Otofacial syndrome
Encyclopedia
Otofacial syndrome is an extremely rare congenital deformity whereby the afflicted is born without a jaw
and subsequently a chin
.
In nearly all cases, the child does not survive because of the inability to breathe and eat properly. Even with reconstructive surgery
, the tongue
is so underdeveloped making unaided breathing and swallowing impossible.
America
n surgeons successfully used bone from the hip of an Irish
teenager named Alan Doherty to rebuild a jaw and chin. Surgeons began the procedures in June 2007 and completed the final of seven surgeries on 25 August 2008. Doherty is now able to smile, but is still unable to breathe, eat, or speak on his own.
Jaw
The jaw is any opposable articulated structure at the entrance of the mouth, typically used for grasping and manipulating food. The term jaws is also broadly applied to the whole of the structures constituting the vault of the mouth and serving to open and close it and is part of the body plan of...
and subsequently a chin
Chin
In the human anatomy, the chin is the lowermost part of the face.It is formed by the lower front of the mandible.People show a wide variety of chin structures. See Cleft chin....
.
In nearly all cases, the child does not survive because of the inability to breathe and eat properly. Even with reconstructive surgery
Reconstructive surgery
Reconstructive surgery is, in its broadest sense, the use of surgery to restore the form and function of the body, although Maxillo-Facial Surgeons, Plastic Surgeons and Otolaryngologists do reconstructive surgery on faces after trauma and to reconstruct the head and neck after cancer.Other...
, the tongue
Tongue
The tongue is a muscular hydrostat on the floors of the mouths of most vertebrates which manipulates food for mastication. It is the primary organ of taste , as much of the upper surface of the tongue is covered in papillae and taste buds. It is sensitive and kept moist by saliva, and is richly...
is so underdeveloped making unaided breathing and swallowing impossible.
Treatment
The first challenge to survival is assisted breathing and tubal feeding. This is a lifelong affair, generally requiring the patient to spend nearly all of the time under direct hospital care.America
United States
The United States of America is a federal constitutional republic comprising fifty states and a federal district...
n surgeons successfully used bone from the hip of an Irish
Ireland
Ireland is an island to the northwest of continental Europe. It is the third-largest island in Europe and the twentieth-largest island on Earth...
teenager named Alan Doherty to rebuild a jaw and chin. Surgeons began the procedures in June 2007 and completed the final of seven surgeries on 25 August 2008. Doherty is now able to smile, but is still unable to breathe, eat, or speak on his own.