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

Lee, Yoon Ho; Shin, Dong Min

Original Article

Journal of the Korean Society of Plastic and Reconstructive Surgeons 2000;27(3):319-322.
Published online May 1, 2000.

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

Yoon Ho Lee, Dong Min Shin

Department of Plastic and Reconstructive Surgery, College of Medicine, Seoul National University.

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.

Keywords: Atypical; Apert syndrome; Cranioplasty; Le Fort II osteotomy