. non-PS eyes. Of the total, 325 eyes (69.59%) presented PS. Eyes without PS were younger and had lower AL, ATN-components and prevalence of severe PM than those with PS (p<0.001). Moreover, non-PS eyes had better BCVA (p<0.001). Analysing age-matched cohort (p=0.96); mean AL, A and T components, and severe PM were significantly higher in PS group (p<0.001), as well as N component (p<0.005), showing worse BCVA (p<0.001). Regarding AL-matched cohort (p=0.93); PS group showed worse BCVA (p<0.01), older age (p<0.001), A (p<0.001) and T components (p<0.01), and severe PM (p<0.01). The risk of PS increases by 10% per year-old (odds ratio: 1.109, p<0.001) and by 132% per each mm growth of AL (odds ratio: 2.318, p<0.001). Posterior staphyloma is associated with myopic maculopathy, worse visual acuity, and higher prevalence of severe PM. The AL and age, following this order, constitute the main factors associated with the onset of PS.

Cystinosis in a routine ophthalmologic evaluation

Introduction: Cystinosis is a rare autosomal recessive metabolic disease. It occurs due to a chromosomal mutation that allows the accumulation of this amino acid in lysosomes. Cystine can form crystals in various tissues such as the kidneys, pancreas, thyroid, testicles, bone marrow, brain, eye, and muscles. In the eyes, crystals can cause deposits on the cornea and conjunctiva. In the cornea, deposits occur in the periphery and anterior stroma. As the disease progresses, the crystals move centripetally and posteriorly. Oral and topical treatments with cysteamine have been described. The ophthalmologic solution of cysteamine 0.55% used continuously every 2 hours has shown good results. Clinical Case: Patient in a routine ophthalmologic exam, with findings of cystine crystals in the cornea and past kidney transplantation without diagnosis. Discussion: The patient’s past history is relevant and corroborates with the ophthalmological findings. The patient had severe kidney disease, requiring kidney transplantation, but the disease was not elucidated. Therefore, the ophthalmological examination was fundamental for the diagnosis of the case

TEMPORAL LOBE HERNIATION AND CHIASMOPATHY DURING DOPAMINE AGONIST THERAPY FOR PROLACTINOMA.

Dopamine agonists (DA) are first line treatment for prolactinomas. Optic chiasm herniation can rarely occur during therapy, while brain herniation is very uncommon. A 34 yo woman presented with headaches and vision changes. Prolactin (PRL) was 4300 ng/mL. MRI showed a 4.5 cm pituitary adenoma with chiasm compression. After 3 months, PRL decreased to 201 ng/mL while patient was taking CAB 0.75 mg twice a week. MRI showed ~30% tumor reduction with medial temporal lobe herniation and encephalocele. CAB was stopped and she underwent surgical debulking and encephalocele repair. Histopathology confirmed prolactin tumor. CAB 0.75 mg twice a week was resumed.A 50 yo man had incidental detection of a sellar mass after trauma. MRI showed 3.6 cm tumor with minimal contact of right optic nerve, and PRL 3,318 ng/ml. He received CAB 0.5mg twice a week with PRL improvement to 26 ng/mL after 1 month. After 2 months ophthalmology exam showed new left superotemporal depression. PRL was 68 ng/mL and MRI showed 35% mass reduction and new inferior displacement tethering of the chiasm. CAB dose was decreased to 0.25 mg twice a week. Our cases illustrate that rapid biochemical and radiographic response to DA therapy in large prolactinomas warrants close clinical and neuro-ophthalmologic follow-up. We recommend repeating the MRI 3 months after initiation of DA therapy or sooner in case of new mass effect manifestations. Decision regarding DA dose reduction or chiasmopexy for visual field deficits needs to be multi-disciplinary and on a case-to-case basis.

