Objective: To report various intraocular conditions that mimic retinoblastoma. Methods: A review was conducted of eyeballs enucleated for suspected retinoblastoma between 2003 and 2007, and referred for histopathological confirmation. The slides of cases not histopathologically consistent with the diagnosis of retinoblastoma were reexamined. Clinical records and results of neuroimaging studies were reviewed retrospectively Results: Of the 197 eyeballs examined, 182 (92%) proved to be retinoblastoma on histological exam, while 15 (8%) from 13 patients were pseudoretinoblastomas. The age of patients ranged from 4 months to 9 years, with a mean of 35.5 months. The etiologies of the pseudoretinoblastomas were as follows: persistent primary hyperplastic vitreous (PHPV) in 5 eyeballs (33%); retinal dysplasia in 3 (20%); Coats’disease, phthisis bulbi, and vitreous hemorrhage with retinal detachment in 2 (13%) each; and granulomatous endophthalmitis in 1 (8%). Conclusion The 8% erroneous diagnosis was lower than the published rates of 10 to 20%. The common etiologies of pseudoretinoblastoma were similar to those reported.
Retinoblastoma ; Retinal Telangiectasis ; Retinal Dysplasia

No abstract available.
Diagnosis, Differential ; Fluorescein Angiography ; Fundus Oculi ; Hamartoma/*congenital/diagnosis ; Humans ; Male ; Retinal Diseases/*congenital/diagnosis ; Retinal Pigment Epithelium/*pathology ; Retinal Telangiectasis/*diagnosis ; Tomography, Optical Coherence ; Young Adult

PURPOSE: For vitreous hemorrhage induced by coats-types retinitis pigmentosa, we report a case treated with pars plana vitrectomy and endolaser photocoagulation. CASE SUMMARY: A 24-year-old male who was diagnosed with retinitis pigmentosa in both eyes 6 years earlier presented with decreased visual acuity in his left eye for the last 7 months. Corrected visual acuity was measured at 0.06 in the left eye and fundus examination revealed a vitreous hemorrhage in the left eye as well as an exudative lesion in the right eye's peripheral retina, which suggested Coats-type retinitis pigmentosa. The left eye was treated with pars plana vitrectomy. After removal of the vitreous hemorrhage, endolaser photocoagulation was performed around the peripheral exudative lesion that caused the vitreous hemorrhage. One month later, the best-corrected visual acuity increased to 0.20 in the left eye, and there was an improvement in the vitreous hemorrhage and the exudative lesion. CONCLUSIONS: Pars plana vitrectomy and endolaser can be helpful in vitreous hemorrhage induced by coats-type retinitis pigmentosa.
Humans ; Light Coagulation ; Male ; Retina ; Retinal Telangiectasis ; Retinitis Pigmentosa* ; Retinitis* ; Visual Acuity ; Vitrectomy* ; Vitreous Hemorrhage* ; Young Adult

Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Male ; Retinal Telangiectasis ; diagnosis ; therapy ; Retinopathy of Prematurity ; diagnosis

PURPOSE: To report a case of bilateral macular holes in a patient with bilateral macular telangiectasia (Mac Tel). CASE SUMMARY: A 61-year-old male presented with decreased central vision in both eyes. His best corrected visual acuity (BCVA) was 0.8 in the right eye and 0.6 in the left eye. On fundoscopy, fluorescein angiography (FAG), and optical coherence tomography (OCT), he was diagnosed with Mac Tel type 2 combined with stage 1A of the right eye and stage 1B impending macular hole in the left eye. Two years and 8 months later, the BCVA of both eyes was unchanged. On fundoscopy, FAG and OCT, there were no definitive changes in both eyes. The patient was observed without treatment. CONCLUSIONS: Patients with Mac Tel type 2 may be predisposed to the development of a macular hole.
Eye ; Fluorescein Angiography ; Humans ; Male ; Middle Aged ; Retinal Perforations ; Telangiectasis ; Tomography, Optical Coherence ; Vision, Ocular ; Visual Acuity

