Central cloudy dystrophy of François (CCDF) is a rare corneal dystrophy characterized by bilateral, symmetric, gray, cloudlike lesions in the central posterior stroma. Diagnosis relies on detailed family history and careful slit-lamp examination to differentiate it from Vogt posterior crocodile shagreen. We describe 5 female siblings from two families (mean age, 58.5 years) with typical bilateral lesions consistent with CCDF. Very few cases of CCDF have been reported, and its pathophysiology and relationship to other corneal stromal dystrophies or degenerations remain poorly understood. These 5 cases constitute the second-largest series and the first documented reports of CCDF in the Mexican population, supported by representative illustrations and accompanied by a comprehensive literature review.
We report the case of a 4-year-old boy diagnosed with keratitis-ichthyosis-deafness (KID) syndrome with long-term follow-up, including multiple surgical and nonsurgical treatments. Genetic testing revealed a missense GJB2 mutation (p.Asp50Asn). His ocular condition deteriorated to the point that, at 37 years of age, he required bilateral keratoprosthesis (KPro) implantation, resulting in best-corrected visual acuity of 20/25 in the right eye and 20/40 in the left eye. Our findings suggest that more advanced cases of KID syndrome may benefit from treatment options that rely less on ocular surface stability, such as KPro implantation, whereas therapies that treat the ocular surface may be preferred in early-stage disease.
Purpose:To characterize the epidemiology of pickleball-related eye injuries in the United States. Methods:Data from the National Electronic Injury Surveillance System were analyzed for individuals treated in US emergency departments (ED) for pickleball-related eye injuries from 2010 through 2023. Results:Based on a sample of 117 actual cases, there were an estimated 6,015 individuals treated in US emergency departments for pickleball-related eye injury. From 2022 to 2023, the rate of injuries increased by 37.3%. The mean age of individuals was 59.9 years. Approximately 54.7% of individuals were male. The most common mechanisms of injury were fall with facial trauma (n = 71 [60.7%]), direct impact from a pickleball paddle (n = 18 [15.4%]), and direct impact from a pickleball (n = 12 [10.3%]). The most frequently associated primary diagnoses were laceration (n = 45 [38.5%]), contusions or abrasions (n = 27 [23.1%]), blunt trauma (n = 17 [14.5), and fractures (n = 14 [12.0%]). The top two specific ocular surface or intraocular diagnoses were abrasions and iritis. Most individuals (n = 115 [98.3%]) were treated and released or examined and released without treatment. Conclusion:Based on our analysis of a national database, the frequency of pickleball-related eye injuries treated in US EDs has increased, although most ocular injuries are clinically minor.
A 5-year-old girl, previously in complete remission from common B-cell acute lymphoblastic leukemia (B-ALL), presented at the University Hospital of Leuven with a unilateral, painless red left eye. Over several weeks, the clinical picture evolved from common conjunctivitis into a painful red eye with iris heterochromia and increased iris vascularization unresponsive to topical and oral antibiotics. No signs of leukemic relapse were detected on comprehensive systemic work-up, including bone marrow and lumbar puncture. Aqueous sampling at this time could not confirm the clinical suspicion of isolated ocular relapse of ALL. Oral prednisolone was initiated, with good clinical response. After discontinuation of steroids, the affected eye developed a hypopyon-like mass in the anterior chamber. Urgent anterior chamber washout was performed. Cytology now confirmed the presence of a leukemic infiltrate, indicating an isolated ocular relapse of common B-ALL. Not only because this was the fourth relapse, but primarily because this patient had already undergone extensive prior treatments, including an allogeneic stem cell transplant just 6 months earlier, there were no systemic therapeutic options available. Palliative radiotherapy to the left orbit was therefore initiated. Two months later, the girl presented with similar symptoms in the right eye, which was also treated with radiotherapy.
We report the case of a 75-year-old man with conjunctival squamous cell carcinoma in his anophthalmic socket that had resulted from enucleation at age 5. He had a 6-week history of a palpable, right orbital mass and periocular pain. Examination revealed a right inferolateral orbital mass and keratinization of the inferior fornix. Magnetic resonance imaging revealed an extensive mass in the inferolateral orbit. Biopsy showed squamous cell carcinoma. The patient underwent exenteration with temporalis flap reconstruction followed by adjunctive radiotherapy. A metastasis to the right parotid was excised at 6 months. This case illustrates the potential for extensive orbital invasion and perineural spread by ocular surface squamous neoplasia in an anophthalmic socket despite minimal conjunctival signs.