Refractive Error and Ocular Biometry among Young Adults from Cuiabá, Brazil

Objective To investigate the distribution of refractive errors and their association with ocular biometric data, family history and environment variables in medical university students of first to six semesters of UNIVAG - MT. Methods A general ophthalmological exam was performed including, corneal topography, and ocular optical biometry. Lens power has calculated by Bennett and Rozema’s formula. A questionnaire regarding family history of myopia and lifestyle visual activities was applied. Only university students with normal ophthalmological exams were included. Statistical significance was considered at the level of p&lt;0.05. Results One hundred twenty-eight students were selected, whereas other 13 were excluded. The mean age was 21.28 ± 2.18 years. Forty-four (34.4%) participants were males. Regarding refractive errors, 18 (14.1%) were hyperopic, 41 (32%) were emmetropic, 61 (47.7%) were myopic, and 8 (6.3%) were high myopic. The mean and standard deviations of spherical equivalents, axial lengths, keratometries (K1 and K2), and lens powers were -1.27 ± 2.21 D, 24.17 ± 1.07 mm, 42.90 ± 1.25 D, 43.94 ± 1.37 D, and 22.62 ± 1.73 D, respectively. In relation to the family history of myopia, one parent was myopic in 28 (50%) of subjects, and both parents were myopic in 7 (12.5%) subjects. The average of hours per week spent outdoors were 5.82 +/- 7.38 hs. and spent 6.85 +/- 6.26 hs. at sports activities at night. Conclusion Myopia was the most frequent refractive error among the medical university students subject of this study, and was approximately three times higher than those reported for other samples of Brazilian population. There was a positive correlation between refraction errors and axial length.

Pediatrik Beta-Talasemi Majörde Vitreus Kamara Uzunluğu, Korneal Hacim ve Glob Biyometrisinin Kesitsel Vaka Kontrollü Entegre Analizi

Objective: To determine ocular changes, particularly in vitreous chamber length (VCL) and corneal volume (CV), analyze globe biometric features in emmetrope pediatric beta-thalassemia major (βTM) patients using Cirrus topography and IOLMaster devices, and compare the results to age- and gender-matched healthy individuals. Material and Methods: This cross-sectional case-control study included 36 multi-transfused β-TM patients (Group 1, 72 eyes) with a mean age of 9.18±3.14 years and no other hemoglobinopathies or anemias unrelated to β-TM. A control group (Group 2, 72 eyes) included 36 healthy children who had routine ophthalmology exams. A comprehensive ophthalmologic exam was performed, including auto-refraction, best-corrected visual acuity, and intraocular pressure (IOP), followed by dilated slit-lamp biomicroscopy. The corneal topography and globe biometric evaluation were followed by a pair-wise data comparison. Results: The axial length (AL) (22.58±0.64 vs 23.06±0.71 mm), VCL (15.48±0.68 vs 15.92±0.69 mm), CV (55.47±2.95 vs 57.65±2.83 mm3), IOP (12.68±2.34 vs 11.08±1.68 mmHg), keratometry values (K1, K2, Kmean, Kapex), as well as central corneal thickness (523.00±28.41 vs 547.29±26.45 μm), were all significantly different between groups 1 and 2, respectively (p0.05). Conclusion: β-TM patients appear to have significant ocular growth retardation than relatively age-matched healthy children, as demonstrated by shorter AL and VCL, as well as lower CV. This circumstance could have prompted compensatory biometric modifications, as evidenced by a relatively steeper cornea and thicker crystalline lens, to accomplish emmetropization.

Optic neuropathies post-Covid 19 - review.

