Hypertelorism
Description
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 Aperts, Crouzons, and related syndromes), abnormalities or developmental problems related to nasal deformity; facial clefting; hydrocephalus; or Encephalocele.
Hypertelorism is diagnosed-usually at birth-when the interorbital distance is greater than normal, with "normal" defined as less than 20 mm at birth. With growth of the mid-face, this disproportion usually becomes more pronounced. In adults, a distance greater than 25 to 30 mm is considered abnormal.
Treatment
Although it can be corrected at a later time, we prefer to correct this deformity between the ages of three and five years after there has been a certain amount of development of the mid-facial and orbital bones.
In young children, the usual procedure is an inverted U-avoiding the tooth-bearing area. In older children, we may use either total translocation of the four walls of the orbit or a bipartition procedure, depending on the type and involvement of the deformity.
The primary correction of hypertelorism involves surgical reconstruction of the nasal deformity that is universally associated with this deformity. In patients with Encephalocelee, there may be a long-nose syndrome which needs to be corrected. In frontonasal dysplasia and facial clefting, there is a short nose which needs elongation with soft-tissue procedures including VY techniques and forehead flaps.
Due to expansion that tends to recur in the soft tissue and in the medial canthal region as the patient grows and develops, these procedures usually must be repeated-more than three times on average-before definitive correction of this deformity is achieved.
Results can be anywhere from good to excellent, depending upon the artistry of the surgeon performing the reconstruction of the nose..