Elnahry Ayman G, Hassan Fatma K, Abdel-Kader Ahmed A
a Department of Ophthalmology , Cairo University , Cairo , Egypt.
Ophthalmic Genet. 2018 Dec;39(6):759-762. doi: 10.1080/13816810.2018.1536220. Epub 2018 Oct 18.
Gyrate atrophy of the choroid and retina is a rare autosomal recessive condition characterized by chorioretinal atrophy due to deficiency of the enzyme ornithine aminotransferase that can be complicated by intraretinal cystic spaces.
A 15-year-old female complaining of gradually progressive diminution of vision in both eyes preceded by night blindness was found to have gyrate atrophy of the choroid and retina with intraretinal cystic spaces that was evaluated using multimodal imaging including fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography. Functional and anatomical improvement of the intraretinal cystic spaces was achieved with monthly intravitreal bevacizumab injections.
Repeated intravitreal bevacizumab injections can result in anatomical and functional improvement of intraretinal cystic spaces in patients with gyrate atrophy of the choroid and retina.
视网膜脉络膜回旋性萎缩是一种罕见的常染色体隐性疾病,其特征为由于鸟氨酸转氨酶缺乏导致脉络膜视网膜萎缩,并可伴有视网膜内囊样间隙。
一名15岁女性,主诉双眼视力逐渐进行性下降,之前有夜盲症状,经检查发现患有视网膜脉络膜回旋性萎缩并伴有视网膜内囊样间隙,采用包括荧光素血管造影、光学相干断层扫描和光学相干断层扫描血管造影在内的多模态成像技术进行评估。通过每月一次玻璃体内注射贝伐单抗,视网膜内囊样间隙在功能和解剖学上均得到改善。
反复玻璃体内注射贝伐单抗可使视网膜脉络膜回旋性萎缩患者的视网膜内囊样间隙在解剖学和功能上得到改善。