PURPOSE: To report a case of orbital leiomyoma presenting with proptosis. CASE SUMMARY: A 69-year-old woman presented with a 3-month history of proptosis in her left eye. Intraocular pressure was 17 mmHg in her right eye and 23 mmHg in her left eye. There was a left hypotropia on upgaze. A fundus examination showed retinal folds in the superotemporal area in her left eye. Computed tomography revealed a 2.6 cm-sized well-defined enhancing solid mass in the superotemporal extraconal space of the left orbit, pushing her left eye forward. Lateral orbitotomy, tumor removal, and biopsy were performed. Pathological findings showed a fascicular pattern of benign spindle cells with mild cellular pleomorphism and hyaline degeneration, without mitotic figures. Immunohistochemical stain was positive with smooth muscle actin (SMA), which was compatible with orbital leiomyoma. CONCLUSIONS: Orbital leiomyoma is rare, but it should be considered as a differential diagnosis of orbital tumor when the presenting symptom is proptosis and limitation of ocular movement.
Actins ; Aged ; Biopsy ; Diagnosis, Differential ; Exophthalmos ; Eye ; Female ; Humans ; Hyalin ; Intraocular Pressure ; Leiomyoma ; Muscle, Smooth ; Orbit ; Retinaldehyde

A 68-year-old woman presented with a visual field defect in her right eye. The fundus of her right eye showed multiple telangiectatic vessels, retinal hemorrhages, and subretinal exudates in the inferior peripheral retina. Nine months later, the subretinal exudates extended to the fovea despite treatment with laser photocoagulation. Cryotherapy was not possible at the time because of the posterior location of the retinal telangiectatic vessels. She was treated with a combination of photodynamic therapy (PDT) and intravitreal bevacizumab injection: three injections were given at 2-month intervals. After this combined therapy, her right fundus revealed a significant regression of abnormal retinal vessels and subretinal exudates. A fluorescein angiography showed no leakage from the abnormal retinal vessels. At 9 months after the combined therapy, she was able to maintain a stable visual acuity and visual field. This is the first case report that demonstrates the efficacy of the combined treatment of PDT and intravitreal bevacizumab injection in Coats's disease. This combined therapy is a kind of treatment modality for adult Coats' disease in cases which cryotherapy cannot be employed and are refractory to laser photocoagulation.
Aged ; Angiogenesis Inhibitors/*administration & dosage ; Antibodies, Monoclonal/*administration & dosage ; Drug Therapy, Combination ; Female ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Intravitreal Injections ; *Photochemotherapy ; Retinal Telangiectasis/diagnosis/*drug therapy/physiopathology ; Treatment Outcome ; Visual Fields

PURPOSE:To describe the clinical features and surgical outcomes of congenital cataracts in patients with oculocerebrorenal syndrome. METHODS: We retrospectively analyzed the age, sex, types of cataracts, operation method and time, perioperative visual acuity, postoperative intraocular pressure, and associated ocular disease in 10 patients (20 eyes) that had been diagnosed with congenital cataracts associated with oculocerebrorenal syndrome and undergone cataract surgery with a minimum follow-up period of 1 year. RESULTS: All 10 patients were male and had bilateral cataracts detected on their first full ophthalmic examination. The mean age at diagnosis was 2.5 (2.5+/-1.4) months, and the mean duration of the follow-up period was 4.9 (4.9+/-4.2) years. The types of cataracts were nuclear sclerosis in 18 eyes of nine patients and cortical opacity in two eyes of one patient. All patients underwent irrigation and aspiration of the lenses, posterior capsulectomy and anterior vitrectomy in both eyes before the age of 1 year. Nine patients (18 eyes) underwent cataract extraction before the age of 6 months, and three patients (6 eyes) underwent the surgery before the age of 2 months. Secondary intraocular lens implantation was done in six eyes of three patients at the age of 34 (34+/-13.2) months. Postoperative best corrected visual acuity ranged from light perception to 0.15. Out of the nine patients who underwent cataract extraction before the age of 6 months, seven patients could fix and follow objects moderately. The visual acuity of patient who underwent cataract extraction of each eye at the age of 9 months and 12 months was hand motion. In addition to cataracts, glaucoma occurred in three eyes of two patients, corneal opacity in two eyes of one patient and strabismus in three patients. CONCLUSIONS: Visual acuity after cataract surgery was poor in patients with oculocerebrorenal syndrome, especially when the diagnosis and surgery was delayed. Earlier identification and surgical removal of cataracts is recommended. Patients should be monitored regularly for other ocular diseases such as glaucoma, corneal opacity and strabismus.
Cataract ; Cataract Extraction ; Corneal Opacity ; Eye ; Follow-Up Studies ; Glaucoma ; Hand ; Humans ; Intraocular Pressure ; Lens Implantation, Intraocular ; Light ; Male ; Oculocerebrorenal Syndrome ; Retrospective Studies ; Sclerosis ; Strabismus ; Visual Acuity ; Vitrectomy

PURPOSE: Single umbilical artery (SUA) is the most common malformation of the umbilical cord. However, there have been no studies on the ocular findings in SUA, except for one case report. This study aimed to investigate the ocular findings in children with SUA. METHODS: Fourteen children (eight boys and six girls) with SUA were evaluated retrospectively. All children underwent a complete ophthalmologic examination. RESULTS: The prevalence of abnormal ocular findings in children was up to 42.9%. Refractive errors are detected in four eyes (14.3%): myopia > or =-1.50 diopters (D) in one eye (3.6%) and hyperopia > or =+2.00 D in three eyes (10.7%). Epiblepharon was found in three children (21.4%), and strabismus was detected in one child (7.1%). CONCLUSIONS: Approximately half of the children with SUA showed abnormal ocular findings, therefore, our case series highlight the need for a comprehensive ocular examination and larger prospective research studies in young patients with SUA.
Child, Preschool ; Eyelid Diseases/*congenital/epidemiology ; Eyelids/*abnormalities ; Female ; Humans ; Hyperopia/diagnosis/*epidemiology ; Infant ; Male ; Myopia/diagnosis/*epidemiology ; Prevalence ; Republic of Korea/epidemiology ; Retrospective Studies ; Single Umbilical Artery/*epidemiology ; Strabismus/*epidemiology ; Umbilical Arteries/*abnormalities