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1.
J Neuroophthalmol ; 2024 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-38946028

RESUMO

BACKGROUND: Although cupping of the optic nerve is classically a sign of glaucomatous optic neuropathy, it has been shown that cupping can sometimes occur after an episode of optic neuritis (ON). The purpose of this study was to compare cupping in patients after ON from multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and to investigate the relationship between cupping and retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thinning. METHODS: This was a retrospective cohort involving patients (≥18 years) with ON from 3 institutions. Patients were eligible if they had optical coherence tomography (Cirrus, OCT) performed ≥6 months after a single unilateral ON. The amount of thinning and cupping was estimated from the difference in the OCT parameters between affected and unaffected eyes. Univariable and multivariable regressions were used to investigate the relationship between cupping and ON etiology. Pearson correlation was used to investigate the relationship between cupping and RNFL and GCC. RESULTS: Eighty-six subjects (MS: 35, NMOSD: 26, and MOGAD: 25) were included. There was no significant difference in gender and race between the groups, and most patients (86.1%) were female. Patients with NMOSD were significantly older than patients with MS or MOGAD (P = 0.002). In the univariate model, cupping was significantly higher in the NMOSD group (P = 0.017); however, after adjusting for age, GCC, and RNFL of the affected eye, the difference was no longer statistically significant (P = 0.949). The correlation between cupping asymmetry and RNFL and GCC of the affected eye was inversely strong in patients with MS (R = -0.60 and R = -0.64, respectively), inversely moderate in patients with MOGAD (R = -0.34 and R = -0.40, respectively), and weak in patients with NMOSD (R = -0.03 and R = -0.17, respectively). CONCLUSIONS: Our results demonstrated that cupping after ON is correlated with RNFL and GCC thinning; although cupping was overall greater in the NMOSD group, once adjusted for age, RNFL, and GCC, it did not differ among patients with MS, NMOSD, and MOGAD.

2.
BMC Ophthalmol ; 24(1): 253, 2024 Jun 12.
Artigo em Inglês | MEDLINE | ID: mdl-38867186

RESUMO

BACKGROUND: The long-term visual outcomes in spasmus nutans patients is largely unknown. The purpose of this study was to characterize visual outcomes and identify comorbid ophthalmic conditions in patients with spasmus nutans. METHODS: We retrospectively reviewed the charts of consecutive patients diagnosed with spasmus nutans between 2000 and 2020. Demographic information, ophthalmic characteristics, and neuroimaging results were assessed over time. RESULTS: Of the 32 patients included in the study, 13 (41%) were female. Underlying medical conditions included a diagnosis of Trisomy 21 in 6 (19%) and prematurity in 8 (25%). Twenty-one patients (66%) self-reported as a race other than Caucasian. 18 patients (56%) had non-private health insurance and 1 (3%) was uninsured. Mean age at diagnosis and resolution were 16 months (range 45 months) and 48 months (range 114 months), respectively. All 32 patients had nystagmus, 31 (97%) had head nodding and 16 (50%) had ocular torticollis. Mean follow-up was 66 months (range 185 months). On initial presentation, 6/32 (19%) had an amblyogenic refractive error and mean best-corrected visual acuity (BCVA) in the better-seeing eye was 0.78 Logarithm of the Minimum Angle of Resolution (LogMAR) (range 1.24). In a sub-analysis that included patients with > 1 exam (n = 23), 17/20 (85%) had an amblyogenic refractive error and mean BCVA in the better-seeing eye was 0.48 LogMAR (range 1.70). At the final exam, 12 patients had measurable stereopsis, eight had strabismus, and three had undergone strabismus surgery. Eight patients required treatment for amblyopia. CONCLUSIONS: We found a high prevalence of amblyogenic refractive error, strabismus and amblyopia among patients with spasmus nutans. Children with spasmus nutans benefit from ongoing ophthalmic follow-up until they are past the amblyopic age range, even after resolution of nystagmus.