The Corona virus infection started at the end of 2019 in Wuhan - China and spread rapidly throughout the world, generating the Covid 19 pandemic. The manifestations of the Covid disease were extremely varied, from a simple flu, with fever, cough, weakness, headache, joint pain, up to severe pneumonia, with severe acute respiratory syndrome (SARS-Cov2) and even death. The symptomatology of the disease, the evolution and the complications that appeared varied, depending on the associated pathology - diabetes mellitus (DM), hypertension (HT), the age and the immune status of the patient. Aim: The ocular manifestations related to Covid 19 were mostly represented by conjunctivitis, but the neurotropic character of Corona virus could justify the appearance of certain neuro-ophthalmological manifestations, such as: optic neuritis (ON), cranial nerve palsies, visual field (VF) anomalies. The aim of this paper was to research the cases of optic neuropathy post-Covid 19, published in the specialty literature between 2020 and 2022. The following were evaluated: risk factors, distribution by age group and gender, evolution and complications, as well as the clinical forms of optic neuropathies. Materials and methods: We used Google Scholar and PubMed databases to find articles on optic neuropathies related to the Covid-19 infection. We followed the articles published during the pandemic and selected 21 cases, belonging to 17 authors, irrespective of their origin and the language in which they were written. Results: 21 patients affected by ON in the Covid-19 disease, 11 women and 10 men, were mentioned. The optic neuropathies described by the authors were: retrobulbar optic neuropathy, only one associated with myelin oligodendrocyte glycoprotein (MOG), papillitis, neuroretinitis, anterior ischemic optic neuropathy (AION), out of which one arteritic anterior ischemic optic neuropathy (AAION) and the others non-arteritic anterior ischemic optic neuropathy (NAAION), one being related to pronation in an oro-tracheal intubated (OTI) patient with acute respiratory distress syndrome (ARDS). Discussions: The neuro-ophthalmological complications associated with Covid 19 disease can be severe, so the patients should be monitored continuously. Many investigations (serological, immunological and imaging exams) are necessary to exclude other etiologies of ON. Conclusions: A complete ophthalmological exam is mandatory for each patient diagnosed with Covid 19 disease, even if they have ocular manifestations or not. Abbreviations: SARS-Cov2 = severe acute respiratory syndrome; DM = Diabetes mellitus; HT = Hypertension; ON = Optic neuritis; VF = Visual field ; NS = Nervous system; CRP = C-reactive Protein; CL = cytokines; IL = interleukins; TNFɑ = tumor necrosis factor; CNS = central nervous system; ACE = angiotensin-converting enzyme; CRVO = central retinal vein occlusion; MOG = myelin oligodendrocyte glycoprotein; MOG-AD = myelin oligodendrocyte glycoprotein antibody disease; BBB = blood-brain barrier; ARDS = acute respiratory distress syndrome; IOP = intraocular pressure; CVP = central venous pressure; MSOF = multiple systems organ failure; AAION = arteritic anterior ischemic optic neuropathy; NAION = non-arteritic anterior ischemic optic neuropathy; AION = anterior ischemic optic neuropathy; OCT = optical coherence tomography; CT = computer tomography; AFG = angiofluorography; MRI = magnetic resonance imaging; ESR = erythrocyte sedimentation rate; RF = rheumatoid factor; ANA = antinuclear antibodies; ANCA = antineutrophil cytoplasmic antibodies; AQP4 = anti aquaporin 4; NMO = neuromyelitis optica; CSF = cerebrospinal fluid; OTI = oro-tracheal intubated; VA = visual acuity; ONTT = optic neuritis treatment trial; RNFL = retinal nerve fiber layer; ICU = intensive care unit; LE = left eye; RE = right eye; MS = multiple sclerosis; ICH = intracranial hypertension; BCVA = best correction visual acuity; LP = light perception; APD = afferent pupillary defect; BM = biomicroscopy; PDN = prednisone; MTX = methotrexate; MTPN = methylprednisolone; NSAID = non-steroidal anti-inflammatory drugs; CGL = cells ganglion layer; VEP = visual evoked potential; CF = counting fingers.

Improvement of hydroxychloroquine retinopathy monitoring by informatic systems

AbstractPurpose: To study the proportion of patients treated with hydroxychloroquine in Health Area VII of Murcia following the screening recommended by the international guidelines and to implement a system to improve the screening efficacy.Methods: We studied the patients in Health Area VII of Murcia, Spain, treated with hydroxychloroquine between January and November 2019 to find out whether patients were correctly screened for retinopathy. Demographic data, medical specialty prescribing hydroxychloroquine, disease, year of onset and examinations performed in our Ophthalmology Service were collected.Results: A total of 202 medical records were reviewed. Baseline ophthalmologic exams were performed in 63.9% of cases: fundus examination (96.9%) and optical coherence tomography (OCT) (33.3%). Follow‐up examination was performed in 61.8% of cases. Follow‐up was complete, with OCT, 10‐2 visual field and autofluorescence image in only 2.4% of patients. The time between revisions was 1.6 years on average. These results strongly deviated from the guidelines. We planned a strategy to improve the screening of these patients. We developed a computer database that allows the voluntary and automatic registration of patients treated with hydroxychloroquine, including contact and demographic data, start date of treatment, the daily dose and retinopathy risk factors. With this database, the patient's appointment in ophthalmology will be provided with the corresponding tests. The examination will be assessed by telemedicine and the time of the next check‐up will be determined according to the results.Conclusions: This plan aims to capture most patients starting the treatment with hydroxychloroquine and to carry out the appropriate tests at each visit with strict follow‐up, thus reducing the care load.

