Schatanek S, Krastel H, Rohrschneider K, Kruse F E, Alexandridis E
Universitäts-Augenklinik Heidelberg.
Ophthalmologe. 1994 Jun;91(3):329-32.
Coats disease itself is a rather rare condition, whereas Coats syndrome has been observed in about 1.2-4.9% of all retinitis pigmentosa (RP) patients. The spectrum of clinical presentations and our therapeutic experience are illustrated with reference to case reports of three patients with clinically and electrophysiologically verified RP and Coats syndrome. As in classic Coats disease, Coats-like changes with RP are defined by aneurysmatic and teleangiectatic vessels and intra- and subretinal lipid deposits leading to exudative retinal detachment. Both entities differ with regard to age of onset, gender, bilaterality and location of the retinal changes. The reports published up to now show that there is no consensus concerning the success of the different treatments of Coats syndrome associated with RP. Laser therapy, cryocoagulation, diathermy and buckling procedures have been suggested for treatment of this complex. As in the therapy of Coats disease, our results confirm that the outcome of laser therapy depends on early detection of Coats-type vascular abnormalities and early initiation of the treatment. Therefore, thorough fundus examination should be regularly performed in all RP patients.
科茨病本身是一种相当罕见的病症，而在所有视网膜色素变性（RP）患者中，约1.2%-4.9%的患者观察到了科茨综合征。本文参考了3例经临床和电生理证实为RP和科茨综合征患者的病例报告，阐述了临床表现范围及我们的治疗经验。与典型的科茨病一样，RP合并科茨样改变的特征为动脉瘤样和毛细血管扩张性血管以及视网膜内和视网膜下脂质沉积，导致渗出性视网膜脱离。这两种病症在发病年龄、性别、双侧性及视网膜病变位置方面存在差异。目前已发表的报告表明，对于与RP相关的科茨综合征的不同治疗方法的疗效尚无共识。有人建议采用激光治疗、冷冻疗法、透热疗法和扣带手术来治疗这一复杂病症。与科茨病的治疗一样，我们的结果证实，激光治疗的效果取决于科茨型血管异常的早期发现及治疗的早期开始。因此，应定期对所有RP患者进行全面的眼底检查。
