Objective To evaluate the effects of retinal cryotherapy and intravitreal triamcinolone injection to treat Coatsprime; disease with exudative retinal detachment.Methods This was a prospective consecutive case study, and 21 patients (21 eyes) with Coatsprime; disease accompanied by exudative retinal detachment were enrolled. There were 19 males and two females patients, aged from two to 18 years. Fifteen eyes had partial retinal detachment (stage 3A) and six eyes had total retinal detachment (stage 3B). All patients underwent cryocoagulation and intravitreal triamcinolone injection. Three eyes underwent sclerotomy to drain subretinal fluid. Four eyes underwent further treatment by photocoagulation or cryotherapy for the residual abnormal blood vessels after the surgery. The follow-up ranged from three to 15 months with an average of seven months. Visual acuity, intraocular pressure, eye position and eye movements, slit lamp microscope, indirect ophthalmoscope and color fundus imaging were followed up. The last followup time was considered as the judgment time for the therapeutic effects. Results Six eyes had increased intraocular pressure after the surgery, which was controlled by local drug treatment. At the end of follow-up, 19 eyes had reattached retina, one eye had partial retinal detachment and one eye had total retinal detachment. The vision improved in three eyes,unchanged in 14 eyes, and decreased in two eyes. Other complications included strabismus (one eye) and cataract (four eyes). Conclusion The combined treatment of cryotherapy and intravitreal triamcinolone injection is an effective therapy for the Coatsprime; disease with exudative retinal detachment, as retina reattaches and visual function is saved in most patients after this treatment.
ObjectiveTo observe the features of the full field electroretinogram (FF-ERG) in type 1 diabetes (T1D) children without diabetic retinopathy (DR). MethodsRetrospective case study. Forty-one T1D children and 25 age-matched normal controls underwent a complete ophthalmic examination, including best-corrected visual acuity, refraction, intraocular pressure, slit lamp, fundus photography, indirect ophthalmoscopy, and spectral domain optical coherence tomography to exclude DR. All FF-ERG tests were performed by an experienced technician. The ERG series includes six protocols: dark-adapted 0.01 ERG (r-b 0.01); dark-adapted 3 ERG (mix-a 3.0, mix-b 3.0); dark-adapted 10 ERG (mix-a 10.0, mix-b 10.0); dark-adapted oscillatory potentials (OPS); light-adapted 3 ERG (c-a 3.0, c-b 3.0); light-adapted 30 Hz flicker (30 Hz FP) ERG. To compare the amplitudes and implicit times of the FF-ERG between the T1D and control group children. ResultsCompared with the control subjects, the FF-ERG amplitudes decreased and the implicit times increased in T1D. Except for r-b 0.01 (t=-0.228, P > 0.05), the amplitudes of other FF-ERGs were all significantly attenuated (t=-1.664, -3.645, -4.324, -6.123, -5.846, -12.9, -14.4, -5.23; P < 0.05) in T1D children. The implicit times of mix-b 3.0, mix-b 10.0, c-b 3.0 and OP2 significantly increased (t=5.242, 2.879, 5.378, 3.506; P < 0.05). The implicit times of r-b 0.01, mix-a 3.0, mix-a 10.0, c-a 3.0 and 30Hz FP changes were not significantly (t=2.331, 1.677, 0.557, 0.84, 0.064; P > 0.05). ConclusionThe FF-ERG amplitudes decreased and implicit times increased in T1D children compared with the control normal subjects.
ObjectiveTo observe and analyze the clinical characteristics and correlation between the eye and nervous system in children with infantile gangliosideosis.MethodsFrom November 2018 to January 2021, 3 children with infantile ganglion lipidosis diagnosed by genetic examination in the Department of Ophthalmology and Neurology, Beijing Children's Hospital of Capital Medical University, and through China National Knowledge Infrastructure and Wanfang database and The National Library of Medicine of the United States (PubMed) were searched, and 53 cases of Chinese infantile gangliosideosis diagnosed by gene, enzyme activity or pathological examination were selected and a total of 56 cases were included in the study. The searching time was from the establishment of the database to February 2021, and the search keywords are "gangliosideosis", "cherry-spot" macula and "Chinese". The demographic characteristics of 56 cases of children and other system manifestations were analyzed such as eyes, nervous system, skin, bones. According to the presence or absence of cherry-spot (CS) on the fundus examination, the children were divided into a fundus CS group (group A) and a fundus without CS group (group B), with 20 and 27 cases, respectively. The age of onset, gender, different types and neurological manifestations of the two groups of children were compared and analyzed. The non-parametric rank sum test was used for age comparison between groups; the χ2 test or Fisher's exact test were used for the comparison of gender, disease type and incidence between groups.ResultsAmong the 56 children, 27 were males and 29 were females; the median age of onset was 7.0 months. There were 33 and 23 cases of GM1 and GM2, respectively. Among 44 children with visual function examination records, 41 cases (93.2%, 41/44) were unable to follow the visual object. Of 47 children who underwent ocular fundus examination, 20 cases (42.6%, 20/47) had CS on the fundus. The main manifestations of the nervous system are neuromotor development regression or retardation (100%, 56/56), convulsions (58.1%, 25/43), and "startle" phenomena (89.7%, 26/29). Among 42 patients with brain magnetic resonance imaging examination records, 39 cases (92.9%) were abnormal. The incidence of "startle" and seizures in group A was higher than that in group B, and the difference was statistically significant (χ2=5.815, 6.182, P=0.021, 0.013).Conclusios Chinese infantile gangliosideosis is more common in GM1 type. Ocular visual impairment is the visual object as the main manifestation, the incidence of fundus CS is 42.6%, and the symptoms of neurological damage in children with CS are more severe.
ObjectiveTo observe and analyze the clinical characteristics of children who died of intraocular retinoblastoma (RB). MethodsA retrospective clinical study. Fourteen children (23 eyes) with intraocular RB who died after receiving treatment in Beijing Children's Hospital from 2009 to 2017 were included in the study. Among the children, there were 7 males (10 eyes) and 7 females (13 eyes); 5 had unilateral and 9 had bilateral tumor. Age were 17.2±15.5 months. All children underwent RetCam examination. RB was staged according to the international intraocular RB classify. Among the 23 eyes, 1 eye was in stage B, 2 eyes were in stage C, 12 eyes in stage D, and 8 eyes in stage E. Treatment methods included a systemic (vincristine, etoposide and carboplatin) chemotherapy (VEC chemotherapy), enucleation surgery, and vitrectomy. The basic conditions including age, time of diagnosis, pathological diagnosis, treatment and main causes of death were retrospectively analyzed. ResultsAmong the 14 cases, the first symptom was leukemia in 12 cases, red eye in 1 case, and squintin in 1 case. Systemic VEC chemotherapy was used for 1-6 courses of treatment; 5 cases were enucleated, 3 cases underwent histopathological examination; 3 cases were treated with vitrectomy. Among the 3 cases who underwent histopathological examination, the sclera and optic nerve, optic nerve and optic disc were invasted respectively. Seven patients died of tumor metastasis and/or intracranial lesions (50.0%, 7/14); the median survival time was 19 months. Four patients died of treatment (28.6%, 4/14), including 3 patients died of chemotherapy-related side effects, and 1 died of organ failure after enucleation surgery (7.1%); the median survival time was 3.5 months. Early abandonment of treatment died in 3 cases (21.4%, 3/14); the median survival time was 15 months. ConclusionIntracranial metastasis is the main cause of death in children with intraocular RB.