Objective To observe the clinical manifestation and treatment effect of Coatsprime; disease in adulthood. Methods The clinical data of 18 adult patients with Coatsprime; disease from 1980 to 2006 at the department of ophthalmology, Peking Union Medical College Hospital which had been diagnosed by ocular fundus examination and fundus fluorescein angiography (FFA), were retrospectively analyzed. The follow-up period was one year. Coatsprime; response diseases in all the patiens were excluded, such as history of radiation therapy, intraocular inflammation, retinal vascular occlusion, age-related macular degeneration (AMD) and more leakage of diabetic retinopathy. The patients, 14 males and 4 females, 11 left eyes and 7 right eyes, all of them occurs in unilateral; aged from 37 to 55 years with the average age of 43 years; the initial diagnostic vision was 0.02 to 1.5, the visual value was 0.1. 17 patients (17 eyes) were treated by laser photocoagulation, 1 patient was treated by release of retinal operation with condensation. The mean follow up period was 3.7 years (ranged from 1 to 15.3 years). Results In 18 patients (18 eyes), vitreous clear in 14 eyes, vitreous few muddy in 4 years when initial diagnosis. The optic discs in 18 eyes are normal. Lesions confined to one or two quadrant, for the most are in the temporal(15/18 eyes), less are in Bice (4/18 eyes), only one eye at top; two eyes lesions involved in 2 quadrant. There are typical retinal vascular anomalies expansion, miliary aneurysm, arterial aneurysm, macroaneurysms and capillary without perfusion areas; yellowwhite hard leakage by a large block or cluster in Lesion corresponding region. More than half of the eyes with macular edema or leakage; limited retinal detachment in 4 eyes, the range was very wide in 1 eye; retinal hemorrhage in 3 eyes. After treatment the exudation or the hemorrhage were absorbed and retinas were reset. Compared the visual acuity with before treatment, mostly (64.3%) improved 2 lines or keep in 1.2-1.5, no one decreased over 2 lines. The last vision was 0.02-1.5, the visual value was 0.1. Conclusions Coatsprime; disease in adulthood diagnosed in first time has similar characteristics with children, such as vascular anomaly of retina, fundus exudation. The differences including limited area of involvement, less hemorrhages, mild damage on macular, slow development of lesions in follow-up period and better visual prognosis. （Chin J Ocul Fundus Dis，2008，24：279-282）
Paediatric retinal disease is the most important part of paediatric ophthalmology. It usually manifests as leukocoria or yellow pupil, typically in retinopathy of prematurity, familial exudative vitreoretinopathy, persistent hyperplastic primary vitreous, Coats disease and retinoblastoma. It also can be manifested as nystamus poor visual fixation or progressive worsening of visual function, typically in Leber congenital amaurosis, Stargardt disease, Best disease and cone and rod dystrophy. Paediatric retinal diseases can be roughly divided into acquired, hereditary and congenital developmental abnormalities. With the development of gene and stem cell technologies, the advent of new medicine, equipments and new techniques, the concept of diagnosis and treatment in paediatric retinal diseases is also changing. In China, the level is improving progressively in both clinical and research areas of paediatric retinal diseases.
The retinal vessel changes are the primary and major features of retinal vascular diseases. The retinal vessel is part of systemic vessels with its own characteristics to sustain normal retinal function. These basic characteristics are important to the correct understanding and proper treatment of retinal vascular diseases. Always keep in mind that the retinal vessels is one part of the systemic vascular system, thus retinal vascular diseases may have systemic etiology, and systemic drug administration may have a profound effects to the whole body. However retinal vascular system also has its own structural and functional characteristics, thus retinal vascular diseases are also different from the systemic diseases. Finally the main function of retinal vascular network is to maintain the neuro-retinal function, thus we should balance the vision protection and treatments against abnormal retinal blood vessels. Over-treatments may damage the retinal vision.
