In recent years, the complexity of intraocular lymphoma has been gradually recognized by ophthalmologists. Although primary vitreoretinal lymphoma is the dominant type of intraocular lymphoma, ophthalmologists should be aware that it is not unique and avoid overgeneralizing specific clinical features to all intraocular lymphoma types. Intraocular lymphoma can be divided into vitreoretinal, uveal (choroid, iris, ciliary body) lymphoma according to the anatomic affected parts. According to pathological cell types, it can be divided into B cells, mantle cells, T cells and natural killer T cells. At the same time, depending on the presence or absence of extra-ocular tissue involvement, it can also be subdivided into isolated intraocular, oculo-central nervous system, oculo-system, and oculo-central nervous system lymphomas. Vitreoretinal lymphoma tends to occur in the elderly with clinical manifestations similar to uveitis and white spot syndrome and limited response to glucocorticoid therapy. The characteristic fundus manifestations include vitreous gauzy or "auroral" opacity and yellowish-white subretinal mass. Optical coherence tomography plays a key role in diagnosis and can reveal specific changes such as vertical strong reflex and intraretinal strong reflex infiltration. It is worth noting that vitreous and retinal involvement may vary, which has guiding significance for the selection of treatment strategies. In contrast, uveal lymphoma has unique clinical and pathological features, such as the chronic course of choroidal mucosa-associated lymphoid tissue (MALT) lymphoma and the equal distribution of T cells and B cells in iris lymphoma. In diagnosis, choroidal lymphoma often requires histopathological examination, and radiotherapy is the first choice for MALT lymphoma. T-cell lymphoma is similar to B-cell lymphoma in ocular fundus appearance, but diagnosis is more difficult and depends on cytopathology and T-cell receptor gene rearrangement. Comprehensive systematic screening is essential for patients with intraocular lymphoma to identify the primary site. Ocular lesions in patients with systemic lymphoma require differential diagnosis, including tumor invasion, secondary infection, and inflammatory lesions. As the incidence of lymphoma increases, ophthalmologists should constantly update their understanding of intraocular lymphoma to provide accurate diagnosis and treatment.
OCT angiography (OCTA) is a fast, noninvasive and quantifiable new technique, which is especially suitable for long-term follow-up in patients with hereditary retinochoroidal degeneration, such as retinitis pigmentosa, Best vitelliform macular dystrophy, adult onset foveomacular vitelliform dystrophy, doyne honeycomb retinal dystrophy, choroideremia and Stargardt disease. During the follow-up, clinicians can find the subtle signs that explain disease development from the blood flow imaging, quantitatively describe the vascular density, timely detect and treat choroidal neovascularization. It is significant to explore the etiology and monitor the course of these diseases. With the development of more treatments for these diseases, OCTA parameters can also be used as indicators to evaluate and compare different therapeutic effects. In the future, more quantitative indicators of OCTA will be applied to evaluate the course of hereditary retinochoroidal degeneration, and provide valuable basis for early diagnosis and treatment.
Torpedo maculopathy is a rare, congenital lesion of RPE, which locates temporal to the macula and along the horizontal raphe. The lesion is torpedo-shaped with its torpedo-like tip pointing towards the fovea. As an incidental finding, it often affects only one eye with no damage to central visual acuity. According to its characteristics on OCT, it is divided into 2 types: typeⅠ, attenuation of outer retinal structures without outer retinal cavitation; typeⅡ, those with both attenuation of outer retinal structures and outer retinal cavitation. Diseases with pigment changes in the RPE layer similar to torpedo maculopathy include congenital hypertrophy of the RPE, RPE lesions in Gardner syndrome, etc. The main point to distinguish the disease from other diseases is its unique location and shape. Most of the torpedo maculopathy lesions are stable and do not require special treatment, but the disease can be complicated by neurosensory retinal detachment, choroidal neovascularization and so on, and symptomatic treatment is needed if necessary.
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.
ObjectiveTo observe the thickness distribution of retina outer nuclear layer (ONL) by directional optical coherence tomography (D-OCT), and analyze variation of ONL thickness with age, gender and anatomical location.MethodsCross sectional observational study. From August 2017 to January 2019, the patients were included who had no abnormal eyes in Beijing Tongren Hospital, and healthy volunteers were included in the study. Cirrus HD-OCT 5-line single line scanning mode was used to scan the macular area horizontally and vertically. The pupil diameter of all the tested eyes was more than 6 mm. The protocol was approved by The Medical College of Wisconsin Institutional Review Board. The detection light was incident on the temporal, nasal, upper and lower sides about 1.5 to 2.0 mm away from the pupil center to obtain an image that was oblique and clearly showed the Henle fiber layer (HFL). The upper and lower bounds of HFL and external limiting membrane (ELM) were manually labeled. The thickness of ONL and HFL+ONL were measured and recorded at 150 μm intervals on the horizontal and vertical radial lines with the fovea as the midpoint. The thickness of ONL in different anatomic location, ages and genders were compared. The influence of age and gender on ONL were analyzed by one-way ANOVA and independent sample t test respectively.Results67 eyes of 67 subjects were enrolled. Among them, the mean age of 27 males (27 eyes) and 40 females (40 eyes) was 38.48±15.33 and 40.98±17.78 years respectively without significantly statistical difference (t=－0.582, P=0.562). The total mean age was 39.97±16.98 years old. There were 11, 22, 22 and 12 patients aged less than 20, 20-39, 40-59 and over 60 years old respectively, according which they were divided into A, B, C and D groups. According to the anatomical location, the thickness of the ONL reached a maximum in the foveola, and then decreased as the eccentricity increased. Horizontally, ONL/ONL+HFL reached the minimum as 36.1% at 0.90 mm on the nasal side, while the minimum was 38.3% at 0.75 mm on the temporal side. Vertically, ONL/ONL+HFL reached the minimum as 36.2% at 0.75 mm inferiorly and 35.6% at 0.9 mm superiorly. There was no significant difference in the ONL thickness of fovea between group A, B,C and D (P＞0.05), however, a significant difference was among the four groups in the parafoveal and the perifovea (P＜0.05). The ONL thickness of the male was larger than that of the female, and the differences between them in parafoveal and perifovea showed statistically significant (P＜0.05).ConclusionsNormal people had the thickest ONL in the fovea. While the location is farther from the fovea, the ONL is thinner. The thickness of ONL in parafovea and perifovea is gradually thin with incerase of age. The thickness of ONL in the male is thicker than that in the female.
