Coats病伴中心凹下纤维结节患眼的黄斑微血管异常观察

Macular vessel defects for Coats'disease associated with subfoveal fibrotic nodule

目的观察Coats病伴中心凹下纤维化结节患眼的黄斑区微血管异常改变。方法横断面研究。2018年1月1日至2021年7月30日于复旦大学附属眼耳鼻喉科医院检查确诊的Coats病伴或不伴中心凹下纤维化结节患者45例45只眼纳入研究。其中,男性40例40只眼,女性5例5只眼;年龄均<21岁。根据是否存在中心凹下纤维结节,将患者分为伴中心凹下纤维结节、不伴中心凹下纤维结节(黄斑水肿或渗出),并据此分为纤维化组、非纤维化组,分别为26例26只眼、19例19只眼。采用光相干断层扫描血管成像仪对患者双眼黄斑区3 mm×3 mm或6 mm×6 mm范围进行扫描,设备自带软件自动处理图像。观察中心凹无血管区(FAZ)是否存在FAZ边缘吻合血管拱环断裂、异常微血管分支(AMB)。结果纤维化组26只眼均可见自中心凹旁视网膜毛细血管网发出的AMB,长入并破坏FAZ边缘吻合血管拱环的完整性;AMB呈交错缠绕状,纡曲扩张。B扫描像可见中心凹下纤维结节内血流信号,其中血流信号横贯于内层视网膜和纤维结节之间23只眼(88.46%,23/26)。非纤维化组患眼血管异常均表现为毛细血管纡曲扩张及缺损,未见FAZ吻合血管拱环断裂、AMB。结论Coats病伴中心凹下纤维结节时,交错紊乱及纡曲扩张的AMB自中心凹旁血管网发出,长入并破坏FAZ边缘吻合血管拱环的完整性,并向下纵向生长至纤维结节内。

Objective:To investigate macular microvascular abnormalities in eyes with subfoveal fibrotic nodules secondary to Coats'disease.Methods:A cross-sectional study.From January 1,2018 to July 30,2021,45 eyes of 45 patients diagnosed with Coats'disease with or without subfoveal fibrotic nodules in Eye and ENT Hospital,Shanghai Medical College of Fudan University were included in this study.There were 40 eyes in 40 males and 5 eyes in 5 females.All were under 21 years old.According to the presence or absence of subfoveal fiber nodules,the patients were divided into fibrotic group(26 cases,26 eyes)and non-fibrotic group(19 cases,19 eyes).Optical coherence tomography angiography was used to scan 3 mm×3 mm or 6 mm×6 mm macular area of both eyes.The software of the device automatically processed the images.The presence of FAZ edge anastomotic vascular arch ring breakage and abnormal microvascular branch(AMB)in the foveal avascular zone(FAZ)were observed.Results:In 26 eyes of fibrosis group,AMB originating from the parafoveal retinal capillary network was observed,which grew into and destroyed the integrity of the vascular arch ring at the edge of FAZ.AMB was crisscrossing and winding,and its curvature expands.B-scan images showed the blood flow signal in the subfoveal fiber nodule,and the blood flow signal traversed between the inner retina and the fiber nodule in 23 eyes(88.46%,23/26).In the non-fibrosis group,all the vascular abnormalities were characterized by capillary dilation and defect,and no breakage of FAZ anastomotic vascular arch ring or AMB was observed.Conclusions:In Coats'disease with subfoveal fiber nodules,staggered and dilated AMBs emerge from the parafoveal vascular network,grow into and destroy the integrity of the vascular arch ring at the edge of FAZ,and grow down longitudinally into the fiber nodules.