Eye and Orbit Deformities
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Encephalocele Enopthalmus Exopthalmus Hypertelorism |
Description The orbit is the bony area surrounding the eyeball. These bony shells of the eye 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 orbit. Craniofacial surgery is often required to correct eye and orbit deformities. |
Encephalocele
An Encephalocele, which may be found anywhere in the craniofacial region, forms when the brain tissue pushes through a bony defect of the skull. This skull defect can be caused by abnormal skull growth and may be part of various syndromes. An Encephalocele usually occurs in the fronto-orbital region or as a herniation located between the orbits, but it also occurs on the top of the head or skull at any location or in the base of the skull. When it presents in the intraoral cavity, it causes clefting of the palate. Read More...
Enopthalmus
Enopthalmus is a condition in which the eyeballs are placed too far back in the orbit or sunken in. It is either congenital or caused by trauma. Its correction involves treatment of the soft-tissue disproportion and the bony orbital contents.
Exopthalmus
This term is used to describe protrusion of the eyeballs form the orbit. It is caused by a disproportion of the orbital bony volume and the volume fothe eye and is most frequently found congenitally in syndromal craniosynostosis, such as Crouzon, Apert, Saethre-Chotzen and other related syndromes. Exopthalmus can exist as a sole deformity. It is also seen in older patients as a so-called"malignant exopthalmus," which is a result of hyperthyroid disease caused by overproduction of TSH hormone.
Surgical correction of exopthalmus is accomplished through expansion of the supraorbital rim with frontocranial remodeling and Lefor III or monobloc advancment, which simultaneously allows reconstruction of the bony orbit. An additional procedure described by Dr. Kenneth Salyer incorporates removal of the abnormallly enlarged ethmoid sinus in affected children, thus allowing for translocation of the orbits into a more normal position medically and simultaneous correction of exopthalmus.
Hypertelorism
Hypertelorism, or wide-set eyes, is a physical finding that may occur alone or as a result of, or in association with, a number of congenital deformities. They include frontonasal dysplasia, craniosynostosis (such as is seen with Apert, Crouzon, and related syndromes), abnormalities or developmental problems related to nasal deformity; facial clefting; hydrocephalus; or Encephalocelee. Read More...