J Korean Soc Plast Reconstr Surg.  2000 May;27(3):319-322.

Surgical Management of Atypical Apert Syndrome Patient Using Combined Cranial and Maxillofacial Surgery

• Lee YH
• Shin DM

Abstract

Apert syndrome is well known condition with craniosynostosis, midface hypoplasia, exorbitism, and typical symmetric syndactyly of hands and feet. Surgical correction method should be selected according to the type and the severity of the deformity and the patient's age. We have experienced an adult atypical Apert syndrome patient who has brachycephaly and midface hypoplasia, without exorbitism, and has limb deformities such as brachymetatarsia and ectrodactyly without syndactyly of hands and feet, which are not typical in Apert syndrome patients. For the simultaneous correction of the cranial vault and the facial deformities, we combined Le Fort II advancement osteotomy and advancement genioplasty as well as transposition cranioplasty at the same time, and obtained successful result. Through our limited experience, we concluded our modified combined methods could be safely applied to the adult patients with various types of craniofacial deformity.

Keyword

Atypical; Apert syndrome; Cranioplasty; Le Fort II osteotomy

MeSH Terms

Acrocephalosyndactylia*
Adult
Congenital Abnormalities
Craniosynostoses
Extremities
Foot
Genioplasty
Hand
Humans
Osteotomy
Surgery, Oral*
Syndactyly