Blue sclerotics is a congenital anomaly characterized by a uniform light-blue coloration of the sclera. It is congenital and hereditary and is usually associated with other abnormalities, such as multiple fractures, dislocation of joints, laxity of ligaments, and deafness (van der Hoeve's syndrome). The condition was first described by Adair-Dighton (1912), but van der Hoeve and de Kleyn (1918) were the first to include deafness definitely with this congenital anomaly, and the symptom-complex is accordingly often known as van der Hoeve's syndrome.
It is generally accepted that the unusual appearance of the sclera is due to its abnormal translucency. The color, which is usually evident at birth, does not change throughout life. The color is, however, darker in the anterior part of the sclera, over the ciliary body. In the absence of a sufficient number of microscopic examinations of the sclera in this syndrome, any dogmatic opinion regarding
GUPTA BKD, SEN GC, BASU RK. Blue Sclerotics with Fragilitas Ossium and Deafness. AMA Arch Ophthalmol. 1957;57(3):435–440. doi:10.1001/archopht.1957.00930050447017
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