Definition
Hypertelorism is a congenital malformation characterized by an abnormal increase in the distance between two organs or parts of the body.
Usually, it refers to the cranial deformation that distances the two eye sockets, caused by an excessive development of the sphenoid wings. The eyes are so far apart, separated by a nose whose root is enlarged.
The hypertelorism must not be confused with the telecanthus, in which the distance between the inner angles of the eyes (intercantal distance) is increased, but that between the external angles remains unchanged. Furthermore, in the telecanthus, the distance between the two pupils is normal, while in hypertelorism it is greater than normal.
Hypertelorism is an anatomical condition associated with a heterogeneous group of congenital diseases, such as Edwards syndrome (trisomy 18) and Turner syndrome.
This malformation is found due to certain chromosomal anomalies also in the context of the "Cri du chat" syndromes, LEOPARD, Apert, Aarskog, DiGeorge, Opitz and Crouzon.
Hypertelorism is generally associated with cranio-facial dysostosis, ocular abnormalities, mental retardation and other bone malformations.
Possible Causes * of Hypertelorism
- Turner syndrome
- Trisomy 13
- Trisomy 18