Comparison between dynamic contour tonometry and Goldmann applanation tonometry correcting equations

In order to investigate the reliability of correcting GAT formulas in comparison with dynamic contour tonometry (DCT), this study included 112 right eyes of 112 healthy subjects aged from 21 to 77 years, whose eyes underwent to a full ophthalmologic exam. IOP was measured in each eye with DCT and then with GAT. IOP values obtained with GAT were corrected with 10 equations and then compared with those provided by DCT. Participants mean age was 42.24 ± 14.08 years; mean IOP measured with DCT was 17.61 ± 2.87 and 15.50 ± 2.47 mmHg, measured with GAT. The mean discordance between DCT and GAT measurements was 2.11 ± 2.24 mmHg. All the correcting formulas, but Srodka one (p ˂ 0.001), tend to increase the difference between GAT and DCT. According to these results Śródka equation provides the best correction, reducing the difference between the two IOP measurement methods of − 0.03 ± 0.85 mmHg. Other equations do not provide a valid improvement of the agreement between the two methods or they provide a worsening of the agreement.

Characteristics of Orbital Injuries Associated with Maxillofacial Trauma.

The incidence of ocular injury associated with maxillofacial trauma remains poorly defined, with reported rates ranging from 0.8% to 92%. Our study aims to more accurately characterize ocular injuries associated with midface fractures. We performed a retrospective review of 1677 patients from 2015 to 2020 with midface fractures at a Level I trauma center. Isolated nasal bone and frontal process of the maxilla fractures were excluded. Demographic information, mechanism of injury, need for surgery, and ophthalmologic findings were documented. Statistical analysis was conducted using SPSS. 773 patients between the ages of 15 and 92 were identified. Trauma most commonly resulted from assault (63.8%). The association between the mechanism of injury and ocular injury was statistically significant (p= 0.003), with 78.6% of gunshot wounds and 44.3% of assault patients having an ocular injury. The Ophthalmology service evaluated 62.6% of cases preoperatively. Minor ocular injury occurred in 36% of patients, including 46.1% of isolated orbital floor, and 28.2% of zygomaticomaxillary complex fractures. Major ocular injury occurred in 10.5% of patients. Over 10% of patients with midface fractures were found to have major ocular injuries. Ophthalmologic exams should be performed for all patients with midface fractures to guide clinical decision making and prevent further intraoperative ocular insults. Level 4. This study represents a retrospective cohort study analyzing ocular injuries detected in patients presenting to a Level I trauma center with maxillofacial fractures between 2015 and 2020 Laryngoscope, 133:1624-1629, 2023.