ObjectiveTo observe the image characteristics of optical coherence tomography (OCT) in patients with primary vitreoretinal lymphoma (PVRL).MethodsA retrospective clinical study. Thirty-two eyes of 19 patients diagnosed with PVRL by vitreous pathology in the Department of Ophthalmology, Beijing Tongren Hospital from September 2016 to October 2019 were included in this study. There were 7 males and 12 females. The median age was 56 years. The mean time from symptom onset to final diagnosis was 6.1±3.8 months. The first diagnosis was uveitis in 12 cases (63.1%, 12/19), retinal vein occlusion in 2 cases (10.5%, 2/19), central retinal artery occlusion in 1 case (5.3%, 1/19), and suspected PVRL of camouflage syndrome in 4 cases (21.1%, 4/19). Routine ophthalmic examination and frequency-domain OCT examination were performed in all the patients, and typical images were stored for analysis. According to the examination results, PVRL OCT signs were divided into vitreous cells, inner retinal infiltration, outer retinal infiltration, retinal pigment epithelial (RPE) infiltration, sub-RPE infiltration, and subretinal fluid.ResultsVitreous cells were found in all eyes (100.0%, 32/32). RPE infiltrated were observed in 19 eyes (59.4%, 19/32), RPE infiltration in 16 eyes (50.0%, 16/32), outer retinal infiltration in 8 eyes (25.0%, 8/32), inner retinal infiltration in 16 eyes (50.0%, 16/32), and subretinal fluid in 4 eyes (12.5%, 4/32).ConclusionsPVRL OCT signs can involve vitreous and retinal anatomical levels, including vitreous cells, inner retinal infiltration, outer retinal infiltration, RPE infiltration, sub-RPE infiltration and subretinal fluid. The same patient can show multiple signs at the same time.
ObjectiveTo observe and analyze the multimodal imaging characteristics of fundus in patients with sympathetic ophthalmia (SO). Methods A retrospective study. From October 2012 to December 2021, 28 patients (36 eyes) diagnosed SO in the Department of Ophthalmology, Beijing Tongren Hospital were inclued in the study. There were 19 males (25 eyes) and 9 females (11 eyes), with the mean age of 51.61±12.02 years. There were 8 exciting eyes and 28 sympathizing eyes. The time to onset after trauma or surgery was 46.10±107.98 months. All patients underwent examinations including vision test, color fundus photograph, optical coherence tomography (OCT), fundus fluorescence angiography (FFA), indocyanine green angiography (ICGA). Angio-OCT (OCTA) was performed on 3 eyes and fundus autofluorescence (AF) was performed on 8 eyes. The early and late phase were defined respectively as ≤2 months and ＞2 months. Their multimodal imaging characteristics were summarized. ResultsIn 8 exciting eyes, subretinal fibrosis with mutifocal retinal atrophy and pigmentation was noted in 5 eyes (62.50%, 5/8), the other 3 eyes showed sunset glow fundus (37.50%, 3/8). In 28 sympathizing eyes, in the early phase, the fundus photograph showed shallow retinal detachment with optic disc edema in 9 eyes (32.14%, 9/28); in the late phase, peripapillary yellowish-white subretinal lesions in 11 eyes (39.29%, 11/28). In the late course of the disease, there were yellow-white lesions around the optic disc (peridisc) and peripheral subretinal area in 11 eyes (39.29%, 11/28). Dalen-Fuchs nodules were found in 10 eyes (35.71%, 10/28). On OCT, multiple serous retinal detachment and irregular choroidal folds were noted in the early phase; hill-like subretinal hyperreflective elevation was noted in peripapillary area and subfovea with presence of cystic spaces in the intraretina in the late phase. FFA examination showed "pinpoint-like" strong fluorescence in the early stage, and "multi-lake-like" fluorescein accumulation and leakage in the late stage; "map-like" weak fluorescence around the disc in the early stage of the disease, dot-like strong fluorescence lesions in each quadrant of the peripheral retina, and fluorescence in the late stage of the disease course. enhanced. ICGA examination showed that the FFA strong fluorescence lesions in the middle and late stages were weak fluorescence. FAF examination, point-like strong and weak autofluorescence lesions with unclear boundaries. Nine sympathizing eyes with subretinal yellow-white lesions has vision without light-0.1 (significantly decreased vison), while 8 eyes with sunset glow fundus was 0.5-1.0 (mildly decreased vison). ConclusionsSO could not only show the semblable features of acute phases of Vogt-Koyanagi-Harada syndrome, but also the yellowish-white lesions in the peripapillary area, macula and periphery. Most of the eyes with peripapillary lesions has a significantly decreased vison, while the eyes with sunset glow fundus has a mildly decreased vison.