Assuntos
Acuidade Visual , Humanos , Feminino , Masculino , Estudos Retrospectivos , Acuidade Visual/fisiologia , Pré-Escolar , Criança , Lactente , Seguimentos , Nistagmo Patológico/fisiopatologia , Ambliopia/fisiopatologia , Ambliopia/epidemiologia , Estrabismo/fisiopatologia
3.
Ophthalmol Sci ; 4(4): 100496, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38682028

RESUMO

Purpose: To develop and test an artificial intelligence (AI) model to aid in differentiating pediatric pseudopapilledema from true papilledema on fundus photographs. Design: Multicenter retrospective study. Subjects: A total of 851 fundus photographs from 235 children (age < 18 years) with pseudopapilledema and true papilledema. Methods: Four pediatric neuro-ophthalmologists at 4 different institutions contributed fundus photographs of children with confirmed diagnoses of papilledema or pseudopapilledema. An AI model to classify fundus photographs as papilledema or pseudopapilledema was developed using a DenseNet backbone and a tribranch convolutional neural network. We performed 10-fold cross-validation and separately analyzed an external test set. The AI model's performance was compared with 2 masked human expert pediatric neuro-ophthalmologists, who performed the same classification task. Main Outcome Measures: Accuracy, sensitivity, and specificity of the AI model compared with human experts. Results: The area under receiver operating curve of the AI model was 0.77 for the cross-validation set and 0.81 for the external test set. The accuracy of the AI model was 70.0% for the cross-validation set and 73.9% for the external test set. The sensitivity of the AI model was 73.4% for the cross-validation set and 90.4% for the external test set. The AI model's accuracy was significantly higher than human experts on the cross validation set (P < 0.002), and the model's sensitivity was significantly higher on the external test set (P = 0.0002). The specificity of the AI model and human experts was similar (56.4%-67.3%). Moreover, the AI model was significantly more sensitive at detecting mild papilledema than human experts, whereas AI and humans performed similarly on photographs of moderate-to-severe papilledema. On review of the external test set, only 1 child (with nearly resolved pseudotumor cerebri) had both eyes with papilledema incorrectly classified as pseudopapilledema. Conclusions: When classifying fundus photographs of pediatric papilledema and pseudopapilledema, our AI model achieved > 90% sensitivity at detecting papilledema, superior to human experts. Due to the high sensitivity and low false negative rate, AI may be useful to triage children with suspected papilledema requiring work-up to evaluate for serious underlying neurologic conditions. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

4.
J Neuroophthalmol ; 2024 Mar 25.
Artigo em Inglês | MEDLINE | ID: mdl-38527126

RESUMO

BACKGROUND: To evaluate the quality & reliability, technical quality, and readability of patient-targeted online information on idiopathic intracranial hypertension. METHODS: In this cross-sectional study, we searched Google and Bing search engines for the terms "idiopathic intracranial hypertension" and "pseudotumor cerebri." We evaluated the first 50 search outcomes for each search term. Peer-reviewed articles, targeted advertisements, book chapters, videos, personal blogs, websites intended for healthcare professionals, and non-English websites were excluded. Websites were classified as institutional or private. Two independent reviewers assessed each website for 1) quality and reliability using DISCERN, Health on the Net Code of Conduct (HONcode), and JAMA criteria and 2) technical quality based on 11 criteria. Readability was evaluated using 6 measures (Flesch-Kincaid Reading Ease score and grade level, Gunning Fog, Simple Measure of Gobbledygook (Smog) index, Coleman-Liau index, and automated readability index). RESULTS: Sixty-three websites (37 institutional) were included. The mean scores for the DISCERN, HONcode, and JAMA criteria were 3.6 ± 1 (1-4.8; 1: worse, 5: best), 10.3 ± 2.8 (2-16; 0: worse, 16: best), and 2 ± 1.3 (0-4; 0: worse, 4: best), respectively. The mean technical quality score was 0.8 ± 0.1 (0.5-1). The mean Flesch-Kincaid grade level score was 8.9 ± 1.8 (3.3-13.3). For Flesch-Kincaid grade level, 47 (74.6%) websites scored a grade level of 10 or less. For Gunning Fog Score, 35 websites (55.6%) scored from 7.3 to 10.8. For the Smog Index, 46 (73%) websites scored 5.7-8. The mean Coleman Liau Index was 16 ± 2.1 (9.6-22.2). For the automated readability index, 30 (50.7%) websites scored less than the eighth grade. No statistically significant difference was present between institutional and private websites except for JAMA, which scored higher in private websites (2.4 vs 1.7, P = 0.03). CONCLUSIONS: Our findings suggest that online information on idiopathic intracranial hypertension/pseudotumor cerebri for patients generally demonstrates moderate to good quality and reliability. Although most websites maintain credible technical quality, the readability exceeds recommended levels for the average American reader. This may impede understanding, emphasizing the need for future improvements.

