CONGENITAL, HEREDITARY CORNEA PLANA COMBI NED WITH PTOSIS, PERIPHERALSCLEROCORNEA AND CONJUNCTIVAL XEROSIS

Background Cornea plana is an extremely rare, congenital hereditary ma lformation of the corneo-scleral shape. The curvatures of cornea and s clera are nearly equal with an indistinct limbus. In addition to the f latness, there is a peripheral sclerocornea that produces a pseudomicr ocornea. The low corneal refraction and the short anterior segment oft en result in hyperopia. Myopia is also described. Usually the posterio r segment is not involved. Materials and Methods A young man of 22 yea rs, his three-and five-year-old sons, and his newborn daughter showed this hereditary abnormality of the cornea. In this uncommon anomaly we measured corneal curvature, refraction, diameter and in three of the four patients echographical length of the bulbi. Results The family sh owed an autosomal dominant inheritance of the cornea plana. The cornea l refraction was less than 32 diopters. The scleral encroachment cause d an oval cornea measuring horizontally between 5 and 6.5 mm: vertical ly 4 to 5 mm. Additionally a pseudoblepharoptosis and a conjunctival x erosis of the father and his sons was observed, which is not regularly found. A-scan measuring of the bulbi revealed age-related normal valu es. Conclusions There is no evidence for progression of this anomaly d uring life. No therapeutical consequences are necessary. To preserve a satisfactory function a conscientious orthoptical maintainance should be guaranteed.