Department of Ophthalmology, Faculty of Medicine, Hadassah-Hebrew University Medical Center, Ein-Karem, 91120, Jerusalem, Israel.
Int Ophthalmol. 2023 Sep;43(9):3107-3113. doi: 10.1007/s10792-023-02710-5. Epub 2023 Apr 17.
To gain insight into the pathogenesis of adult-onset foveomacular vitelliform dystrophy (AFVD) via assessment of its pseudohypopyon stage (PHS).
Retrospectively, data were collected in a tertiary center from established cohorts of a genetically evaluated AFVD and best vitelliform macular dystrophy (BVMD) eyes in the pseudohypopyon stage. Best-corrected visual acuity (BCVA, LogMAR), lesion characterization, including lesion dimensions, liquefaction areas and patterns (altitudinal or lateral), and ellipsoid zone integrity were analyzed from spectral-domain optical coherence tomography images.
Out of 167 eyes of 90 AFVD patients and 56 eyes of 28 BVMD patients, 8 eyes of six AFVD patients and five eyes of four BVMD patients were at the PHS were included. The mean LogMAR BCVA ± SD was 0.21 ± 0.20 and 0.41 ± 0.10 in AFVD and BVMD diseases, respectively (p = 0.13). Seven AFVD eyes (87.5%) demonstrated lateral liquefaction, while all BVMD eyes demonstrated an altitudinal pattern (p = 0.005). Maximal horizontal lesion diameters were 1.41 ± 0.46 mm and 2.64 ± 0.77 mm in AFVD and BVMD, respectively (p = 0.02). AFVD patients were older (69 ± 14) than BVMD patients (22 ± 13; p = 0.009).
The pseudohypopyon stage in AFVD is often characterized by a lateral liquefaction pattern, unlike the altitudinal pattern characterizing BVMD. Age, lesion size, or pathogenesis pathways may underline the different pseudohypopyon stage patterns in AFVD and BVMD.
通过评估假性前房积脓（PHS）阶段，深入了解成年发病性中心性浆液性脉络膜视网膜病变（AFVD）的发病机制。
在一家三级医疗机构，通过回顾性研究，对经基因评估的 AFVD 及假性前房积脓期最佳治疗性湿性年龄相关性黄斑变性（BVMD）眼的队列资料进行了收集。对最佳矫正视力（BCVA，LogMAR）、病变特征，包括病变尺寸、液化区及模式（纵向或横向）、椭圆体带完整性进行了分析，这些资料均来自于频域光学相干断层扫描图像。
在 90 例 AFVD 患者的 167 只眼和 28 例 BVMD 患者的 56 只眼中，纳入了 6 例 AFVD 患者的 8 只眼和 4 例 BVMD 患者的 5 只眼，处于假性前房积脓期。AFVD 和 BVMD 疾病中的平均 LogMAR BCVA ± SD 分别为 0.21 ± 0.20 和 0.41 ± 0.10（p=0.13）。7 只 AFVD 眼（87.5%）显示横向液化，而所有 BVMD 眼均显示纵向液化模式（p=0.005）。AFVD 和 BVMD 的最大水平病变直径分别为 1.41 ± 0.46 mm 和 2.64 ± 0.77 mm（p=0.02）。AFVD 患者年龄较大（69 ± 14），而 BVMD 患者年龄较小（22 ± 13；p=0.009）。
与 BVMD 表现为纵向液化模式不同，AFVD 的假性前房积脓期常表现为横向液化模式。年龄、病变大小或发病机制途径可能是 AFVD 和 BVMD 中假性前房积脓期不同模式的基础。
