scholarly journals Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Tariq A. Alzahem ◽  
Antonio Augusto Cruz ◽  
Azza M. Y. Maktabi ◽  
Fernando Chahud ◽  
Hind Alkatan
Keyword(s):  
Systemic Disease ◽  
Combined Treatment ◽  
Case Series ◽  
Bone Destruction ◽  
Severe Form ◽  
Cystoid Macular Oedema ◽  
Variable Degree ◽  
Retrospective Case ◽  
Rosai Dorfman Disease ◽  
Males And Females

Abstract Background To provide basic demographic information and clinicopathologic features of ophthalmic Rosai–Dorfman disease (RDD) with a literature review. Methods A multi-centre retrospective case series reviewing all patients with histopathologically confirmed ophthalmic RDD at three tertiary eye care centres between January 1993 and December 2018. Results Eleven eyes of eight patients with histopathologically confirmed ophthalmic RDD were included, with equal numbers of males and females. The median age was 40.25 years (range: 26.6–72.4). Two patients had familial RDD. The orbit was the most commonly involved site (90.9% eyes). One patient (one eye) presented with a scleral nodule, anterior uveitis and cystoid macular oedema. Visual acuity ranged from 20/25 to light perception. Six patients had an extra-nodal ophthalmic disease, and the remaining two had an associated submandibular lymphadenopathy (nodal RDD). Conclusions Ophthalmic RDD can be the only manifestation of this systemic disease, with the orbit being the most commonly involved site, exhibiting bone destruction, intracranial and/or sinus involvement and variable degree of visual loss. Ophthalmic familial RDD represent a severe form with a malignant course. Steroid monotherapy may be inadequate to control orbital RDD; thus, combined treatment is usually necessary. A comprehensive approach to assessment and management is recommended.

scholarly journals COVID-19 Disease Leading to Chronic Spontaneous Urticaria Exacerbation: A Romanian Retrospective Study

Healthcare ◽  
2021 ◽  
Vol 9 (9) ◽  
pp. 1144
Author(s):  
Ioana Adriana Muntean ◽  
Irena Pintea ◽  
Ioana Corina Bocsan ◽  
Carmen Teodora Dobrican ◽  
Diana Deleanu
Keyword(s):  
Inflammatory Markers ◽  
Clinical Presentation ◽  
Significant Proportion ◽  
Case Series ◽  
Treated Group ◽  
Severe Form ◽  
Chronic Spontaneous Urticaria ◽  
Retrospective Case ◽  
Markers Of Inflammation ◽  
Urticaria Activity Score

(1) Background: The COVID-19 pandemic has resulted in the exacerbation of various chronic diseases. Due to the potential impact of SARS-CoV-2 on mast cells, we aimed to analyze the relevance of COVID-19 disease on chronic spontaneous urticaria (CSU) clinical presentation and biological profile. (2) Methods: This study is a retrospective case series of patients with CSU diagnosed and treated in the Allergy Department of the Professor Doctor Octavian Fodor RIGH, (Cluj-Napoca, Romania). Patients were assessed for disease activity and level of control with the weekly urticaria activity score and the visual analogue scale. Results were correlated with COVID-19 severity and with nonspecific markers of inflammation during and after the SARS-CoV-2 infection. (3) Results: SARS-CoV-2 impacted a significant proportion (33%) of the CSU patients, of which 71% developed a moderate-severe form of COVID-19. Most of the patients (68%) had moderate-severe forms of CSU and 65% took AH1 treatment (one dose, two-fold dose or four-fold dose). The rest of them (35%) received the second-line treatment (40.3% Omalizumab, 53% Prednisolone and 4.8% Cyclosporine). In Omalizumab treated group of UCS patients we observed that COVID-19 disease was not severe. We established a positive correlation between the severity of the infection and that of the CSU clinical presentation, with most bothersome symptoms of urticaria being experienced by moderate to severe COVID-19 CSU patients (47%). Inflammatory markers were positively correlated (p = 0.01) with a more severe clinical profile of CSU, in accordance with our hypothesis that the level of inflammation triggered by COVID-19 disease has a role in CSU exacerbation. The non-specific inflammatory markers, such as CRP, were positively associated with the UAS7 score (R2 = 0.363; p = 0.001). An increased rate of exacerbation of CSU was observed in moderate-severe COVID-19 infection. 4) Conclusions: COVID-19 disease can result in the exacerbation of chronic spontaneous urticaria, more likely in moderate to severe forms of infection.

scholarly journals Outcomes of intravitreal methotrexate to salvage eyes with relapsed primary intraocular lymphoma

