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.
ObjectiveTo observe the clinical features of familial exudative vitreoretinopathy (FEVR). Methods202 FEVR patients (404 eyes) from 84 families were included in this retrospective study, all the clinical diagnosis was made by the same expert in our Department. All patients were examined with slit lamp microscope, indirect ophthalmoscopy, children's retinal imaging system or Heidelberg HR2 fundus cameras for fundus photographs. Fluorescein angiography (FFA) was also performed for all patients, for those≤7 years old it was performed under general anesthesia. Data collected from charts included gender, age at presentation and family history. According to the fundus presentation and FFA results, FEVR was classified into 0-5 stages. Stage 0 (normal) is for the health fellow eye in unilateral FEVR; stage 1: retinal peripheral avascular zone with no fluorescence leakage in FFA; stage 2: retinal peripheral avascular zone, with fluorescence leakage in FFA; stage 3: partial retinal detachment not involving the macular; stage 4: partial retinal detachment involving the macular; stage 5: total retinal detachment. ResultsThere were 119 male patients and 83 females. The average age of diagnosis was 16 months, the average gestational age was 39 weeks and the average birth weight was 3223 g in the proband. At the first clinical visit, there was clear family history of FEVR in 4 patients (1.98%); family history of other eye disease (nystagmus, cataract, small eyes, strabismus and others) in 25 patients (12.38%); not family history of eye disease in 173 patients (85.64%). FEVR family history was confirmed by fundus examination and FFA for the family members. In all 404 eyes, 9 eyes (2.23%) was normal, 162 eyes (40.10%) was stage 1, 97 eyes (24.01%) was stage 2, 72 eyes (17.82%) was stage 3, 20 eyes (4.95%) was stage 4, and 44 eyes (10.89%) was stage 5. In 202 patients, 59 cases (29.21%) was asymmetry bilateral disease with different stage for each eye. In 404 eyes, retinal fold was found in 74 eyes (18.32%). ConclusionsFEVR was common in full-term and/or normal birth weight newborns. More than half of patients are in stage 1 and 2 without any clinical features. 29.21% eyes were asymmetry bilateral diseases.
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.
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.