PURPOSE: We report the detection of peripheral retinal vascular abnormalities in the fellow eye with normal fundus in children with unilateral Coats' disease. METHODS: The clinical records of patients diagnosed with Coats' disease were retrospectively reviewed. We recorded the subjects' characteristics and obtained fundus photography and fluorescein angiography (FA) images. The main outcome measure was peripheral vascular abnormalities in the contralateral eye with normal fundus in children with unilateral Coats' disease, observed with FA. RESULTS: Out of 47 patients with Coats' disease, two (4.3%) were diagnosed with clinically bilateral Coats' disease. Of the 45 patients with presumed unilateral Coats' disease, four (8.9%) had bilateral abnormal peripheral vasculature in FA. The mean age of these four patients was 6.4 ± 5.4 years (range, 1 to 14 years), and three patients were male (75%). All four had peripheral retinal nonperfusion, and two (50%) received laser photocoagulation due to peripheral leakage with telangiectatic vessels. CONCLUSIONS: Coats' disease may more often be a bilateral disease with asymmetry than previously thought. Patients with Coats' disease should undergo careful examination of the fellow eye with FA in order to detect and treat vascular abnormalities that are not visible clinically.
Child ; Fluorescein Angiography ; Fluorescein ; Humans ; Light Coagulation ; Male ; Outcome Assessment (Health Care) ; Photography ; Retinal Telangiectasis ; Retinaldehyde ; Retrospective Studies

PURPOSE: To evaluate the clinical characteristics and treatment result of Coats' disease in children. METHODS: Data on demographics, clinical presentation, and ocular findings were analyzed for 67 eyes of 67 patients with Coats' disease by retrospective chart review. Among these, treatment results were analyzed from 56 eyes which had received local treatment or subretinal fluid drainage (SRFD). Treatment was considered successful when telangiectatic vessels or exudates had regressed after local treatment or when pupillary block glaucoma was treated or prevented after SRFD. RESULTS: Mean age at diagnosis was 5.0 years (5 months-15 years) and 90% were males. 67% of retinal telangiectasias were located in the temporal. There were exudative retinal detachments in 46 (69%) eyes. Among them, SRFD was performed in 19 (28%) to treat or prevent pupillary block glaucoma. The mean age at diagnosis of patients that underwent treatment was 5.1 years. In 37 of 56 patients, 95% of retinal telangiectasias were regressed after primary local treatment, and 21 eyes (31%) underwent SRFD. Management of glaucoma was achieved after SRFD in 16 patients, and one more SRFD was needed after primary SRFD in 5 patients. CONCLUSIONS: In Coats' disease, it is necessary to eradicate retinal telangiectasia with local treatment and to follow up carefully for secondary glaucoma, which can be treated with SRFD in cases of severe exudative retinal detachment.
Child* ; Cryotherapy ; Demography ; Diagnosis ; Drainage ; Exudates and Transudates ; Follow-Up Studies ; Glaucoma ; Humans ; Male ; Retinal Detachment ; Retinaldehyde ; Retrospective Studies ; Subretinal Fluid ; Telangiectasis

PURPOSE: To investigate the clinical and demographic features of idiopathic macular telangiectasia (MacTel) in Korean patients since the introduction of spectral domain optical coherence tomography (SD-OCT). METHODS: We reviewed medical records of patients who were diagnosed with MacTel from 2009 to 2013. All patients underwent fluorescein angiography and SD-OCT and were classified as type 1 or type 2 according to the classification system proposed by Yannuzzi. RESULTS: Over a period of 5 years, 4 (18.2%) patients were diagnosed with type 1 MacTel and 18 (81.8%) patients were diagnosed with type 2 MacTel. All patients with type1 MacTel were male, and their mean age was 51 +/- 8.6 years. Among patients with type 2 MacTel, 3 (16.7%) were male, 15 (83.3%) were female, and the mean age was 60 +/- 13.6 years. Whereas all type 1 MacTel patients had either metamorphopsia or mild scotoma, of the 18 patients with type 2 MacTel, only 4 (22.2%) had those symptoms, 10 (55.6%) complained of only mild visual impairment, and the other 4 (22.2%) had no symptoms. Intraretinal cystoid spaces were observed in 26 (72.2%) of 36 eyes with type 2 MacTel by SD-OCT. These cystoid spaces had irregular boundaries and did not correspond to angiographic leakages. CONCLUSIONS: Type 2 MacTel was most common in the present study. The wider availability of SD-OCT may have contributed to the diagnosis of type 2 MacTel. Type 2 MacTel may be more prevalent than type 1 in Koreans, which corresponds to the results of Western countries.
Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Male ; Middle Aged ; Republic of Korea/epidemiology ; Retina/pathology ; *Retinal Telangiectasis/diagnosis/epidemiology/pathology/physiopathology ; Retrospective Studies