2020 ◽  
pp. bjophthalmol-2020-317199
Author(s):  
Mona Mohammad ◽  
Richard M Andrews ◽  
P Nicholas Plowman ◽  
Gordon Hay ◽  
Amit K Arora ◽  
...  
Keyword(s):  
Systemic Chemotherapy ◽  
External Beam Radiotherapy ◽  
Case Series ◽  
Central Nervous System Lymphoma ◽  
Intraocular Lymphoma ◽  
Cystoid Macular Oedema ◽  
Primary Intraocular Lymphoma ◽  
Retrospective Case ◽  
Tumour Control

PurposeTo report the outcomes of intravitreal methotrexate (MTX) injections to rescue eyes with relapsed primary intraocular lymphoma (PIOL).MethodsRetrospective case series of patients with ocular relapse of PIOL who had initially received systemic chemotherapy (all five cases) and external beam radiotherapy (EBRT) to brain and orbits (two cases). Injections of MTX (400 µg/0.1 mL) were given one time per week for 1 month, every other week for 4 months, followed by a maintenance phase of one injection one time per month for 8 months (total of 20 injections in a year).ResultsFrom April 2008 to February 2016, there were nine eyes of five patients (three men; average age at first presentation 62 years) treated with our rescue protocol of intravitreal MTX injections. Ocular relapse occurred at a mean interval of 15 months (range 5–34 months) after the completion of initial systemic treatment. At mean follow-up of 31 months (range 5–104 months), tumour control was achieved in eight out of nine eyes (89%); one eye failed, with persistent retinal infiltrates despite increasing the frequency of injections, resulting in severe keratopathy. The only other complication occurred in one eye, developing cystoid macular oedema from MTX injections that resolved with topical anti-inflammatory medications and reduced frequency of MTX. There were no cases of reduced vision or ocular relapse, but two patients died (one of central nervous system lymphoma).ConclusionsIntravitreal MTX was a safe and effective treatment modality for relapsed PIOL after systemic chemotherapy and radiotherapy, achieving local tumour control in 89%, and hence represents an optimal choice. However, given the rare nature of PIOL, larger collaborative studies with longer follow-up are needed to corroborate this.

scholarly journals Multi-modal Imaging in Etanercept-induced Uveitis: A Case Series

2020 ◽  
Vol 14 (1) ◽  
pp. 70-74
Author(s):  
Jaidip Gill ◽  
Robert Barry ◽  
Shreekanth Sreekantam ◽  
Bashar Mohammed
Keyword(s):  
Macular Oedema ◽  
Clinical Course ◽  
Retinal Vasculitis ◽  
Case Series ◽  
Corticosteroid Treatment ◽  
Cystoid Macular Oedema ◽  
Fundus Fluorescein Angiography ◽  
Retrospective Case ◽  
Etanercept Treatment ◽  
New Onset

Introduction: To report with multi-modal imaging the clinical course of 3 patients with new-onset uveitis following treatment with etanercept. Methods: Retrospective case-note reviews were conducted of 3 patients previously established on etanercept who developed new-onset acute uveitis. Results and Discussion: Three patients were assessed with a mean age of 44.3 (43-47). Etanercept was indicated for the treatment of ankylosing spondylitis in two patients and psoriatic arthritis in 1 patient. Duration of etanercept treatment ranged from 7 to 10 years; however, in two cases, treatment recently changed to an etanercept biosimilar agent. Two patients were diagnosed with bilateral panuveitis and one patient had chronic relapsing anterior uveitis. Infection screen was negative in all three patients. 2 patients developed cystoid macular oedema as viewed on Spectral Domain OCT. Fundus fluorescein angiography was performed in one patient who demonstrated bilateral retinal vasculitis. All three patients were started on systemic and topical treatment. One patient received sub-tenon triamcinolone injection. Etanercept was discontinued for all patients. 1 of 3 patients lost vision at 7 months. 2 patients demonstrated long-term remission and one patient required intravitreal steroid implantation to stabilize an ongoing intraocular inflammation. Two patients who had complete remission were commenced on Adalimumab while the third patient was commenced on Secukinumab. Conclusion: The clinical course of uveitis developing paradoxically following etanercept treatment is variable. Multi-modal imaging is useful for the clinician that helps in diagnosing and monitoring associated macular oedema and retinal ischaemia. Cessation of etanercept and systemic corticosteroid treatment are often required to prevent ocular morbidity.

