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Craniofacial Conditions: Eye & Orbit Deformities

Many craniofacial conditions and syndromes affect the eye region, changing the shape and depth of the orbits (bony sockets), eye placement and eye function. The orbits expand for the first two years of life as the eye grows. Abnormal growth of the bones of the skull and face can cause the displacement of the orbits.

The most common conditions are:
Enopthalmus is a condition present at birth or due to trauma, in which the eyeballs are positioned too deeply into the orbits. Its correction involves surgically reconstructing the orbits and rebalancing the soft-tissue proportions.

Exopthalmus is a condition in which the eyeballs are positioned shallowly within the eye sockets. Considerable portions of the eyeballs may protrude from the orbit. Exopthalmus is caused by a disproportion of the orbital bony volume and the volume of the eye, and is most frequently found congenitally (present at birth) in craniofacial syndromes such as Apert, Crouzon, and Saethre-Chotzen. Exopthalmus may also exist as a sole anomaly, not associated with any syndrome. In older patients, it may be seen as what is known as malignant exopthalmus, which is a result of hyperthyroid disease caused by overproduction of TSH hormone.

Exopthalmus is treated by surgically expanding the outer orbital rim, remodeling the facial bones and advancing the mid-face. When advancement/reconstruction is performed, the enlarged ethmoid sinus may be removed to allow enough space for orbit repositioning.

Hypertelorism is a condition in which the eyes are positioned far apart, hypertelorism may occur alone or as a result of, or in association with, a number of congenital anomalies including: frontonasal dysplasia, craniosynostosis (Apert, Crouzon and related syndromes), and developmental problems related to a nose anomaly, etc.

Hypertelorism is usually diagnosed at birth when the interorbital distance is greater than what is typically seen—less than 20mm at birth (25-30mm is normal for an adult). If not recognizable at birth, hypertelorism may become evident as a baby grows. As the face grows, the distance between the eyes increases, making any disproportion more pronounced. Vision may be (or become) impaired.

When the distance between the eyes is not extreme, surgical correction may include removal of a small amount of mid-line bone, moving the bony eye sockets closer together and eliminating excess skin (if needed). If there is significant distance between the orbits, more extensive correction and reconstruction of the midline area may be required—for example, opening the frontal skull area (frontal craniotomy), removing a specific amount of bone, and bringing the orbits together into a more normal position and slant.