5.
Ophthalmic Surg Lasers Imaging Retina ; 55(4): 235-239, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38319054

RESUMO

A 13-year-old Black male patient with a history of Kikuchi-Fujimoto disease (KFD) and sickle cell trait presented with acute painless vision loss and no light perception vision (NLP) in his left eye. The examination was indicative of occlusive retinal vasculitis with near total central retinal artery occlusion (CRAO). He was started on oral steroids with dramatic reperfusion and improvement of the retinal hemorrhages. However, his vision remained at NLP. Oral steroids were tapered, and rituximab infusion was initiated. While ocular involvement is uncommon in KFD, vision-limiting complications, such as occlusive retinal vasculitis, ophthalmic artery occlusion, and CRAO can occur. Early systemic immunosuppression is key in achieving rapid remission. [Ophthalmic Surg Lasers Imaging Retina 2024;55:235-239.].


Assuntos
Angiofluoresceinografia , Linfadenite Histiocítica Necrosante , Vasculite Retiniana , Traço Falciforme , Humanos , Masculino , Linfadenite Histiocítica Necrosante/diagnóstico , Linfadenite Histiocítica Necrosante/complicações , Linfadenite Histiocítica Necrosante/tratamento farmacológico , Traço Falciforme/complicações , Traço Falciforme/diagnóstico , Vasculite Retiniana/diagnóstico , Vasculite Retiniana/etiologia , Adolescente , Angiofluoresceinografia/métodos , Acuidade Visual , Oclusão da Artéria Retiniana/diagnóstico , Oclusão da Artéria Retiniana/etiologia , Fundo de Olho , Glucocorticoides/uso terapêutico , Glucocorticoides/administração & dosagem
6.
Am J Ophthalmol ; 263: 126-132, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38395330

RESUMO

PURPOSE: Cerebral venous sinus thrombosis (CVST) is a rare but life-threatening event with significant neurologic and visual morbidity. In this study, we report on the natural history and visual outcomes of papilledema in children with CVST. DESIGN: Retrospective case series. METHODS: Patients with CVST evaluated by the Department of Ophthalmology between 2000 and 2023 were included. Records were reviewed for presence and course of papilledema, treatment, and final visual outcomes following papilledema resolution. RESULTS: The study included 35 patients with a mean age of 9 ± 5 years and 40% were female. The most common risk factors for CVST were infection (69%), dehydration (26%), and hypercoagulability (23%). 31 patients (89%) had papilledema. Of these patients, 9 (29%) had progression of papilledema despite treatment, 17 patients (55%) did not have progression, and 5 patients (16%) lacked follow-up records. Initial Frisén grade among all cases was 2 ± 1, and cases with progression reached a grade of 4 ± 1 between 10 and 32 days following initial identification. Most patients (97%) were treated with anticoagulation and 100% required acetazolamide and/or lumbar puncture. Among 26 patients with follow-up, papilledema resolved in 107 ± 128 days. Fifty-four percent of patients had permanent ophthalmic sequelae. An initial Frisén grade ≥3 (odds ratio 7.54, 95% confidence interval 6.53-8.70, P< .001) was significantly associated with eventual optic atrophy. CONCLUSIONS: Children with CVST are at high risk for ophthalmologic sequelae. Papilledema can progress despite appropriate therapy. Our results highlight the importance of ophthalmologic follow-up during treatment course to prevent irreversible vision loss.