PMON99 A Rare Case of an Intrasellar Epidermoid Cyst And Panhypopituitarism

Abstract Introduction Epidermoid cysts are rare intracranially occurring mainly in subarachnoid spaces such as parasellar cisterns, middle fossa and cerebellopontine angle. Only few cases of infundibular/sellar epidermoid cysts have been described. We present the case of a large sellar epidermoid cyst resulting in panhypopituitarism. Case 49 year old male with history of obesity and hypertension presented to the emergency department with acute onset progressively worsening headaches. He denied vision changes, nausea, vomiting or dizziness. Examination showed stable vital signs and ophthalmologic exam did not reveal any visual deficits. Further, he did not have any signs of pituitary hormone excess including hypercortisolemia or acromegaly. Brain MRI with and without IV contrast revealed a peripherally enhancing multilobulated cystic pituitary mass measuring 19.5 mm×17 mm×20.5 mm deviating the infundibulum to the left causing compression of the caudal aspect of the optic chiasm. Initial endocrinology work-up revealed noon time cortisol of 10.7 mcg/dL (range 7-23), 7 pm ACTH of 34.6 pg/mL (range 7.2-63.3), TSH of 1.71 mIU/mL (range 0.35- 4.94), free T4 of 0.93 ng/dL (range 0.5-1.39), FSH of 4.45 mIU/mL (range 1.27-19.26), LH 5.51 mIU/mL (range 1.24-8.62), IGF-1 level of 199 ng/mL (range 68-225), prolactin &amp;lt;0.60 ng/mL (range 2.64-13.13), and testosterone level of 186 ng/dL (range 175-800). A presumed diagnosis of non- functioning pituitary macroadenoma with compression of chiasm was made and he underwent transsphenoidal hypophysectomy after evaluation by a multi-disciplinary team including neurosurgery, ENT and endocrinology. Post-operative course was uneventful, he was started on empiric steroids which were tapered slowly. Final pathology of surgical specimen demonstrated epidermoid cyst, focal necrosis, lymphocytes and macrophages infiltration consistent with reactive granulation tissue. On subsequent laboratory evaluation, he was found to have hypogonadotropic hypogonadism with an undetectable testosterone. FT4 was also low at 0.61 ng/dL (range 0.5-1.39). He is closely following with endocrinology for management of hypogonadotropic hypogonadism and hypothyroidism. Conclusion Intracranial epidermoid cysts are uncommon benign congenital lesions arising from trapped surface of ectodermal elements. Very rarely, they are found in the sellar region. Sellar epidermoid cysts tend to present late and often after significant growth and mass effects. Complete resection is challenging and recurrence is a feared complication. Our patient presented with acute symptoms from expansion of a large epidermoid sellar cyst mimicking a pituitary macroadenoma. It also resulted in hypopituitarism. Although, a rare entity, early recognition and prompt management of such lesions is key. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Pineal Gland Mass presenting as Parinaud Syndrome in a 21 Year Old Patient

We report a case of a 21-year-old Filipino, with a history of progressive diplopia, who presented with headache, dizziness and vomiting. Ophthalmologic exam revealed light near dissociation, physiologic anisocoria, bilateral upgaze palsy, convergence retraction nystagmus and pseudo-abducens palsy of the left eye. Imaging was done, which revealed pineal neoplasm with obstructive hydrocephalus. This text aims to present a case of pineal gland neoplasm which presented as diplopia alongside signs and symptoms of Dorsal Midbrain Syndrome (Parinaud Syndrome).

Morphological changes in retinochoroidal microvasculature after caffeinated versus decaffeinated coffee consumption

BackgroundTo investigate changes in retinochoroidal microvascular morphology after caffeinated versus decaffeinated coffee consumption in age- and gender-matched healthy individuals using optical coherence tomography (OCT) and OCT angiography (OCTA). MethodsIn this prospective, randomized clinical study, a staff member in charge of record keeping randomly assigned 48 healthy volunteers to two groups: caffeinated coffee consumers (24 eyes) and decaffeinated coffee consumers (24 eyes). Participants' ages and genders were recorded before consumption, and a comprehensive ophthalmologic exam was performed, followed by OCT and OCTA analyses before, 30 min, one, six, and 24 h after blindly consuming either of the coffees. ResultsCaffeinated and decaffeinated coffee consumers had mean ages of 23.45 ± 0.92 and 22.73 ± 1.13, respectively (p = 0.407). The following parameters changed significantly in caffeinated coffee consumers 30 min and 1 h post-consumption (pre-consumption versus 30 min versus one hour post-consumption; p < 0.05): a) parafoveal superficial capillary plexus vessel density (%): 54.45 versus 51.8 versus 51.92, b) parafoveal deep capillary plexus vessel density (%): 55.16 versus 52.45 versus 52.83, c) outer retinal flow area (%): 8.87 ± 1.91 versus 8.03 ± 1.88 versus 8.11 ± 1.93, d) choriocapillaris flow area (mm2): 20.95 ± 0.98 versus 19.82 ± 1.20 a versus 19.62 ± 0.95, and e) sub-foveal choroidal thickness (µm): 295.06 ± 5.45 versus 277.08 ± 5.33 versus 260.71 ± 58.61. No significant differences in any OCT and OCTA parameters were found between consecutive measurements in decaffeinated coffee consumers (p > 0.05). ConclusionsCaffeinated coffee appears to transiently reduce parafoveal vessel density, capillary flow area, and sub-foveal choroidal thickness. Lack of these microvascular morphological changes in decaffeinated coffee suggests a potential caffeine-induced vasoconstrictive effect.

