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.

Rajesh had lived like this through his life until he and his family came to know of Richardsons dental and craniofacialhospital where such cases are treated regularly. He came to the OPD two days back and was explained about the entire treatment by world renowned craniofacial surgeon Prof. Phillipe Pellerin and Dr.Sunil Richardson. Being such an extensive disorder his deformities would be addressed in staged procedures. A CT scan was done for the patient and in a computer linked software,the surgeons did a virtual planning of the defect to be corrected in the surgery. His blood work up was also done. Surgery was planned for the following day.

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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