ObjectiveTo observe the application value and therapeutic efficacy of wide-field digital pediatric retinal imaging system (RetcamⅢ) fundus fluorescein angiograms (FFA) assisted photocoagulation on familial exudative vitreoretinopathy (FEVR). MethodsThe study included 46 eyes of 34 patients with staging 2 FEVR. All patients received color fundus photography and FFA under general anesthesia. The blood vessel reliability of color fundus photography and FFA was comparatively determined. Binocular indirect ophthalmoscope laser photocoagulation was applied to peripheral retina with abnormal leakage as indicated by FFA, the wavelength was 532nm, the duration was 0.25 s and the energy was 200-280 mW. After laser photocoagulation, fundus imaging and FFA was repeated. Further laser photocoagulation was immediately added to areas with vessel leakage but missing the photocoagulation. After treatment, the mean follow-up duration was 14.4 months. The follow up focused on neovascularization, exudative lesions, vitreous traction and merging of photocoagulation spots within 3 months, and on fibrosis membrane resulting in macular traction, tractional retinal detachment, vitreous hemorrhage or Coats disease-like retinal exudates after 3 months. ResultsIt was hard to identify the blood vessels based on the color fundus images and some avascular zone maybe missed. Neovascularization can't be determined by shape of the blood vessels. On the other hand, those new blood vessels can be easily recognized by FFA as leakage sites at the boundary of avascular zone. The surgeon could quickly and accurately locate the FEVR area guided by the color fundus images and FFA from same angle under binocular indirect ophthalmoscope. During the treatment, there was no retinal FEVR area missed laser photocoagulation for all patients. There was no neovascularization, exudative lesions, vitreous traction within 3 months, and no fibrosis membrane, tractional retinal detachment, vitreous hemorrhage or Coats disease-like retinal exudates after 3 months. There were no ocular and systemic complications during and after the FFA and laser photocoagulation. ConclusionWide-field RetcamⅢFFA can help retinal specialists to identify abnormal neovascularization, locate the lesion area, and thus increase the success rate of laser photocoagulation, reduce the ocular and systemic complications for FEVR.
Retinal degeneration mainly include age-related macular degeneration, retinitispigmentosa and Stargardt’s disease. Although its expression is slightly different, its pathogenesis is photoreceptor cells and/or retinal pigment epithelial (RPE) cel1 damage or degeneration. Because of the 1ack of self-repairing and renewal of retinal photoreceptor cells and RPE cells, cell replacement therapy is one of the most effective methods for treating such diseases.The stem cells currently used for the treatment of retinal degeneration include embryonicstem cells (ESC) and various adult stem cells, such as retinal stem cells (RSC), induced pluripotent stem cells (iPSC). and mesenchyma1 stem cells (MSC). Understanding the currentbasic and clinical application progress of ESC, iPSC, RSC, MSC can provide a new idea for the treatment of retinal degeneration.
Retinal angiomatous proliferation (RAP) is a genetic distinct subgroup of exudative age-related macular degeneration which shows a rapid and severe vision loss and high recurrence rates. The pathophysiological mechanisms of RAP is unclear. Recent histopathologic study and en face optical coherence tomography angiography have furthered our understanding of RAP. Clinical features frequently associated with RAP include bilateral disease, presence of reticular pseudodrusen and pigment epithelial detachments. Indocyanine green angiography is the gold standard diagnostic tool. Recently, more and more accurate optical coherence tomography has improved the acknowledgement of stage and diagnosis of RAP. The treatment efficacy of RAP is highly dependent on the stage. Anti-vascular endothelial growth factor therapy is currently the first line of treatment. Other treatment options including combination of photodynamic therapy with antiangiogenic agent intravitreal injections also achieve a reasonable therapeutic outcome. There remain several important questions such as pathogenesis and treatment regimen, to be answered in future RAP research studies.
Radiotherapy is the prior treatment for uveal melanoma, but a major problem confronted most of the patients is radiation retinopathy, which accompanied with severe visual loss and secondary enucleation potential. There is no optium choice and normative strategy so far, the intraocular melanoma society has focused on application of anti-vascular endothelial growth factor drugs injection and glucocorticoids. This article reviews a series of potential managements for radiation retinopathy and its further stage .