Assuntos
Anticoagulantes , Papiledema , Trombose dos Seios Intracranianos , Acuidade Visual , Humanos , Papiledema/diagnóstico , Papiledema/etiologia , Papiledema/tratamento farmacológico , Papiledema/fisiopatologia , Feminino , Masculino , Trombose dos Seios Intracranianos/diagnóstico , Trombose dos Seios Intracranianos/tratamento farmacológico , Trombose dos Seios Intracranianos/complicações , Trombose dos Seios Intracranianos/fisiopatologia , Estudos Retrospectivos , Criança , Acuidade Visual/fisiologia , Adolescente , Anticoagulantes/uso terapêutico , Pré-Escolar , Fatores de Risco , Progressão da Doença , Seguimentos , Acetazolamida/uso terapêutico , Imageamento por Ressonância Magnética
7.
Ophthalmology ; 131(3): 341-348, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37742723

RESUMO

PURPOSE: To determine the sensitivity, specificity, and cutoff of macular ganglion cell layer (GCL) volume consistent with optic atrophy in children with syndromic craniosynostosis and to investigate factors independently associated with reduction in GCL volume. DESIGN: Retrospective cross-sectional study. PARTICIPANTS: Patients with syndromic craniosynostosis evaluated at Boston Children's Hospital (2010-2022) with reliable macular OCT scans. METHODS: The latest ophthalmic examination that included OCT macula scans was identified. Age at examination, sex, ethnicity, best-corrected logarithm of the minimum angle of resolution (logMAR) visual acuity, cycloplegic refraction, and funduscopic optic nerve appearance were recorded in addition to history of primary or recurrent elevation in intracranial pressure (ICP), Chiari malformation, and obstructive sleep apnea (OSA). Spectral-domain OCT software quantified segmentation of macula retinal layers and was checked manually. MAIN OUTCOME MEASURES: The primary outcome was determining sensitivity, specificity, and optimal cutoff of GCL volume consistent with optic atrophy. The secondary outcome was determining whether previously elevated ICP, OSA, Chiari malformation, craniosynostosis diagnosis, logMAR visual acuity, age, or sex were independently associated with lower GCL volume. RESULTS: Median age at examination was 11.9 years (interquartile range, 8.5-14.8 years). Fifty-eight of 61 patients (112 eyes) had reliable macula scans, 74% were female, and syndromes represented were Apert (n = 14), Crouzon (n = 17), Muenke (n = 6), Pfeiffer (n = 6), and Saethre-Chotzen (n = 15). Optimal cutoff identifying optic atrophy was a GCL volume < 1.02 mm3 with a sensitivity of 83% and specificity of 77%. Univariate analysis demonstrated that significantly lower macular GCL volume was associated with optic atrophy on fundus examination (P < 0.001), Apert syndrome (P < 0.001), history of elevated ICP (P = 0.015), Chiari malformation (P = 0.001), OSA (P < 0.001), male sex (P = 0.027), and worse logMAR visual acuity (P < 0.001). Multivariable median regression analysis confirmed that only OSA (P = 0.005), optic atrophy on fundus examination (P = 0.003), and worse logMAR visual acuity (P = 0.042) were independently associated with lower GCL volume. CONCLUSIONS: Surveillance for optic atrophy by GCL volume may be useful in a population where cognitive skills can limit acquisition of other key ophthalmic measures. It is noteworthy that OSA is also associated with lower GLC volume in this population. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.


Assuntos
Craniossinostoses , Hipertensão Intracraniana , Atrofia Óptica , Apneia Obstrutiva do Sono , Criança , Humanos , Masculino , Feminino , Adolescente , Células Ganglionares da Retina , Estudos Transversais , Estudos Retrospectivos , Atrofia Óptica/diagnóstico , Tomografia de Coerência Óptica
8.
J Neuroophthalmol ; 43(4): 520-524, 2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-37247249

RESUMO

BACKGROUND: Optic neuropathy in childhood leukemia occurs through multiple direct and indirect mechanisms, including leukemic infiltration of the optic nerve, infection, blood dyscrasias, or adverse effects of treatment. We aimed to characterize visual outcomes in pediatric patients with leukemia-associated neuro-ophthalmic manifestations. METHODS: We retrospectively identified patients with leukemia and optic nerve pathology over 13 years by diagnostic billing codes. We collected information on demographics, presentation, treatment course, and visual outcomes directly from medical records. RESULTS: Of the 19 patients who met inclusion criteria, 17 (89.5%) had pseudotumor cerebri and 2 had direct optic nerve infiltration. Causes of increased intracranial pressure included central nervous system infiltration (6 of 17), hyperviscosity/leukemia (2 of 17), venous sinus thrombosis (3 of 17), medication induced (5 of 17), and bacterial meningitis (1 of 17). 47.1% (8 of 17) had papilledema at the time of leukemia diagnosis, and 94.1% (16 of 17) of patients with pseudotumor cerebri were treated with acetazolamide. At presentation, 3 patients had decreased vision secondary to macular ischemia, subhyaloid vitreous hemorrhage, or steroid induced glaucoma. Following treatment of pseudotumor cerebri, binocular visual acuity was ≥20/25 in all patients. One patient with optic nerve infiltration had a final visual acuity of count fingers in the affected eye. CONCLUSIONS: In our chart review, the most common mechanism of neuro-ophthalmic involvement in pediatric leukemia was elevated intracranial pressure from a myriad of causes. Visual outcomes from patients with elevated intracranial pressure were excellent. Understanding the mechanisms by which leukemia can cause optic nerve disease in pediatric patients can facilitate earlier diagnosis and treatment and potentially improve visual outcomes.


