Treacher Collins syndrome: Management of a severe craniofacial deformity
Rajesh a 32 year old male had been
suffering from his birth from a severe craniofacial deformity called Treacher Collins Syndrome. This disorder involves severe deformities of the face and the
associated structures. The cheek bones are rudimentary, eyes are slanting
downward, ears are malformed, chin is very small and retruded. The overall
appearance of a patient suffering from this disorder is abnormal. Apart from the
cosmetic concerns,the individual is also short of hearing and may suffer from
some vision disturbances.
The aim of the surgery was:
- a. to augment his cheek bones,
- b. to augment lateral orbital wall and floor of orbit
- c. to correct his slanting of the eyes
For this to be done, bone would be
harvested from the skull and the hip bone. The surgery started with giving an incision
on his scalp which would be concealed well within his hairline. The face was
dissected uptil his cheek bones. Bone graft was taken from both sides of his
skull and his hip bone and shaped in the form of a pyramidal cheek bone.It was
then inserted into the area where this augmentation was needed. The soft
tissues of the eye were dissected and lateral canthopexy was done. It was a
lengthy operation but the recovery was smooth and uneventful. His facial contour
has almost normalised.
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