ObjectiveTo analyze the clinical pathological features of mushroom shaped uveal melanoma.MethodsRetrospective clinical study. From June 2001 to Aprilh 2013, pathological sections from 102 eyes of 102 patients with uveal melanoma diagnosed in Beijing Tongren Eye Center were included in the study. The sections were divided into group A (mushroom shaped uveal melanoma, 30 eyes) and group B (flat, hemisperical and spherical uveal melanoma, 72 eyes). The height-to-base ratio was calculated. The tumors size was divided into small, medium and large according to the standards of American Multicentre Study of Ocular Melanoma. The tumor cell type was divided into spindle, epithelioid, mixed and others by Improved Callender taxonomy. Motic Med 6.0 software was used to collect and measure the images. The retinal thinning rate of group A and B was compared by χ2 test.ResultsIn group A, large size in 19 eyes (63.3%), medium size in 11 eyes (36.7%). In group B, large size in 49 eyes (68.1%), medium size in 16 eyes (22.%), small size in 7 eyes (9.7%). In group A and B, the average tumor base diameter were 14.2±5.1 mm and 18.7±6.4 mm, respectively; the average tumor height were 10.0±2.1 mm and 7.6±3.9, respectively; the height-to-base ratio were 0.77±0.29 and 0.44±0.28, respectively. In group A, spindle type in 24 eyes (80.0%), epithelioid type in 1 eye (3.3%), mixed type in 5 eyes (16.7%). In group B, spindle type in 52 eyes (72.2%), epithelioid type in 5 eyes (6.9%), mixed type in 11 eyes (15.3%), and others in 4 eyes (5.6%). The proportion of retinal thinning were 73.3% (22 eyes) and 29.2% (21 eyes) in group A and B, respectively. The difference in the proportion of retinal thinning between group A and B was significant (χ2=16.94, P=0.000).ConclusionThe height of the tumor increased rapidly and the retinal thinning rate is high in mushroom shaped uveal melanoma.
ObjectiveTo observe the changes of vision and visual evoked potentials (VEP) in patients with Leber hereditary optic neuropathy (LHON) before and after gene therapy.MethodsA retrospective cohort study. From December 2017 to October 2018, 35 cases of 70 eyes of m11778G.A/MT-ND4 mutant LHON patients who were diagnosed in the Tongji Hospital of Huazhong University of Science and Technology and received gene therapy were included in the study. There were 30 males (87.71%) and 5 females (12.29%), with the mean age of 23.31±6.72 years. The gene therapy method was intravitreal injection of rAAV2-ND4 (recombinant adeno-associated virus carrying NADH-ubiquinone oxidoreductase subunit 4 gene) into one eye. The eye with poor visual acuity was chosen as the injection eye. If both eyes had the same visual acuity, the right eye was designated as the injection eye. Seventy eyes were divided into the injected eye group and the non-injected eye group, in which were both 35 eyes. The best corrected visual acuity (BCVA) and pattern VEP (PVEP) examinations were performed in the injected eye group and the non-injected eye group before treatment (baseline), 1, 3, and 6 months after injection. Compare the changes of BCVA and PVEP between the injected eye group and the non-injected eye group at baseline, 1 month, 3 months, and 6 months after injection. Independent sample t test, paired sample t test or two independent sample nonparametric test were performed to compare the two groups.ResultsCompared with baseline, 1, 3, and 6 months after treatment, the BCVA of the injected eye group (t=3.530, 4.962, 5.281; P=0.001, 0.000, 0.000) and the non-injected eye group (t=3.288, 2.620, 2.252; P= 0.002, 0.013, 0.031) increased, and the difference was statistically significant; there was no statistically significant difference between VEP IT (tinjected eye group=−0.158, 1.046, −1.134; Pinjected eye group = 0.875, 0.303, 0.190; tnon-injected eye group=0.773, −0.607, −0.944; Pnon-injected eye group= 0.445, 0.548, 0.352) and VEP A (Zinjected eye group=−0.504, −0.934, −1.065; Pinjected eye group= 0.614, 0.351, 0.287; Znon-injected eye group=−0.521, −0.115, −0.491; Pnon-injected eye group = 0.602, 0.909, 0.623).ConclusionAfter gene therapy, the visual acuity of the injected and non-injected eyes of LHON patients improved; PVEP did not change significantly, and remained stable compared with baseline.
ObjectiveTo investigate the clinical, ophthalmological and pathological features of primary uveal lymphoma.MethodsRetrospective clinical study. From 2012 to 2018 in Beijing Tongren Eye Cener, 4 cases and 4 eyes of patients with primary uveal lymphoma were included in the study. Among them, 3 cases were male and 1 case was female. The average age was (54 ± 13.58) years old. The average time from initial diagnosis to pathological diagnosis was (18.50 ± 9.29) months. 3 cases were enucleated and 1 case was biopsied. Extranodal marginal zone lymphoma (EMZL) of the mucosa associated lymphoid tissue (MALT) was confirmed by pathological examination. BCVA, fundus color photography, color Doppler ultrasound and orbital MRI were performed in all eyes. UBM, OCT, FFA and ICGA were performed in 2 eyes, 3 eyes, 3 eyes and 2 eyes respectively. The clinical, imaging and pathological changes were observed. Following up time was ≥ 6 months.ResultsAt the initial diagnosis, BCVA was 0.6, 0.02 and 0.01 in 1, 2 and 1 eye respectively. Choroid, ciliary body and iris were involved in 3 eyes, choroid in 1 eye. The fundus of the eyes showed infiltration of choroid in yellow and white color, and the lesions were beyond the vascular arch to the equator and peripheral areas. Color Doppler ultrasonography showed that choroidal diffuse thickening and extrascleral extension (ESE) which was the corresponding hypoechoic areas behind the sclera. Among them, ESE showed crescent thickening in 1 eye and nodular thickening in 3 eyes. UBM showed that the echo of ciliary body was thicken and the internal echo was decreased with the iris involved. OCT showed that RPE was wavy and local retinal neuroepithelial layer detached. FFA showed that the early lesions were mottled with strong and weak fluorescence, and the late fluorescence leakage. The posterior wall of the eyeball was thickened and enhanced in MRI.ConclusionThe clinical manifestations of uveal lymphoma are various, color Doppler ultrasound has characteristic manifestations and ESE of crescent or nodular thickening is valuable in diagnosis.