Assuntos
Leucemia , Doenças do Nervo Óptico , Papiledema , Pseudotumor Cerebral , Humanos , Criança , Pseudotumor Cerebral/complicações , Pseudotumor Cerebral/diagnóstico , Pseudotumor Cerebral/terapia , Estudos Retrospectivos , Papiledema/diagnóstico , Papiledema/etiologia , Papiledema/tratamento farmacológico , Olho , Doenças do Nervo Óptico/etiologia , Doenças do Nervo Óptico/complicações , Leucemia/complicações
9.
J AAPOS ; 27(1): 30.e1-30.e5, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36638958

RESUMO

BACKGROUND: The ophthalmologic complications of optic nerve head drusen (ONHD) in adults have been documented, whereas data on the degree of visual morbidity from OHND in children are limited. METHODS: The medical records of all patients diagnosed with ONHD at a single, tertiary care ophthalmology department from January 1, 2010, until July 1, 2018, were reviewed retrospectively. Patients were identified using ICD-9 and ICD-10 codes. Inclusion criteria were age ≤18 years of age and formal documentation of ONHD by ancillary testing. RESULTS: A total of 213 patients (386 eyes with ONHD) met inclusion criteria. Mean age at diagnosis was 10.13 ± 4.09 years, and mean follow-up was 2.76 ± 2.91 years. Formal visual fields were available for 208 eyes. Repeatable visual field defects were noted in 24 eyes (11.5%). The most common defect was a nasal step, which occurred in 11 eyes (45.8%). Fifteen eyes had visual field defects at presentation, and 9 eyes developed field loss within 1.39 ± 0.55 years of diagnosis. There was no correlation found between intraocular pressure and degree of visual field loss. Choroidal neovascular membranes were clinically apparent in 5 eyes and treatment was required in 3 eyes. Nonarteritic ischemic optic neuropathy developed in 2 eyes. CONCLUSIONS: Visual morbidity associated with ONHD in children is common and may develop in a short period of time after initial diagnosis. There was no correlation found with intraocular pressure.


Assuntos
Drusas do Disco Óptico , Disco Óptico , Adulto , Criança , Humanos , Adolescente , Estudos Retrospectivos , Drusas do Disco Óptico/complicações , Testes de Campo Visual/efeitos adversos , Transtornos da Visão/diagnóstico , Morbidade , Tomografia de Coerência Óptica
10.
Curr Opin Ophthalmol ; 33(6): 494-500, 2022 Nov 01.
Artigo em Inglês | MEDLINE | ID: mdl-36094020

RESUMO

PURPOSE OF REVIEW: The purpose of this review is to provide an overview of the ophthalmic findings associated with peripapillary hyperreflective ovoid mass-like structures (PHOMS) in both adult and pediatric patients. RECENT FINDINGS: PHOMS have recently been identified in a number of different ophthalmic disease entities ranging from nonpathologic to pathologic, including but not limited to anatomic abnormalities (tilting in myopia), optic nerve head drusen, optic disc edema from inflammation (optic neuritis, white dot syndromes), vascular insults (ischemic optic neuropathy, retinal vascular occlusion), and papilledema. The mechanism underlying the formation of PHOMS has not been fully elucidated although it has been hypothesized that PHOMS occur secondary to axoplasmic stasis from crowding at the optic nerve head. SUMMARY: Although the clinical significance of the presence of PHOMS remains unclear, PHOMS are associated with several disease processes. Understanding the mechanism behind their formation and their impact on optic nerve head structure and visual function may be relevant in patients with optic nerve head pathology. The presence of PHOMS may also correlate with disease severity and duration. Future studies to evaluate whether the formation of PHOMS may be useful as an early indicator of disease or a prognostic tool are warranted.