Arachnoid cysts confined to the internal auditory canal or facial nerve canal

2011 ◽  
Vol 125 (10) ◽  
pp. 1053-1058 ◽  
Author(s):  
Q Zhang ◽  
K Kaga ◽  
M Sano ◽  
H Takegoshi
Keyword(s):  
Facial Nerve ◽  
Internal Auditory Canal ◽  
Case Series ◽  
Morphological Characteristics ◽  
Arachnoid Cysts ◽  
Variable Degree ◽  
Retrospective Case ◽  
Facial Nerve Canal ◽  
Nerve Dysfunction ◽  
Thin Walled

AbstractObjective:To illustrate the morphological characteristics and pathological significance of arachnoid cysts confined to the internal auditory canal or facial nerve canal.Design:Retrospective case series.Subjects:Three patients' cases were reviewed, and temporal bone sections examined histologically.Results:In this series, three intracanalicular arachnoid cysts were found in the internal auditory canal and one in the facial nerve canal. All lesions consisted of distinct, space-occupying cysts with a sharp boundary with surrounding tissues. They were thin-walled and compressed the surrounding nerve trunks to a variable degree. However, there was no indication that patients suffered meatal nerve dysfunction.Conclusion:Arachnoid cysts can develop within the internal auditory canal or facial nerve canal. In our series, they were asymptomatic, which is quite different from most intracanalicular arachnoid cysts encountered in clinical practice.

scholarly journals Are eyes the windows to COVID-19? Systematic review and meta-analysis

2020 ◽  
Vol 5 (1) ◽  
pp. e000563
Author(s):  
Rina La Distia Nora ◽  
Ikhwanuliman Putera ◽  
Dhiya Farah Khalisha ◽  
Indah Septiana ◽  
Asri Salima Ridwan ◽  
...  
Keyword(s):  
Meta Analysis ◽  
Systemic Disease ◽  
Case Series ◽  
Severe Form ◽  
Ocular Manifestation ◽  
Systemic Manifestation ◽  
Ocular Manifestations ◽  
Source Of Infection ◽  
Pooled Prevalence ◽  
Meta Analyses

ObjectiveTo review and critically appraise the ocular manifestation and the presence of SARS-CoV-2 through PCR positivity from ocular samples in COVID-19-related patients. Moreover, to evaluate the time and severity association of ocular manifestation to systemic disease of COVID-19.Methods and analysisA systematic literature search from PubMed, ScienceDirect and Google Scholar databases was performed using standardised Preferred Reporting Items for Systematic Reviews and Meta-Analyses guideline. Selected keywords were related to COVID-19, ocular manifestation and PCR testing of SARS-CoV-2. Studies were assessed for their validity, and the data were extracted by two independent reviewers. Observational, case series and case report studies were included if they met the selection criteria. Meta-analysis was performed to estimate the pooled prevalence of ocular manifestations and PCR positivity from tears.ResultsThirty-one articles were qualitatively reviewed, and 14 studies were included in the meta-analysis. The pooled prevalence of ocular manifestation among COVID-19-related patients was 0.05 (95% CI 0.02% to 0.08). The overall PCR from tears samples positivity rate from COVID-19-related patients presenting with ocular manifestation was 0.38 (95% CI 0.14% to 0.65). Ocular manifestation could precede systemic manifestation in about 0.28 (95% CI 0.05% to 0.58) of COVID-19-related patients with ocular manifestations. Besides, ocular manifestation was not associated with a severe form of COVID-19.ConclusionAlthough the overall number of ocular manifestation and SARS-CoV-2 PCR positivity rate from ocular samples was very low, around a quarter of COVID-19-related patients with ocular manifestation presented their ocular manifestation earlier than the systemic manifestation regardless of the severity. Interestingly, SARS-CoV-2 PCR was positive from one-third of ocular samples, which could potentially be the source of infection to the respiratory tract and the environment, although the infectivity is yet to be determined.

Children With Trisomy 21 and Lennox-Gastaut Syndrome With Predominant Myoclonic Seizures

2021 ◽  
pp. 088307382110260
Author(s):  
Matthew Macdonald ◽  
Jacqueline Crawford ◽  
Anita N. Datta
Keyword(s):  
Trisomy 21 ◽  
Case Series ◽  
Severe Form ◽  
Seizure Type ◽  
Pediatric Epilepsy ◽  
Retrospective Case ◽  
Spike Wave Discharges ◽  
Long Term Prognosis ◽  
Atypical Absence

