Hypertelorism

The term hypertelorism is used to describe an abnormal increase in the distance between the bony orbits (sockets) in which the eyes lie.  It is not a diagnosis or a condition, but the feature of wide set eyes with excess bone in between.

Causes of hypertelorism

Hypertelorism can have many different underlying causes. Before treatment, it is important to identify the underlying cause. Genetic testing, as well as CT and MRI scans, are useful tools when diagnosing the cause of hypertelorism. The following are some of the most common causes:

• A mass or growth pushing the two orbits apart
• Abnormal development of the bones of the forehead and base of skull
• Premature fusion of the bony plates of the skull
• A cleft between the bones of the nose or upper face
• A cleft in the bone between the eyes
• Encephaloceles
• Craniofrontonasal dysplasia
• Craniosynostosis syndromes
• Apert syndrome
• Crouzon syndrome
• Aarskrog syndrome

Treatment of hypertelorism

Treatment for hypertelorism is complex and typically requires a team of skilled craniofacial surgeons and ophthalmologists. As mentioned, it is essential to determine the cause of hypertelorism before planning corrective surgery. Surgeons need to know if the condition was caused before birth or by a growth that is worsening. Once all the facts are known, treatment decisions can be made to ensure the best, long-term results for the child. There are several types of surgery that can be performed depending on the severity of the condition.

• Extracranial correction: This surgery is performed for minor cases of orbital hypertelorism. Without going inside the skull, the surgeon brings the bones of the inner portions of the eye sockets and nose closer together.
• Intracranial correction: When orbital hypertelorism is more severe, the surgical correction becomes more complicated. With intracranial correction, an incision is made from ear to ear. The forehead is temporarily removed and the brain is retracted. This surgery is usually performed by a plastic surgeon and a neurologist working together. The entire eye sockets are then cut and moved along with the eyes toward the middle of the face. Excess bone is also removed. If other areas of the face are displaced, they can be moved at this time as well. Soft tissue is then redraped.
• Secondary surgeries: Sometimes, secondary procedures are needed to rebalance the eyes, improve drainage, remove excess soft tissue and other irregularities that may occur as the child grows and develops.
• Box osteotomy: This technique involves removing a piece of bone between the eyes and moving the bones containing the eye sockets closer together and into that area.
• Facial bipartition: This surgery involves removing a piece of bone shaped like an upside down triangle from the area between the eyes. The two sides of the upper face are then rotated inward to bring the eyes closer together.

Often surgery for hypertelorism can be combined with other craniofacial procedures as needed.

Timing of treatment for hypertelorism

The timing of treatment is extremely important. The bones of very young children are not strong enough for surgery. There is also a chance surgery done early could interfere with the growth and development of the face. Unless other complications such as vision problems exist, surgery is typically not performed until after the age of 5. However, because psychosocial issues tend to worsen around age 8, it is often a goal to complete surgery by this time.

As with all craniofacial surgeries, follow-up is essential to watch for complications and to make sure development continues as expected.

Please contact the Craniofacial Team of Texas if you would like to schedule an appointment.

If you would like more information about this craniofacial anomaly, please contact the Craniofacial Team of Texas by calling 512-377-1142 or toll free 877-612-7069 to schedule an appointment or complete an online appointment request.