Assuntos
Drusas do Disco Óptico , Disco Óptico , Neurite Óptica , Papiledema , Adulto , Criança , Humanos , Disco Óptico/patologia , Drusas do Disco Óptico/complicações , Drusas do Disco Óptico/patologia , Neurite Óptica/complicações , Papiledema/diagnóstico , Tomografia de Coerência Óptica
11.
Front Hum Neurosci ; 15: 769259, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34776912

RESUMO

Cerebral visual impairment (CVI) is a leading cause of visual impairment in children in developed countries, but diagnostic tools to detect CVI are limited. We sought to analyze the visual acuity of children with CVI as assessed by visual evoked potentials (VEPs) and preferential looking test (PLT) to determine whether the relationship between the visual outcomes on these two testing methods may serve as a biomarker of CVI. We performed a retrospective chart review of patients with a confirmed diagnosis of CVI and at least one ophthalmological assessment with visual acuity measured by VEP and PLT. Of the 218 patients included in the study, the most common condition associated with CVI was an underlying genetic disorder (36%, 79/218). Treatment for seizures occurred in the majority of the entire cohort of patients (80%, 175/218). Ophthalmic comorbidities included retinal disease in 23 patients, optic nerve disease in 68 patients, nystagmus in 78 patients, and strabismus in 176 patients. When assessed by either VEP or PLT, visual acuity in children with CVI fell below expected norms. At initial and final presentations, VEP acuity exceeded PLT acuity by one or more octaves, and this difference was greater than expected compared with normal visual development. We propose utilizing this quantifiable disparity between VEP and PLT as a biomarker of CVI.

13.
Semin Ophthalmol ; 36(4): 322-328, 2021 May 19.
Artigo em Inglês | MEDLINE | ID: mdl-33689572

RESUMO

Discovery and characterization of serologic biomarkers has revolutionized the diagnostic framework of systemic and paraneoplastic autoimmune neuro-ophthalmic diseases. Expanding recognition of the multiple ocular and visual manifestations of these conditions highlights the important role of the referring provider in identifying potential cases. Increasing ease of access to serologic testing also enables these practitioners to initiate the diagnostic work-up in suspected cases. We aimed to provide an update on the current knowledge surrounding and use of relevant autoimmune biomarkers by correlating specific clinical neuro-ophthalmic manifestations with autoantibody biomarkers. The utility of select biomarkers for myasthenia gravis, neuromyelitis optica spectrum disorder, myelin oligodendrocyte glycoprotein-IgG-associated disorder, opsoclonus-myoclonus syndrome, anti-collapsin-response mediator protein-5 optic neuropathy, and glial fibrillary acidic protein-IgG-associated disease are discussed with particular focus on the clinical contexts in which to consider testing.


Assuntos
Neuromielite Óptica , Oftalmologia , Doenças do Nervo Óptico , Autoanticorpos , Biomarcadores , Humanos , Doenças do Nervo Óptico/diagnóstico
14.
Am J Ophthalmol Case Rep ; 21: 101017, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33521381

RESUMO

PURPOSE: To report a case of 2-month-old boy with Stevens-Johnson syndrome (SJS)/Toxic epidermal necrolysis (TEN) and ocular involvement that was successfully treated with cryopreserved amniotic membrane transplantation (AMT). OBSERVATION: A 2-month-old otherwise healthy boy was referred to Boston Children's Hospital with extensive rash and desquamation concerning for SJS/TEN. A skin biopsy was performed which showed full-thickness epidermal necrosis. AMT was performed at the bedside under general anesthesia. A combination of tobramycin and dexamethasone ointment was prescribed four times per day. On reassessment two weeks following AMT, the entire ocular surface had healed with no signs of conjunctival and/or corneal inflammation or ulceration. CONCLUSION AND IMPORTANCE: To the best of our knowledge, our case represents the youngest patient with SJS/TEN to be managed by AMT and one of very few cases where acetaminophen is suspected to be the offending agent. This case highlights the efficacy of AMT at such a young age and feasibility of performing the procedure at bedside in these patients It also highlights that SJS/TEN can develop at such young age.