Introduction: Lennox-Gastaut syndrome is a severe form of pediatric epilepsy that is classically defined by a triad of drug-resistant seizures, including atonic, tonic, and atypical absence seizures; slow spike-and-wave discharges and paroxysmal fast activity on electroencephalography (EEG); and cognitive and behavioral dysfunction. In the vast majority, Lennox-Gastaut syndrome develops in patients with an identified etiology, including genetic or structural brain abnormalities. Long-term prognosis is generally poor with progressive intellectual deterioration and persistent seizures. At present, there are few reported cases of Lennox-Gastaut syndrome and trisomy 21 in the literature. To further delineate the spectrum of epilepsy in trisomy 21, we reviewed children with trisomy 21 and Lennox-Gastaut syndrome at one center over 28 years. Methods: This is a retrospective case series. At our institution, all EEG results are entered into a database, which was queried for patients with trisomy 21 from 1992 to 2019. Pertinent electroclinical data was obtained from medical records. Results: Of 63 patients with trisomy 21 and epilepsy, 6 (10%) had Lennox-Gastaut syndrome and were included in the study. Four of the 6 patients were male and 5 of 6 had neuroimaging, which was normal. Follow-up ranged from 3 to 20 years. Notably, 5 of 6 had predominant myoclonic seizures throughout the course of their epilepsy, associated with generalized spike-wave discharges, <100 milliseconds. Conclusion: We observed myoclonic seizures to be a predominant seizure type in patients with trisomy 21, suggestive that trisomy 21 patients may have a unique pattern of Lennox-Gastaut syndrome.

scholarly journals P.104 Children with Trisomy 21 and Lennox-Gastaut Syndrome with predominant myoclonic seizures

2021 ◽  
Vol 48 (s3) ◽  
pp. S49-S49
Author(s):  
MJ MacDonald ◽  
J Crawford ◽  
A Datta
Keyword(s):  
Trisomy 21 ◽  
Case Series ◽  
Severe Form ◽  
Seizure Type ◽  
Pediatric Epilepsy ◽  
Retrospective Case ◽  
Spike Wave Discharges ◽  
Long Term Prognosis ◽  
Neuro Imaging

Background: Lennox-Gastaut syndrome (LGS) is a severe form of pediatric epilepsy that is classically defined by a triad of drug-resistant seizures, characteristic EEG patterns, and intellectual disability. Long-term prognosis is generally poor with progressive intellectual deterioration and persistent seizures. At present, there are few reported cases of LGS and Trisomy 21 (T21) in the literature. To further delineate the spectrum of epilepsy in T21, we reviewed children with T21 and LGS at one center over 28 years. Methods: This is a retrospective case series. At our institution, all EEG results are entered into a database, which was queried for patients with T21 from 1992-2019. Pertinent electro-clinical data was obtained from medical records. Results: 63 patients with T21 and epilepsy, 6 (10%) had LGS and were included in the study. Four of the six patients were male and 5/6, had neuro-imaging, which was normal. Follow-up ranged from 3-20 years. Notably, 5/6 had predominant myoclonic seizures throughout the course of their epilepsy, associated with generalized spike-wave discharges. Conclusions: Myoclonic seizures appear to be a predominant seizure type in patients with T21, suggestive that T21 patients may have a unique pattern of LGS.

scholarly journals Extranodal Rosai-Dorfman of the Pancreas Presents with Bilateral Granulomatous Anterior Uveitis

2018 ◽  
Vol 5 (4) ◽  
pp. 229-233 ◽  
Author(s):  
A’sha Brown ◽  
Sara V. Branson ◽  
Ghazala A. Datoo O’Keefe
Keyword(s):  
Anterior Uveitis ◽  
Systemic Disease ◽  
Cervical Lymphadenopathy ◽  
Pancreatic Mass ◽  
Ocular Involvement ◽  
African American Female ◽  
Retrospective Case ◽  
Granulomatous Uveitis ◽  
Rosai Dorfman Disease ◽  
Systemic Manifestations

Background/Aims: To report a case of a 65-year-old female who presented with bilateral anterior granulomatous uveitis, which led to the diagnosis of Rosai-Dorfman Disease (RDD) and the discovery of an associated pancreatic mass. Methods: This is a retrospective case report of a single patient. Results: A 65-year-old African American Female received a full workup for bilateral anterior granulomatous uveitis and was found to have a pancreatic mass on imaging. Biopsy of this mass was diagnostic for RDD, a rare proliferative histiocytic disorder, which classically presents with painless cervical lymphadenopathy, leukocytosis, and hypergammaglobulinemia. Conclusion: Although rare, ocular involvement, including eyelid and orbital masses, and anterior uveitis, can herald the development of other systemic manifestations or RDD and can aid in the diagnosis of this systemic disease.

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