Background: Idiopathic intracranial hypertension (IIH) is a disorder characterized by elevated intracranial pressure without secondary causes on neuroimaging. IIH typically occurs in young, obese female patients and, when severe, can cause permanent and irreversible vision loss. The association between skull base thinning in patients with intracranial hypertension and obesity has been previously reported; however, no study has reported these findings in IIH. The goal of our study is to determine whether IIH is independently associated with skull base and calvarial thinning. Methods: A retrospective, matched case-control study was performed. Each patient diagnosed with IIH (case) was matched with a patient diagnosed with headache (control) by age, gender, and race. Patients were included if they underwent computed tomographic imaging of the head, maxillofacial, or orbits within 3 months of their diagnosis. Exclusion criteria were history of skull base or frontal bone pathology because of surgery or skull trauma, central nervous system infections, or incomplete radiologic data. Patient demographics, medical history, clinical examination, and skull base, calvarial, and zygoma thickness were recorded. Skull base thickness was measured by the height of the auditory canal in the coronal plane. Calvarial thickness was measured just anterior to the foramen rotundum in the coronal plane. Extracranial zygoma thickness was mea-
Objective: To assess the association of skin color using Fitzpatrick Skin Type (FST) with metastatic risk of uveal melanoma. Subjects: 854 consecutive patients with uveal melanoma and documented FST Methods: Retrospective detailed review of patient charts was performed for FST (type I- white, II-fair, III-average, IV-light brown, V-brown, VI-black), clinical details of the patient and the uveal melanoma, tumor cytogenetic classification according to The Cancer Genome Atlas (TCGA), and outcome of melanoma-related metastasis and death. Results: The FST classification was type I (n=97 patients), type II (n=665), type III (n=79), type IV (n=11), type V (n=2), type VI (n=0). A comparison of patient FST (type I vs. II vs. III-V) revealed significant differences in mean age at presentation (64.1 vs. 58.5 vs. 49.8 years, p<0.001), race white (100% vs. 98% vs. 75%, p<0.001), presence of ocular melanocytosis (3% vs. 3% vs. 10%, p=0.01), visual acuity <20/200 at presentation (6% vs. 7% vs. 13%, p=0.03), genetic results showing TCGA group B tumors (11% vs. 14% vs. 26%, p=0.01) or TCGA group D tumors (22% vs. 11% vs. 9%, p=0.01), 10-year incidence of melanoma-related metastasis (25% vs. 15% vs. 14%, p=0.02) and 10-year incidence of melanoma-related death (9% vs. 3% vs. 4%, p=0.04). FST was a significant predictor of melanoma-related metastasis (p=0.02, Hazard ratio 2.3). Conclusions: Fitzpatrick skin type may be a predictor of melanoma-related metastasis, with metastasis and TCGA Group D tumors being more common in patients with FST I.
Objective: To determine the correlation of Fitzpatrick Skin Type (FST) and iris color with tumor size (tumor thickness and basal diameter) in patients with uveal melanoma. Methods: Retrospective cohort from a single ocular oncology center of 823 patients with uveal melanoma and documented FST, iris color, and tumor size. Patients were classified into FST (type I, II, and III-V) and iris color (blue, green, and brown) on the basis of external facial photography. There were no FST type VI patients. Tumor thickness was classified into small [<3 millimeter (mm)], medium (3.1-8.0 mm), or large (>8.0 mm), and basal diameter into small (<10 mm), medium (10.1-15 mm) or large (>15mm). The correlation of FST and iris color with tumor thickness and basal diameter was evaluated using the Kruskal-Wallis H test. Results: The FST classification was type I (n=92, 11%), type II (n=643, 78%), or III-V (n=88, 11%), and iris color was blue (n=472, 57%), green (n=102, 12%), or brown (n=249, 30%). A comparison of FST revealed differences in mean tumor thickness (p= 0.04) and basal diameter (p=0.006). Iris color showed no difference for mean tumor thickness (p=0.41) or basal diameter (p=0.48). There was a difference with brown iris color relative to FST III-V for mean tumor thickness (p=0.003) and basal diameter (p=0.001) but no difference with blue or green iris color (p > 0.05). Conclusions: Iris color alone showed no difference in tumor size, but those with brown iris color and FST type III-V demonstrated larger tumor thickness and basal diameter.
