Singh Markandeya, Kuli Jawahar Jyoti, Dutta Anupam, Gogoi Arpita
Department of Ophthalmology, Assam Medical College and Hospital, Dibrugarh, IND.
Department of General Medicine, Assam Medical College and Hospital, Dibrugarh, IND.
Cureus. 2025 Aug 26;17(8):e91018. doi: 10.7759/cureus.91018. eCollection 2025 Aug.
Background This study aimed to evaluate the spectrum of ocular manifestations in sickle cell disease (SCD) patients, with a particular emphasis on retinal involvement, to improve early diagnosis and management strategies. Methodology A cross-sectional, observational study was conducted over one year at Assam Medical College and Hospital, Dibrugarh, recruiting 96 SCD patients aged 10 and above. Comprehensive ophthalmological evaluations included best-corrected visual acuity, slit-lamp biomicroscopy, direct/indirect ophthalmoscopy, spectral-domain optical coherence tomography (OCT), and, in selected cases, fundus fluorescein angiography. Sickle cell retinopathy (SCR) was graded using Goldberg's classification. Results The mean age of the participants was 31.00 ± 13.89 years, with 52 (54.17%) males and 71 (76.04%) from the tea tribe community. Systemic features included pallor in 85 (88.54%) and generalized weakness in 83 (86.46%) participants. Visual acuity of 6/6 to 6/9 was found in 32 (33.33%) (right eye) and 33 (34.38%) (left eye) participants. Anterior segment involvement (corkscrew vessels) was noted in 50 (52.08%) participants, and posterior segment changes were noted in 51 (53.13%) participants. Non-proliferative SCR was present in 46 (47.92%) and proliferative SCR in 5 (5.21%) participants. OCT revealed vitreomacular traction, epiretinal membranes, and full-thickness macular holes. Patient age correlated with retinopathy severity (R² = 0.76, p < 0.01), and systemic severity correlated with retinopathy (R² = 0.94, p < 0.001). Conclusions SCD patients exhibit significant ocular manifestations, particularly retinal pathology, which correlates with age and systemic disease severity. Early and routine ophthalmologic screening is crucial for timely management and preventing vision loss.
背景 本研究旨在评估镰状细胞病（SCD）患者的眼部表现谱，特别关注视网膜受累情况，以改进早期诊断和管理策略。方法 在迪布鲁格尔的阿萨姆医学院和医院进行了为期一年的横断面观察性研究，招募了96名10岁及以上的SCD患者。全面的眼科评估包括最佳矫正视力、裂隙灯生物显微镜检查、直接/间接检眼镜检查、光谱域光学相干断层扫描（OCT），并在选定病例中进行眼底荧光血管造影。镰状细胞视网膜病变（SCR）采用戈德堡分类法分级。结果 参与者的平均年龄为31.00±13.89岁，其中52名（54.17%）为男性，71名（76.04%）来自茶部落社区。全身特征包括85名（88.54%）参与者有面色苍白，83名（86.46%）参与者有全身无力。32名（33.33%）（右眼）和33名（34.38%）（左眼）参与者的视力为6/6至6/9。50名（52.08%）参与者有前段受累（螺旋状血管），51名（53.13%）参与者有后段改变。46名（47.92%）参与者存在非增殖性SCR，5名（5.21%）参与者存在增殖性SCR。OCT显示玻璃体黄斑牵引、视网膜前膜和全层黄斑裂孔。患者年龄与视网膜病变严重程度相关（R² = 0.76，p < 0.01），全身疾病严重程度与视网膜病变相关（R² = 0.94，p < 0.001）。结论 SCD患者表现出显著的眼部表现，尤其是视网膜病变，这与年龄和全身疾病严重程度相关。早期和常规眼科筛查对于及时管理和预防视力丧失至关重要。
