First and second branchial arch syndrome: aspects on the embryogenesis, elucidations, and rehabilitation using the osseointegration concept.
BACKGROUND
The osseointegration concept has dramatically changed the possibility of rehabilitating patients with craniofacial defects due to branchial arch syndromes.


OBJECTIVE
This article describes some problems related to the investigative routines and rehabilitation of individuals with malformations of the first and second branchial arches of the craniofacial region. Animal model systems have increased the knowledge of basic embryonic processes that can explain the extent of the malformations. Though most clinical first and second branchial arch syndromes are likely to be caused by sporadic mutations, inherited syndromes occur and also teratogenically induced syndromes are known. Prenatal diagnosis ruling out heredity and exogenous influence seems possible in the future. The possibility of preventing and alleviating fulminant syndromes prenatally also could be conceivable in the future.


METHODS
The rehabilitation process starts early after birth and should involve a team of specialists including clinical geneticists, pediatricians, audiologists, plastic surgeons, maxillofacial surgeons, otosurgeons, anaplastologists, speech pathologists, pedodontists, and orthodontists. With the development of the osseointegration concept in which craniofacial prostheses and hearing aids can be adapted on implants anchored in the craniofacial skeleton, a new field in the rehabilitation of these malformations has opened.


RESULTS
Important aspects in the use of the osseointegration concept include determination of the lowest age for implant surgery, accessibility of adequate bone for implants, the growth of the craniofacial skeleton during childhood, and the possibility for the patient and his or her parents to care for the skin penetration. Adverse tissue reactions, durability of craniofacial prostheses, and the possibility of unknown adverse reactions to metal implants in the body over a long time are other aspects of concern.


CONCLUSIONS
Patients with branchial arch syndromes benefit from a well-planned multidisciplinary rehabilitation process in which osseointegrated bone-anchored hearing aids and bone-anchored ear prostheses can be useful tools.
Granström
G
G
Department of Otolaryngology, Head and Neck Surgery, and Department of Pedodontics, University of Göteburg, Gothenburg, Sweden. gosta.granstrom@orlss.gu.se
Jacobsson
C
C
D000293:Adolescent; D000818:Animals; D001934:Branchial Region / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D004195:Disease Models, Animal; D006310:Hearing Aids; D006801:Humans; D007223:Infant; D008342:Mandibulofacial Dysostosis / Q000175:diagnosis / Q000196:embryology / Q000534:rehabilitation*; D019931:Maxillofacial Prosthesis Implantation / Q000295:instrumentation*; D016348:Osseointegration; D019736:Prostheses and Implants; D051381:Rats; D017207:Rats, Sprague-Dawley; D013577:Syndrome