Hypertelorbitism

What is Hypertelorbitism?

Hypertelorbitism, also known as orbital hypertelorism, is an abnormally increased distance between the orbits (the bony sockets holding the eyes). Hypertelorbitism can be found in several different conditions, including:

  • Apert syndrome
  • Crouzon syndrome
  • Craniofrontonasal dysplasia
  • Neurofibromatosis
  • Noonan syndrome
  • Wolf–Hirschhorn syndrome
  • 22q11 deletion syndrome
  • Atypical facial clefts (usually clefts #12, 13 or 14)
  • Encephaloceles, which are a gap in the skull bone through which the brain and/or its surrounding membranes protrude

What are Signs and Symptoms of Hypertelorbitism?

In addition to the widened distance between the eyes, patients may have a flat nasal bridge. If an encephalocele is present, there may be a defect in the skull over the nose and forehead with pulsations from the brain.

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How is Hypertelorbitism Diagnosed?

The diagnosis of hypertelorbitism can usually be made by examining the patient. A CT scan can help accurately measure the distance between the bony orbits.

How is Hypertelorbitism Treated?

The treatment of hypertelorbitism depends on the underlying cause.

  • Encephalocele repair: If an encephalocele is present, the plastic surgeon and neurosurgeon work together to replace the brain and its surrounding membranes inside the skull, and to repair the gap in the bone.
  • Facial bipartition: In this procedure, bone is removed from the area between the eyes to narrow the distance between the eyes and improve hypertelorbitism. This operation is usually performed between 7 and 10 years of age.
  • Box osteotomy: This procedure allows movement of the orbits in any directions, usually to narrow the distance between the eyes. This operation is usually performed after 12 years of age, after the permanent canines have erupted.
  • Rhinoplasty: Rhinoplasty is often needed, usually with rib graft, to improve the projection of the nose.
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