先天性局域性脉络膜发育不良的3D-OCT表现

目的观察先天性局域性脉络膜发育不良的3D—OCT表现特征。方法回顾性分析11例（13眼）临床诊断为先天性局域性脉络膜发育不良患者的3D—OCT检查结果，结合眼底彩照重点分析该病在3D—OCT分层上的表现。结果先天性局域性脉络膜发育不良患者的3D—OCT有以下几个特点：①黄斑区色素上皮带及脉络膜带的弧度发生改变，出现切迹共有8例（9眼），切迹均位于中心凹的颞侧且在黄斑区的水平缝及其附近，断层显示切迹区对应的视网膜色素上皮（RPE）层存在明显的凹陷；②黄斑区视网膜色素上皮带出现连续性中断，其中1眼断层显示缺损区对应的RPE及脉络膜层存在明显的凹陷；③黄斑区色素上皮带和脉络膜带全层缺损，其中2眼表现为中心凹下色素上皮带及脉络膜带闭合不全，呈中空现象；④在色素上皮和脉络膜发育不全处脉络膜反射增高，有的病例显著增高，这是脉络膜结构发生纤维化改变所致。结论先天性局域性脉络膜发育不良患者都有不同程度的黄斑区色素上皮带、脉络膜带的弧度改变或RPE带的连续性中断及缺损，说明眼泡胚胎裂闭锁不够完善，当不合并其他异常时很难发现。因此，对于临床上一些视力不能矫正且无确切弱视诱因的“弱视”患者，建议常规作3D—OCT检查，可能会发现一些亚临床的局域性脉络膜发育不良。

Characteristics of three-dimensional optical coherence tomography in congenital zonal choroidal dystrophy

Objective To observe the characteristics of three-dimensional optical coherence tomography (3D-OCT) in congenital zonal choroidal dystrophy (CZCD) patients. Methods 3D-OCT data from 11 patients (13 eyes) who were diagnosed with CZCD was retrospectively analyzed and combined with fundus photography. Results The characteristics of 3D-OCT in CZCD patients were as follows: ①The reflex curve changes of the retinal pigment epithelium (RPE) and choroid band as well as the notch were observed in 9 eyes of 8 patients. And the notches were all located on the temporal side of the fovea. The separated layer of the retina revealed by 3D-OCT showed that there was an obvious concavity of the RPE corresponding to the notch. ②RPE was discontinuous in 1 eye. The separated layer of the retina revealed by 3D -OCT showed that there was an obvious corresponding concavity in the RPE and choroid band. ③The RPE and choroid band were absent in the macular area in 2 eyes. There was a hollow in the RPE and choroid band under the fovea. ④A higher reflex was observed in the choroid where the RPE and choroid band had dystrophy. This is the result of the fibration of the choroid structure. Conclusion There are different degrees of change in the RPE and choroid membrane of the macular area. These changes include a change in the reflex curve, a discontinuity of the RPE and the absence of the RPE. All of this is the result of an anormaly of the embryonic fissure. It is difficult to discover where there is no complication. Therefore, we suggest that 3D-OCT should be performed routinely when a patient's visual acuity is not normal even if there is no evidence of eye disease. It may be diagnosed as CZCD by 3D-OCT.