PURPOSE: To investigate the clinical and demographic features of idiopathic macular telangiectasia (MacTel) in Korean patients since the introduction of spectral domain optical coherence tomography (SD-OCT). METHODS: We reviewed medical records of patients who were diagnosed with MacTel from 2009 to 2013. All patients underwent fluorescein angiography and SD-OCT and were classified as type 1 or type 2 according to the classification system proposed by Yannuzzi. RESULTS: Over a period of 5 years, 4 (18.2%) patients were diagnosed with type 1 MacTel and 18 (81.8%) patients were diagnosed with type 2 MacTel. All patients with type1 MacTel were male, and their mean age was 51 +/- 8.6 years. Among patients with type 2 MacTel, 3 (16.7%) were male, 15 (83.3%) were female, and the mean age was 60 +/- 13.6 years. Whereas all type 1 MacTel patients had either metamorphopsia or mild scotoma, of the 18 patients with type 2 MacTel, only 4 (22.2%) had those symptoms, 10 (55.6%) complained of only mild visual impairment, and the other 4 (22.2%) had no symptoms. Intraretinal cystoid spaces were observed in 26 (72.2%) of 36 eyes with type 2 MacTel by SD-OCT. These cystoid spaces had irregular boundaries and did not correspond to angiographic leakages. CONCLUSIONS: Type 2 MacTel was most common in the present study. The wider availability of SD-OCT may have contributed to the diagnosis of type 2 MacTel. Type 2 MacTel may be more prevalent than type 1 in Koreans, which corresponds to the results of Western countries.
Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Male ; Middle Aged ; Republic of Korea/epidemiology ; Retina/pathology ; *Retinal Telangiectasis/diagnosis/epidemiology/pathology/physiopathology ; Retrospective Studies

PURPOSE: To report a case of bilateral Coats' disease. CASE SUMMARY: A 19-month-old boy presented with esodeviation of his eyes, which started 5 months prior. A fundus exam showed total bullous exudative retinal detachment with retinal vascular telangiectasia in the right eye and localized exudative retinal detachment with vascular telangiectasia at the inferior periphery in the right eye. Fluorescein angiogram of the left eye showed retinal telangiectatic vessels, avascular area and fluorescein leakeage from telangiectatic vessels. The patient received external drainage of subretinal fluid and intravitreal air injection of the right eye and Argon LASER photocoagulation and cryotheraphy of the left eye. A cytologic exam of the subretinal fluid drained from the right eye showed no malignant cells. Forty-four months after the operation, his best corrected visual acuity was no light perception in the right eye and 0.4 in the left eye. Both fundi were flat and stable. No complications, such as glaucoma, recurred retinal detachment, or pain, occurred. CONCLUSIONS: Coats' disease rarely occurs bilaterally and can be involved asymmetrically. The disease presents more severely when bilateral and can progress after long-term observation. Proper treatment and long-term follow-up of both eyes are necessary to prevent visual loss and preserve eyes.
Argon ; Drainage ; Esotropia ; Eye ; Fluorescein ; Follow-Up Studies ; Glaucoma ; Humans ; Infant ; Light ; Light Coagulation ; Retinal Detachment ; Retinaldehyde ; Subretinal Fluid ; Telangiectasis ; Visual Acuity