Intraorbital hypertelorism with midface defect

Orbital hypertelorism is not a syndrome, but a physical finding in many craniofacial anomalies. It is defined as an increase in the bony interorbital distance. The ethmoid sinuses are normally over-expanded and in some cases of severe hypertelorism, there is an inferior displacement of the cribriform plate.

The midface is in some cases affected with an inverted V-shaped occlusal plane in the maxilla.

Etiology
It is both etiologically and pathogenetically heterogenous and can be found in patients with:

• Frontonasal Malformation
• Craniofrontonasal Dysplasia
• Median and Paramedian Craniofacial Clefts
• Craniosynostosis