IN THE EYE OF THE STORM: HYPERVIRULENT KLEBSIELLA PNEUMONIAE

IN THE EYE OF THE STORM: HYPERVIRULENT KLEBSIELLA PNEUMONIAE

AML-372 Acute Promyelocytic Leukemia: A Case of Pseudotumor Cerebri Syndrome During Treatment Consolidation

Acute promyelocytic leukemia (APL) is a subtype of acute myeloid leukemia with distinctive morphological, clinical, and genetic features. Pseudotumor cerebri, also known as idiopathic intracranial hypertension, describes a neurologic syndrome characterized by headache, visual changes, papilledema without cerebral mass lesion, venous sinus thromboses, or obstructive hydrocephalus. Risk factors for the development of pseudotumor cerebri are obesity, women in the childbearing years, and hypervitaminosis A. The modified Dandy criteria are used for pseudotumor cerebri syndrome (PTCS) diagnosis. This complication has a high incidence in the pediatric population, particularly within 2-3 weeks of induction treatment for APL. Pseudotumor cerebri is a rare complication during consolidation therapy for APL. The reported incidence of "probable" PTCS is 1.7% in APL patients treated with ATRA. Case Report: We report here the case of a 20-year-old woman, obese with a BMI of 34.5 kg/m2, diagnosed with low-/intermediate-risk APL and treated with ATRA and ATO as induction treatment. After induction, she achieved MRD-negative complete morphological remission. During the first 2 weeks of the first cycle of consolidation-ATRA and ATO-the patient developed progressive diplopia and headaches without response to standard analgesic therapy. Additionally, she complained of nausea and vomiting. The ophthalmological exam with fundus examination revealed bilateral papilledema. The neurological exam was normal. There were no abnormal findings in the head CT scan or MRI of the brain. Thus, a diagnosis of "probable" pseudotumor cerebri was established. ATRA was discontinued and she received acetazolamide and dexamethasone with resolution of neurological symptoms within the next 7 days. Upon the next consolidation ATRA and ATO, the headaches resumed. The patient received all the ATRA consolidation cures as scheduled but with a dose reduction to 80% of the initial dose and in association with acetazolamide. She completed consolidation treatment and continues to be in molecular complete remission. Conclusions: This case report highlights the possibility of pseudotumor cerebri development in young adult patients with APL during consolidation therapy. The neurological symptoms were reversible, and the patient received all the ATRA consolidation. It is important to exclude other causes of intracranial hypertension including thromboses, infections, and disease involvement.

Leveraging weak supervision to perform named entity recognition in electronic health records progress notes to identify the ophthalmology exam

ObjectiveTo develop deep learning models to recognize ophthalmic examination components from clinical notes in electronic health records (EHR) using a weak supervision approach. MethodsA corpus of 39,099 ophthalmology notes weakly labeled for 24 examination entities was assembled from the EHR of one academic center. Four pre-trained transformer-based language models (DistilBert, BioBert, BlueBert, and ClinicalBert) were fine-tuned to this named entity recognition task and compared to a baseline regular expression model. Models were evaluated on the weakly labeled test dataset, a human-labeled sample of that set, and a human-labeled independent dataset. ResultsOn the weakly labeled test set, all transformer-based models had recall > 0.93, with precision varying from 0.815 to 0.843. The baseline model had lower recall (0.769) and precision (0.682). On the human-annotated sample, the baseline model had high recall (0.962, 95 % CI 0.955–0.067) with variable precision across entities (0.081–0.999). Bert models had recall ranging from 0.771 to 0.831, and precision >=0.973. On the independent dataset, precision was 0.926 and recall 0.458 for BlueBert. The baseline model had better recall (0.708, 95 % CI 0.674–0.738) but worse precision (0.399, 95 % CI -0.352–0.451). ConclusionWe developed the first deep learning system to recognize eye examination components from clinical notes, leveraging a novel opportunity for weak supervision. Transformer-based models had high precision on human-annotated labels, whereas the baseline model had poor precision but higher recall. This system may be used to improve cohort and feature identification using free-text notes.Our weakly supervised approach may help amass large datasets of domain-specific entities from EHRs in many fields.