Waizenegger U R, Kohnen T, Weidle E G, Schütte E
Abteilung Augenheilkunde, Bundeswehrkrankenhaus Ulm.
Klin Monbl Augenheilkd. 1995 Aug;207(2):111-6. doi: 10.1055/s-2008-1035357.
Cornea plana is an extremely rare, congenital hereditary malformation of the corneo-scleral shape. The curvatures of cornea and sclera are nearly equal with an indistinct limbus. In addition to the flatness, there is a peripheral sclerocornea that produces a pseudomicrocornea. The low corneal refraction and the short anterior segment often result in hyperopia. Myopia is also described. Usually the posterior segment is not involved.
A young man of 22 years, 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.
The family showed an autosomal dominant inheritance of the cornea plana. The corneal refraction was less than 32 diopters. The scleral encroachment caused an oval cornea measuring horizontally between 5 and 6.5 mm, vertically 4 to 5 mm. Additionally a pseudoblepharoptosis and a conjunctival xerosis of the father and his sons was observed, which is not regularly found. A-scan measuring of the bulbi revealed age-related normal values.
There is no evidence for progression of this anomaly during life. No therapeutical consequences are necessary. To preserve a satisfactory function a conscientious orthoptical maintainance should be guaranteed.
扁平角膜是一种极其罕见的角膜巩膜形状的先天性遗传性畸形。角膜和巩膜的曲率几乎相等，角膜缘不明显。除了扁平外，还有周边巩膜角膜，可产生假性小角膜。角膜低屈光力和前段短小常导致远视。也有近视的描述。通常后段不受影响。
一名22岁的年轻男子、他3岁和5岁的儿子以及他刚出生的女儿都表现出这种角膜遗传性异常。在这种罕见的异常情况中，我们测量了角膜曲率、屈光力、直径，并且在四名患者中的三名中测量了眼球的超声长度。
该家族呈现扁平角膜的常染色体显性遗传。角膜屈光力小于32屈光度。巩膜侵犯导致角膜呈椭圆形，水平径在5至6.5毫米之间，垂直径为4至5毫米。此外，还观察到父亲及其儿子有假性上睑下垂和结膜干燥症，这并非经常出现。眼球的A超测量显示与年龄相关的正常值。
没有证据表明这种异常在生命过程中会进展。无需进行治疗。为保持满意的功能，应确保认真的视光维护。
