视网膜和视网膜色素上皮联合错构瘤

Combined hamartoma of the retina and retinal pigment epithelium

视网膜和视网膜色素上皮联合错构瘤是少见的良性视网膜肿瘤。好发于儿童,单眼患病。表现为后极眼底单个灰黑色扁平隆起的病变,伴紊乱增生及受牵拉的血管及视网膜前膜。光学相干层析成像(OCT)示病变视网膜增厚,结构紊乱,呈高反射,其后则为低反射影,几乎都伴视网膜前膜。临床上需与黄斑前膜、犬弓蛔虫病、永存胚胎性血管等疾病相鉴别。年幼儿童给予遮盖治疗防止弱视。该类患者应定期观察,如出现视力持续下降,见前膜收缩及血管渗漏加重,并经OCT及荧光素眼底血管造影(FFA)证实,且前膜与视网膜间存在间隙的可考虑玻璃体手术。

Combined hamartoma of the retina and retinal pigment epithelium is a rare benign retinal tumor,common in children with unilateral occurrence. It manifests as solitary black grayish slightly elevated lesion located at posterior pole of fundus, associating with irregular proliferation of blood vessels and preretinal membrane. Optical coherence tomography （OCT） shows localized thickened ancl disorganized retina, with high reflectivity and shadow posterior to it, almost always accompanied by preretinal membrane. Differential diagnosis includes idiopathic preretinal membrane, ocular toxocariasis and persistent fetal vasculature. For treatment, occlusion treatment to prevent amblyopia may improve visual acuity in children. Regular follow-up is needed. When progressive deterioration of vision with increasing contraction of preretinal membrane and vascular leakage are seen and confirmed by OCT and fundus fluorescein angiography （FFA） , especially a potential space is present between preretinal membrane and retina, vitrectomy could be considered.