The Value of the Maxillo-Malar Osteotomy in the Treatment of Crouzon Syndrome With Exorbitism

Abstract

Premature fusion of the cranial sutures along with midface hypoplasia, shallow orbits, and ocular proptosis are the principal features of Crouzon syndrome. Treatment varies according to the variable expressivity of the disease and usually begins during a child's first year with fronto-orbital advancement with cranial decompression. Subsequent development of midfacial hypoplasia needs correction. Procedures for this purpose include the Le Fort III osteotomy or its segmental variants, monobloc frontofacial advancement, or bipartition osteotomy. Adult Crouzon often presents with marked midface hypoplasia and exorbitism. We report an adult-diagnosed Crouzon syndrome case with typical facial features and exorbitism corrected by orbital decompression and zygomaticomaxillary advancement.

Crouzon Syndrome: Clinico-Radiological Illustration of a Case

“…[8] Surgical treatment varies according to the variable expressivity of the disease and usually begins during a child's first year with fronto-orbital advancement with cranial decompression. [10] Subsequent development of midfacial hypoplasia needs correction. Procedures for this purpose include the Le Fort III osteotomy or its segmental variants, monobloc frontofacial advancement, or bipartition osteotomy.…”

“…Adult CS, as in our case presenting with marked midface hypoplasia and exorbitism, can be corrected by orbital decompression and zygomaticomaxillary advancement. [910]…”