Bilateral Simple Ectopia Lentis Associated with FBN1 Gene Mutation

PURPOSE: To report a case of bilateral simple ectopia lentis associated with FBN1 gene mutation. CASE SUMMARY: A 47-year-old women presented with a one-month history of ocular pain and decreased visual acuity of the right eye. She had a family history of crystalline lens dislocation but showed no systemic abnormality or trauma history. Intraocular pressure was 45 mmHg in the right eye, which showed a myopic shift (−6.5 D). The crystalline lens of the right eye was subluxated to the anterior chamber, and the angle was closed. Phacoemulsification with scleral fixation of the posterior chamber intraocular lens of the right eye was performed. After that, best corrected visual acuity of the right eye was 1.0, and intraocular pressure was 15 mmHg. After 2 years, she presented with intermittent ocular pain and decreased visual acuity of the left eye. The crystalline lens of the left eye was subluxated to the anterior chamber. Phacoemulsification with scleral fixation of the posterior chamber intraocular lens of the left eye was performed. After that, intermittent ocular pain and visual acuity of the left eye were improved. Genetic testing confirmed an FBN1 gene mutation in the patient. CONCLUSIONS: A bilateral ectopia lentis patient without history of definite trauma should undergo complete systemic and ophthalmic examination to rule out accompanying disease, and a detailed family history should be collected. If hereditary ectopia lentis is suspected, genetic testing of probands and their family should be performed and will be helpful for genetic counseling and ophthalmic surveillance.

Effects and Prognostic Factors of Automated Thermodynamic System Treatment for Meibomian Gland Dysfunction

PURPOSE: To evaluate the effect and prognostic factors of automated thermodynamic treatment (thermal compression therapy device [KCL 1100®]) for Meibomian gland dysfunction (MGD). METHODS: Patients (48 eyes of 24 subjects) with MGD were recruited for a prospective clinical trial. Patients received 15-minute treatments twice a day using the KCL 1100®. Severity of dry eye symptoms were evaluated using the Standard Patient Evaluation for Eye Dryness (SPEED) and Ocular Surface Disease Index (OSDI), and severity of Meibomian gland function was evaluated using the Meibomian gland expressibility (MGE), Meibomian gland secretion (MGS) score and lipid layer thickness measured by LipiView®. To evaluate ocular surface, we measured tear break-up time (BUT) and fluorescein corneal staining score (Oxford scale). Data were presented for baseline and at 2 weeks and 1 month post-treatment. RESULTS: Dry eye symptom (SPEED, OSDI), Meibomian gland function (MGE, MGS), and ocular surface index (BUT, Oxford scale) of patients were significantly improved from baseline to 2 weeks (p 0.05). There were no significant adverse events during the treatment. CONCLUSIONS: KCL 1100® automated thermodynamic treatment is an effective and safe treatment for MGD. Additionally, KCL 1100® is more effective in patients with moderate dry eye symptom and MGD.

Adult-Onset Asthma and Periocular Xanthogranuloma in a 75-Year-Old Man

PURPOSE: Adult xanthogranulomatous disease of the orbit including adult-onset asthma and periocular xanthogranuloma is rare. To the best of the author's knowledge, adult-onset asthma and periocular xanthogranuloma have not been previously reported in Korea. CASE SUMMARY: A 75-year-old man presented with a 15-year history of progressive bilateral yellowish eyelid mass. He had a history of adult-onset asthma diagnosed 13 years ago. On ocular examination, there were firm yellowish bilateral eyelid masses and ptosis markedly on the right eye. Exophthalmos was noted in the right eye. There were exotropia, hypertropia, and gaze restriction of the right eye. Orbit magnetic resonance imaging demonstrated an ill-defined bilateral infiltrative orbital mass extending to the lid, lacrimal gland, and sinuses. A right orbital mass extended to the posterior orbit. Enlargement of extraocular muscles was also noted in the right eye. Left upper eyelid mass debulking surgery was performed. A combination therapy of azathioprine and prednisolone was started. Mass debulking surgeries of the right upper and lower eyelids were performed. CONCLUSIONS: Adult-onset asthma and periocular xanthogranuloma is known as a refractory disease. Combination therapy with azathioprine and steroid therapy with mass debulking surgery is a good treatment option for adult-onset asthma and periocular xanthogranuloma.