Objective To investigate the characteristics and diagnostic value of fundus fluorescein angiography (FFA) for familial exudative vitreoretinopathy (FEVR). Methods 34 children (68 eyes) with FEVR and 64 parents (128 eyes) were included. All the clients were received examinations of slit-lamp biomicroscopy and indirect ophthalmoscopy. Meanwhile the children were examined by RetcamⅡ,the best corrected visual acuity of parents were recorded. The children and their parents were classified according to the ocular findings. Among 68 eyes of children, 3 eyes (4.41%) were normal, 4 eyes (5.88%) were in stage 1, 7 eyes (10.29%) were in stage 2, 2 eyes (2.94%) were in stage 3, 8 eyes (11.76%) were in stage 4 and 44 eyes (64.71%) were in stage 5. Among 128 eyes of parents, 74 eyes (57.81%) were normal, 51 eyes (39.84%) were in stage 1, 1 eyes (0.78%) were in stage 2 and 2 eyes (1.56%) were in stage 5. FFA was performed on the children with RetcamⅡunder anesthesia and on the parents with HR2 in order to observe the FFA characteristics in different stage. Results FFA characteristics in children included uncompleted vascularization of the periphery, peripheral avascular zone (stage 1); neovascularization and/or peripheral subretinal and intraretinal exudation (stage 2); subtotal retinal detachment with attached fovea (stage 3); subtotal retinal detachment with detached fovea (stage 4) and total retinal detachment (stage 5). FFA characteristics in parents included abrupt cessation of the peripheral retinal capillary network and a peripheral avascular zone (stage 1); abnormal peripheral arteriovenous shunts, neovascularization or exudation (stage 2) and atrophia bulbi (stage 5). Conclusions FEVR in different stage has different FFA characteristics. FFA plays an important role in early diagnosis of FEVR.
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.
ObjectiveTo investigate the effects of limbal vitrectomy for persistent hyperplastic primary vitreous (PHPV) with disappeared anterior chamber and corneal opacity. MethodsSixteen eyes of 16 children with unilateral PHPV were included in this retrospective study. All the 16 eyes had both anterior and posterior disease, including opaque cornea, disappeared anterior chamber, pupil occlusion and extensive synechia, and lens opacity. The visual acuity was hand movement in 1 eye, and light perception in 15 eyes. There were 7 eyes with microphthalmia, 4 eyes with total retinal detachment, 3 eyes with retinal dysplasia, 1 eye with retinal folds, and 4 eyes with high intraocular pressure preoperatively. All the eyes underwent limbal vitrectomy, including dissection of synechia, pupil formation, lensectomy, posterior lens membrane peeling and vitrectomy. Four eyes with retinal detachment received long-acting gas tamponade following vitrectomy. The mean follow-up was 15.8 months (range from 9 to 21 months). ResultsNo eye had intraoperative complications. After surgery, all the 16 eyes had normal anterior chamber, round pupil and improved corneal transparency. Two eyes achieved a final visual acuity of 20/940 or better. Of the 4 eyes with retinal detachment, 3 eyes achieved retinal reattachment. Of the 4 eyes with preoperative high intraocular pressure, 3 eyes had controlled intraocular pressure postoperatively and the remaining 1 eye underwent glaucoma procedure at 6 months after the primary surgery. ConclusionPHPV eyes with disappeared anterior chamber and corneal opacity have a potential for developing better vision with improved cosmetic outcome and controlled intraocular pressure after vitrectomy by limbal approach.
Recently, the new term "lamellar hole-associated epiretinal proliferation" was introduced to describe a specific epiretinal proliferation. Different from conventional epimacular retinal membrane, lamellar hole-associated epiretinal proliferation (LHEP) can be found around the edge of lamellar macular holes and part of full thickness macular holes. It is defined as a thick homogenous layer of yellowish material without any contractive properties on the epiretinal surface with medium reflectivity on optical coherence tomography images. Immunocytochemical analysis showed the presence of glial cells, fibroblasts, hyalocytes and collagen type Ⅱ. Electron microscopy revealed fibroblasts and hyalocytes as predominant cell types, densely packed in cell agglomerations. LHEP is a secondary event in lamellar macular hole formation and may represent a repair process after large and deep retinal defect. Further studies on its clinical features and clinical significance are still required.
Morning glory syndrome (MGS) is a congenital optic disc anomaly. The characteristic ophthalmoscopic findings consist of a generally enlarged, funnel-shaped and excavated optic disc, surrounded by an elevated annulus of chorioretinal pigment disturbance, with a central glial tuft, multiple narrow branches of retina vessels radiating from the disc. There are peripheral non-perfusion retinal areas in most cases. The pathogenesis of MGS remains unclear. MGS might be associated with many ocular and systemic abnormalities, involving facial, central nervous, cerebrovascular and endocrine systems. Persistent hyperplastic primary vitreous and retinal detachments (RD) are the most common ocular complications of MGS. The mechanism RD in MGS is unclear. Vitrectomy with long-acting gas or silicone tamponade and photocoagulation around the breaks or the enlarged disc might be efficient for rhegmatogenous RD of MGS. Early diagnosis is crucial for recognition and treatment of the ocular and systemic complications, and maintenance of the visual function.