15.
J AAPOS ; 25(1): 62-64, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33348041

RESUMO

We present 2 cases of pediatric patients with reactive infectious mucocutaneous eruption with ophthalmic involvement. In both cases, the disease processes behaved similarly to that seen in patients with Mycoplasma pneumoniae-induced rash and mucositis. Visual outcomes were good.


Assuntos
Exantema , Mucosite , Pneumonia por Mycoplasma , Criança , Olho , Humanos , Mycoplasma pneumoniae
18.
Am J Ophthalmol ; 219: 351-356, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32574770

RESUMO

PURPOSE: To evaluate the natural history and ophthalmologic morbidity of Mycoplasma pneumoniae-induced rash and mucositis (MIRM) and propose a treatment algorithm. DESIGN: Retrospective, interventional case series. METHODS: Retrospective chart review of all MIRM patients examined by the department of ophthalmology at a tertiary children's hospital. Diagnosis was established clinically concomitant with either positive Mycoplasma pneumoniae IgM or PCR testing from January 1, 2010, until December 31, 2019. The main outcome measures were best-corrected visual acuity, long-term ocular sequelae, and duration and type of ophthalmic intervention. RESULTS: There were 15 patients (10 male and 5 female) aged 10.9 ± 4.2 years who had primary episodes of MIRM; of those, 4 had multiple episodes. All patients required topical steroid treatment, 3 required amniotic membrane transplantation, and 1 patient underwent placement of a sutureless biologic corneal badage device. There were no patients who suffered visual loss, but 1 was left with mild symblephara near the lateral canthus in each eye and 2 others had scarring of the eyelid margins and blepharitis. CONCLUSIONS: The ocular morbidity is significantly less in MIRM than in other closely related syndromes such as erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis. However, these patients still require close observation and a low threshold for intervention to avoid permanent ophthalmic sequelae and possible blindness.


Assuntos
Âmnio/transplante , Exantema/microbiologia , Infecções Oculares Bacterianas/microbiologia , Doenças Palpebrais/microbiologia , Glucocorticoides/uso terapêutico , Mucosite/microbiologia , Mycoplasma pneumoniae/isolamento & purificação , Pneumonia por Mycoplasma/microbiologia , Adolescente , Criança , DNA Bacteriano/genética , Exantema/diagnóstico , Exantema/terapia , Infecções Oculares Bacterianas/diagnóstico , Infecções Oculares Bacterianas/terapia , Doenças Palpebrais/diagnóstico , Doenças Palpebrais/terapia , Feminino , Seguimentos , Humanos , Imunoglobulina M/sangue , Masculino , Mucosite/diagnóstico , Mucosite/terapia , Mycoplasma pneumoniae/genética , Mycoplasma pneumoniae/imunologia , Pneumonia por Mycoplasma/diagnóstico , Pneumonia por Mycoplasma/terapia , Reação em Cadeia da Polimerase , Estudos Retrospectivos , Acuidade Visual/fisiologia
19.
Curr Neurol Neurosci Rep ; 20(3): 4, 2020 03 03.
Artigo em Inglês | MEDLINE | ID: mdl-32124097

RESUMO

PURPOSE OF REVIEW: The purpose of this review is to provide an update on advances in the understanding of pediatric demyelinating optic neuritis. RECENT FINDINGS: In the past decade, the disease phenotypes for demyelinating syndromes in children have been more clearly defined. Pediatric optic neuritis may present as a clinically isolated syndrome or in the setting of underlying neurologic disease. In addition to optic neuritis associated with multiple sclerosis or neuromyelitis optica, recent work has identified antibodies to the myelin oligodendrocyte glycoprotein (MOG IgG) as a unique demyelinating cause with distinct features regarding treatment and prognosis. The disease phenotypes for demyelinating pediatric optic neuritis have expanded. Treatment strategies vary and are not universally effective for each cause of demyelinating disease. Accurately distinguishing among these unique clinical syndromes is therefore critical for initiation of appropriate treatment to prevent disability, to maximize visual outcomes, and to provide insight into long-term prognosis.


