Abstract：
OBJECTIVE: To gain insight into the pathogenesis of adult-onset foveomacular vitelliform dystrophy (AFVD) via assessment of its pseudohypopyon stage (PHS).
METHODS: 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.
RESULTS: 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).
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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).
CONCLUSIONS: 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.
摘要：
目的：通过评估成年型囊状卵黄样营养不良（AFVD）的假性发育不全（PHS），了解其发病机制。
方法：回顾，数据是在一个三级中心从已建立的队列中收集的,这些队列包括经过基因评估的AFVD和假性卵黄样黄斑营养不良(BVMD)眼.最佳矫正视力（BCVA，LogMAR),病变表征,包括病变尺寸，液化区域和模式（高度或横向），从光谱域光学相干断层扫描图像中分析了椭球区的完整性。
结果：在90例AFVD患者的167只眼和28例BVMD患者的56只眼中，纳入6例AFVD患者的8只眼和4例BVMD患者的5只眼。在AFVD和BVMD疾病中，平均LogMARBCVA±SD为0.21±0.20和0.41±0.10，分别(p=0.13)。7只AFVD眼（87.5%）表现为侧向液化，而所有BVMD眼睛都表现出高度模式（p=0.005）。AFVD和BVMD的最大水平病变直径分别为1.41±0.46mm和2.64±0.77mm，分别（p=0.02）。AFVD患者年龄（69±14）比BVMD患者（22±13；p=0.009）大。
结论：AFVD的假性不足阶段通常以侧向液化模式为特征，与表征BVMD的海拔模式不同。年龄,病变大小,或发病途径可能强调了AFVD和BVMD的不同假性卵泡减少阶段模式。
方法：回顾，数据是在一个三级中心从已建立的队列中收集的,这些队列包括经过基因评估的AFVD和假性卵黄样黄斑营养不良(BVMD)眼.最佳矫正视力（BCVA，LogMAR),病变表征,包括病变尺寸，液化区域和模式（高度或横向），从光谱域光学相干断层扫描图像中分析了椭球区的完整性。
结果：在90例AFVD患者的167只眼和28例BVMD患者的56只眼中，纳入6例AFVD患者的8只眼和4例BVMD患者的5只眼。在AFVD和BVMD疾病中，平均LogMARBCVA±SD为0.21±0.20和0.41±0.10，分别(p=0.13)。7只AFVD眼（87.5%）表现为侧向液化，而所有BVMD眼睛都表现出高度模式（p=0.005）。AFVD和BVMD的最大水平病变直径分别为1.41±0.46mm和2.64±0.77mm，分别（p=0.02）。AFVD患者年龄（69±14）比BVMD患者（22±13；p=0.009）大。
结论：AFVD的假性不足阶段通常以侧向液化模式为特征，与表征BVMD的海拔模式不同。年龄,病变大小,或发病途径可能强调了AFVD和BVMD的不同假性卵泡减少阶段模式。
