Hypertelorism
Description of 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 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.
Preoperative frontal view of 1-year-old with hypertelorism and deformed skull. Postoperative frontal view 3 years after surgical corrections using the specialized techniques developed by Dr. Salyer. Intraoperative views showing surgical correction.
Hypertelorism from frontonasal dysplasia. Preoperative view 21-year-old patient with wide set eyes. Postoperative view 5 years after correction of hypertelorism and nasal reconstruction. Preoperative lateral view shows short nose and profile before corrective surgery. Postoperative lateral view after lengthening of the nose and improved profile.
Preoperative frontal view of a 12 year old patient with hypertelorism and nasal deformity. Postoperative frontal view 2 years after hypertelorism correction with nasal reconstruction. Pre and postoperative lateral views.
Preoperative frontal view of a 15-year-old patient with a midfacial cleft and hypertelorism, a bifid nose, and a deformed maxilla. Postoperative frontal view 3 years after surgical correction. Preoperative submental vertex view shows lack of nasal projection of the bifid nose. Postoperative submental vertex of the same patient after reconstruction of the nose and lip.
Expectations and Treatment for Hypertelorism
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.
Orbital Reconstruction
Hypertelorism involves having wide-set eyes alone or as part of a congenital syndrome that includes other facial abnormalities besides hypertelorism. While hypertelorism is often diagnosed at birth, it can be diagnosed as the baby grows. A growing baby will often show worsening of the hypertelorism to the point where the child needs orbital reconstruction to correct the problem.
A decision will be made as to when to perform the orbital reconstruction as well as what degree of hypertelorism is tolerable for the child. Your doctor will explain to you how the hypertelorism worsens with age as the child’s midface grows, separating the eyes over time. Hypertelorism isn’t dangerous; however, it is often part of a congenitally unacceptable facial appearance and can affect binocular vision. Orbital reconstruction is the only really acceptable treatment for the condition of hypertelorism.
Orbital reconstruction is often done when the child has grown to the age of three to five years. In deciding to undergo orbital reconstruction, the degree of hypertelorism, the overall health and the vision of the child must be taken into account. If it seems that orbital reconstruction is necessary, your doctor will show you how it is done and explain what can be achieved after the orbital reconstruction is over. Your child will have X-rays taken and will have a preoperative examination. If possible, the orbital reconstruction procedure will be explained to the child in as simple terms as possible.
The day of the orbital construction can feel like a whirlwind experience. Your child may be having more facial work done than just for the hypertelorism. During surgery, the facial bones, including the nasal bones are reshaped and some bone is removed so that the eyes are closer together. Any related abnormalities can be corrected at the same time.
A child born with hypertelorism often undergoes more than one orbital reconstruction over time. Even when the hypertelorism is fixed the first time, the problem often persists as the child’s face grows so that orbital reconstruction is needed again. When the child has completely grown, the hypertelorism is generally completely corrected and no further orbital reconstruction is necessary.
