Li Qian, Zhang Shengjuan, Kang Lihua, Wang Donglin, Jiao Xiaodong, Hejtmancik J Fielding, Wang Lifei, Peng Xiaoyan
Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology & Visual Sciences Key Lab, PR China.
Hebei Eye Hospital, Hebei, PR China.
Retina. 2023 Apr 1;43(4):649-658. doi: 10.1097/IAE.0000000000003695.
To characterize the clinical features of macular neovascularization (MNV) secondary to Bietti crystalline dystrophy.
The imaging data of 157 eyes in 79 patients with Bietti crystalline dystrophy were retrospectively reviewed. 12 individuals (19 eyes) were found to have MNVs. Multimodal retinal imaging was used to evaluate the features of MNVs and the primary chorioretinopathy.
The MNV lesions were shown as typical type 2 MNVs with subretinal hyperreflective material (SHRM), and usually detected along the borders of the retinal pigment epithelium/choriocapillaris dropout. The active MNVs were noted in earlier stages of Bietti crystalline dystrophy, while the activity was observed to be reduced in advanced cases. On spectral domain optical coherence tomography, the outer retinal structures were demonstrated to be partially preserved above the SHRMs compared with the extensive atrophy contiguously. Fibrotic scaring of the MNVs was commonly observed and arteriolarization was usually shown within the scars.
MNV was demonstrated to be a common complication secondary to Bietti crystalline dystrophy. The lesions were typical type 2 MNV of varied activities possibly associated with the degrees of the primary degeneration. Choriocapillaris hypoperfusion may participate in MNV development.
描述比埃蒂结晶状营养不良继发黄斑新生血管（MNV）的临床特征。
回顾性分析79例比埃蒂结晶状营养不良患者157只眼的影像学资料。发现12例（19只眼）存在MNV。采用多模式视网膜成像评估MNV及原发性脉络膜视网膜病变的特征。
MNV病变表现为典型的2型MNV，伴有视网膜下高反射物质（SHRM），通常在视网膜色素上皮/脉络膜毛细血管脱失边界处检测到。活动性MNV在比埃蒂结晶状营养不良早期出现，而在晚期病例中活动性降低。在频域光学相干断层扫描上，与广泛的连续性萎缩相比，SHRM上方的视网膜外层结构部分保留。MNV常见纤维化瘢痕形成，瘢痕内常显示小动脉化。
MNV是比埃蒂结晶状营养不良继发的常见并发症。病变为具有不同活动度的典型2型MNV，可能与原发性变性程度有关。脉络膜毛细血管灌注不足可能参与MNV的发生发展。