According to the World Glaucoma Association (WGA), pediatric glaucoma comprises all disorders with elevated intraocular pressure (IOP)-related damage to the eye in childhood. Unlike adult glaucoma, this definition is not solely based on optic nerve appearance or visual field defects which can be difficult to ascertain in children. Many classification schemes have been used to organize pediatric glaucomas but consensus in the 2013 WGA meeting organizes pediatric glaucomas as primary or secondary. The primary forms, as previously mentioned, include PCG and JOAG. Secondary forms can be acquired from trauma, uveitis, steroid use, tumor, retinopathy of prematurity, or after cataract surgery. Secondary nonacquired forms can include those associated with systemic conditions such as chromosomal disorders, phakomatoses, or those associated with ocular abnormalities such as Axenfeld-Rieger, aniridia, peters anomaly, and iridotrabecular dysgenesis 14 (Table 1).While the WGA discourages the use of words with inconsistent definitions such as developmental, congenital, or infantile, PCG is frequently broken down by age of presentation to: (1) newborn onset (0 to 1 mo), (2) infantile onset (1 to 24 mo), (3) late onset or late recognized ( > 24 mo), and (4) spontaneously arrested cases. Spontaneously arrested cases are rare but represent cases with normal IOP and optic discs but with other typical signs of PCG that are not progressive. Timing of diagnosis may be different in areas with variable health care access and is influenced by severity of disease. While PCG will commonly present between 3 and 9 months of age, newborn onset is the most severe. 15 Etiology and PathogenesisPCG is most often sporadic, however, there are autosomal recessive cases with penetrance ranging from 40% to 100%. 16 Linkage analysis have thus far identified 5 loci that are involved with the development of PCG: GLC3A (located on chromosome 2p22-p21), GLC3B (1p36.2-p36.1), GLC3C (14q24.3), GLC3D (14q24.2-q24.3, not overlapping with GLC3C), and GLC3E (9p21) (16,18). Genes have been identified with 3 of the 5 loci. The GLC3a loci contains the CYP1B1 gene, the GLC3D locus contains latent transforming growth factor beta binding protein 2 (LTBP2) and the GLC3E loci contains tunica internal endothelial cell kinase gene (TEK/TIE2). 15 CYP1B1 mutations are associated with 15% to 20% of PCG cases in Japan and the United States and LTBP2 mutations have been reported in consanguineous Iranian, Pakistani, and Slovakian Roma families. 4,[17][18][19] Genetic testing can identify a cause in 40% of cases of PCG; in cases of parental consanguinity, it is essential to screen current and future siblings. 4
Pediatric uveitis is uncommon, accounting for only 5% to 10% of all uveitis cases. 1 Similar to adults, children with uveitis may be asymptomatic or may present with eye pain, redness, photophobia, and blurry vision. 2 Uveitis can be categorized as anterior uveitis, intermediate uveitis, posterior uveitis, or panuveitis, depending on the part of the eye that is involved. 2,3 Diagnosis is made by examination; findings include anterior chamber (AC) cell and flare, AC fibrin, keratic precipitates, posterior iris synechiae, vitreous cell and haze, vitreous exudates (snowballs), chorioretinitis, and/or retinal vasculitis. 2 AC cell and flare can be graded based on the Standardization of Uveitis Nomenclature Criteria. 2 Complications of uveitis are extensive, including cataracts, glaucoma, hypotony, macular edema, epiretinal membrane, optic disc edema, band keratopathy, posterior synechiae, vision loss, and even permanent blindness. 2,4 Juvenile idiopathic arthritis (JIA) is the most common cause of secondary uveitis in children, with a reported incidence of 4.9 to 6.9 per 100,000 persons per year. 1,5 It is associated with chronic anterior uveitis in 10% to 30% of JIA patients. 2,4 While the majority of patients with JIAassociated uveitis maintain a good best-corrected visual acuity of 20/40 or better, 1 systematic review found that 9.4% of patients with JIA-associated uveitis had a best-corrected visual acuity worse than 20/40. 6 In addition, the incidence of cataracts was 20.5%, glaucoma was 18.9%, and band
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