Search Results (4)

Marfan syndrome (MFS) is a genetic disorder that affects the connective tissue in several systems, with ocular, cardiovascular, and skeletal system manifestations. Its ocular manifestations include ectopia lentis (EL), myopia, astigmatism, and corneal abnormalities. This review examines refractive alterations related to MFS such as EL, microspherophakia, lens coloboma, altered corneal biomechanics (flattening, thinning, and astigmatism), and myopia and their impact on visual acuity. The pathogenesis of these manifestations stems from mutations in the FBN1 gene (encoding fibrillin-1). Moreover, the current medical and surgical management strategies for MFS-related refractive errors, including optical correction (eyeglasses, contact lenses, etc.), and surgical interventions like lensectomy, intraocular lens (IOL) implantation (anterior chamber, posterior chamber, scleral-fixated, iris-fixated), and the use of capsular tension rings/segments are further discussed. Considering the likelihood of underdiagnosing and underestimating ocular involvement in MFS, this updated review highlights the critical need to identify and address these refractive issues to enhance the visual outcomes for those affected. Full article

While the typical patient with idiopathic intracranial hypertension (IIH) is an obese female of childbearing age, there are unique patient populations, such as non-obese females, that have not been well studied. Characterizing this subpopulation may increase awareness our of it, which may prevent underdiagnosis and improve our understanding of IIH’s underlying pathophysiology. We retrospectively reviewed electronic medical records and compared the clinical and radiological characteristics of non-obese (BMI < 30) and obese (BMI > 30) female patients with IIH. Two hundred and forty-six patients (age 32.3 ± 10) met our inclusion criteria. The non-obese patients (n = 59, 24%) were significantly younger than the obese patients (29.4 ± 9.9 vs. 33.2 ± 10.2, p = 0.004) and had higher rates of severe papilledema (Friesen 4–5; 25.4% vs. 11.8%, p = 0.019), scleral flattening (62.7% vs. 36.9%, p = 0.008), and optic nerve dural ectasia (78.0% vs. 55.6%, p = 0.044). Non-obese patients also had a tendency to have a higher lumbar puncture opening pressure (368 ± 92.7 vs. 344 ± 76.4, p = 0.062). Non-obese patients were three times more likely to present with a combination of scleral flattening and optic nerve dural ectasia (OR = 3.00, CI: 1.57–5.72, χ² = 11.63, α < 0.001). Overall, non-obese females with IIH were found to have a more fulminant presentation, typified by higher rates of severe papilledema and radiological findings typical for IIH. Full article

The aim of this study is to analyze the anterior and posterior corneal surface shape and the corneal thickness difference outcomes between before and after scleral lens (ScCL) wear in post-LASIK ectasia subjects for one year. Twenty eyes with post-LASIK ectasia wearing scleral lenses were evaluated in a visit before contact lens and after 1, 6, and 12 months. The study variables analyzed included the apex, nasal, temporal, inferior, and superior corneal thickness; the anterior and posterior surface corneal at corneal diameters of 8, 6, 4, and 2 mm, and high-contrast visual acuity. A statistically significant increment of corneal thickness (p < 0.05) was observed in the inferior area after 6 months and in the superior area in the 12-month follow-up after wearing ScCLs. The anterior corneal curvature presented a flattening and a statistically significant steepening (p < 0.05) in the central and peripheral radii, respectively, after one year. The posterior corneal curvature showed a significant (p < 0.05) steepening, which mainly affected the central region after one year. Despite these changes, high-contrast visual acuity with ScCL correction remained at the same values. The prolonged use of scleral lenses in post-LASIK subjects showed significant changes in the corneal curvature and thickness. These outcomes recommend more detailed and periodic topographic and vision quality checks to monitor the wear in ScCL patients. Full article

Background: Aside from primary pseudotumor cerebri syndrome (PTCS) with an unknown etiology (i.e., idiopathic intracranial hypertension), which typically occurs in association with obesity, several conditions including cerebral venous abnormalities, drug use, and hormonal imbalance may be a secondary cause of PTCS. However, a focal space-occupying lesion outside of the brain as a cause of PTCS has rarely been reported. Case Presentation: A previously healthy 34-year-old man presented with blurred vision for three weeks. The patient had a three-month preceding history of worsening headache. On admission, he was hypertensive (160/90 mmHg) and underweight with a body mass index of 18.4 kg/m². Fundus examination documented papilledema in both eyes. Neurological examination was unremarkable except for mild nuchal rigidity, and results of routine serologic testing were normal. Gadolinium-enhanced brain magnetic resonance imaging revealed bilateral posterior scleral flattening, suggesting intracranial hypertension. There was no other abnormal brain parenchymal lesion or meningeal enhancement. Cerebrospinal fluid (CSF) assay showed a markedly increased opening pressure (30.0 cmH₂O) with normal CSF composition. A tentative diagnosis of PTCS was made based on ophthalmological, neuroradiological, and laboratory findings. During differential diagnosis, abdomen computed tomography demonstrated a huge benign cystic lesion (14.7 × 10.6 × 16.4 cm) in the right retroperitoneal space, which originated from the mesentery and resulted in hydronephrosis and renovascular hypertension due to external compression of the right kidney. Other evaluations were unremarkable. After successful surgical removal of the cyst, clinical symptoms such as headache, blurred vision, and papilledema on fundus examination were markedly improved, and blood pressure was normalized during the three-month follow-up period. Conclusions: A large retroperitoneal cyst that can increase intra-abdominal pressure could be a rare cause of PTCS. Therefore, meticulous evaluation is warranted for patients with PTCS, especially those without known risk factors. Full article