Case ReportsRecurrent Keratoconus in a Patient with Leber Congenital AmaurosisStoiber, Josef M.D.; Muss, Wolfgang H. Ph.D.; Ruckhofer, Josef M.D.; Thaller-Antlanger, Helga M.D.; Alzner, Egon M.D.; Grabner, Günther M.D.Author Information From Salzburg Eye Clinic (J.S., J.R., H.T.-A., E.A., G.G.), and the Institute of Pathology (W.H.M.), LKA Salzburg, Salzburg, Austria. Submitted February 2, 1999. Revision received July 28, 1999. Accepted July 29, 1999. Address correspondence and reprint requests to Dr. J. Stoiber, Salzburg Eye Clinic, LKA Salzburg, Muellner Hauptstrasse 48, 5020 Salzburg, Austria. Cornea: May 2000 - Volume 19 - Issue 3 - p 395-398 Buy Abstract Purpose. 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. Methods. 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. Results. 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. Conclusion. 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. © 2000 Lippincott Williams & Wilkins, Inc.