Stoiber J, Muss W H, Ruckhofer J, Thaller-Antlanger H, Alzner E, Grabner G
Salzburg Eye Clinic, LKA Salzburg, Austria.
Cornea. 2000 May;19(3):395-8. doi: 10.1097/00003226-200005000-00028.
Clinical history of a 17-year-old patient with Leber congenital amaurosis (LCA) with histologically proven recurrent keratoconus (KC) two years after corneal transplantation in one eye and a recurrence-like appearance with a more global contour on the other eye four years after corneal grafting is reported. The possible mechanisms for this recurrence are discussed in light of the fact that this is, to the best of our knowledge, the first penetrating keratoplasty reported in LCA.
Computerized videokeratography (CVKG) and specular microscopy were performed preoperatively. The patient underwent regrafting, and the excised corneal button was examined by light microscopy and transmission electron microscopy.
Analysis of CVKG showed a keratoconus-like pattern on the right eye, with the left eye demonstrating the aspects usually seen in keratoglobus. Histologic examination revealed the features usually observed in progressed keratoconus.
Recurrence of keratoconus in a graft has not yet been described after such a short time until now. A "true" recurrence of the disease is postulated; it could be caused by an "aggressive" genetic factor that also leads to the frequent KC in patients with LCA. This mechanism also could explain the high incidence and rapid progress of KC in this disease.
报告一名17岁患有莱伯先天性黑蒙（LCA）患者的临床病史。该患者一只眼睛角膜移植两年后组织学证实复发性圆锥角膜（KC），另一只眼睛角膜移植四年后出现类似复发且外形更呈球形的表现。鉴于据我们所知这是首次报道的LCA患者穿透性角膜移植术，讨论了这种复发的可能机制。
术前进行计算机化视频角膜地形图（CVKG）和角膜内皮显微镜检查。患者接受再次移植，对切除的角膜植片进行光学显微镜和透射电子显微镜检查。
CVKG分析显示右眼呈圆锥角膜样形态，左眼表现出通常在球形角膜中所见的特征。组织学检查揭示了进展期圆锥角膜通常观察到的特征。
迄今为止，尚未描述过在如此短时间内移植角膜出现圆锥角膜复发的情况。推测为疾病的“真正”复发；可能由一种“侵袭性”遗传因素引起，该因素也导致LCA患者频繁发生KC。这种机制也可以解释该疾病中KC的高发病率和快速进展。