Assuntos
Esclerose Múltipla , Neuromielite Óptica , Neurite Óptica , Aquaporina 4 , Autoanticorpos , Criança , Humanos , Esclerose Múltipla/complicações , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/terapia , Glicoproteína Mielina-Oligodendrócito , Neurite Óptica/diagnóstico , Neurite Óptica/terapia
20.
JAMA Ophthalmol ; 137(12): 1363-1370, 2019 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-31600369

RESUMO

Importance: Gun violence represents a substantial public health issue, and firearm-related injuries rank second among the causes of injury-related deaths in children aged 0 to 17 years in the United States. Ocular trauma from firearm-related injuries can lead to devastating vision loss, but little is known to date about the specific demographics and characteristics of such injuries in children. Objective: To evaluate the epidemiologic pattern of pediatric firearm-related ocular injuries. Design, Setting, and Participants: This retrospective analysis used deidentified data from the National Trauma Data Bank, the largest national registry of hospitalized trauma cases in the United States. The firearm-related ocular injuries (n = 1972) of pediatric patients (defined as those younger than 21 years) hospitalized between January 1, 2008, and December 31, 2014, were analyzed. Statistical analyses were conducted from July 15, 2017, to June 15, 2019. Exposure: Firearm-related ocular trauma. Main Outcomes and Measures: Pediatric patients with firearm-related ocular injuries were identified using International Classification of Diseases, Ninth Revision, Clinical Modification codes and external causes of injury codes. Patient demographics (age, sex, and race/ethnicity), type of ocular injury, injury intent, geographic location, length of hospital admission, health insurance status, disposition at discharge, Injury Severity Score (ISS), and Glasgow Coma Scale (GCS) score were collected. Results: A total of 8715 firearm-related ocular injuries were identified. Of these injuries, 1972 (22.6%) occurred in pediatric patients, most of whom were male (1678 [85.1%]) and adolescents (1037 [52.6%]), with a mean (SD) age of 15.2 (5) years. Common locations of injury were home (761 [38.6%]) and street (490 [24.8%]). Mean (SD) hospital length of stay was 7.6 (12) days, ISS was 16 (13.1), and GCS score was 11 (5.1). The most common types of firearm-related ocular injuries were open wound of the eyeball (820 [41.6%]) and ocular adnexa (502 [25.5%]), orbital injuries or fractures (591 [30.0%]), and contusion of the eye or adnexa (417 [21.1%]). Patients aged 0 to 3 years had greater odds of unintentional injuries (odds ratio [OR], 4.41; 95% CI, 2.51-7.75; P < .001) and injuries occurring at home (OR, 5.39; 95% CI, 2.81-10.38; P < .001), and those aged 19 to 21 years had greater odds of assault injuries (OR, 2.17; 95% CI, 1.77-2.66; P < .001) and injuries occurring on the street (OR, 1.61; 95% CI, 1.3-1.98; P < .001). Black patients had the greatest odds of having injuries with assault intention (OR, 4.53; 95% CI, 3.68-5.59; P < .001), and white patients had the greatest likelihood for self-inflicted injury (OR, 7.1; 95% CI, 5.92-9.51; P < .001). Traumatic brain injury resulted mostly from self-inflicted trauma (OR, 5.99; 95% CI, 4.16-8.63; P < .001), as did visual pathway injuries (OR, 2.86; 95% CI, 1.95-4.20; P < .001). The inpatient mortality rate was 12.2%. Conclusions and Relevance: This study found that pediatric firearm-related ocular injuries from 2008 through 2014 were predominantly sight-threatening and associated with traumatic brain injury. If the possible risk factors, including sex, age, race/ethnicity, and injury intention, can be confirmed for 2015 through 2019, these findings may be useful in developing strategies to prevent pediatric firearm-related ocular injuries.


Assuntos
Ferimentos Oculares Penetrantes/epidemiologia , Transtornos da Visão/epidemiologia , Ferimentos por Arma de Fogo/epidemiologia , Adolescente , Distribuição por Idade , Criança , Pré-Escolar , Bases de Dados Factuais , Etnicidade , Feminino , Humanos , Lactente , Recém-Nascido , Escala de Gravidade do Ferimento , Classificação Internacional de Doenças , Masculino , Sistema de Registros , Estudos Retrospectivos , Fatores de Risco , Distribuição por Sexo , Estados Unidos/epidemiologia
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