Frontal cranioplasty in fronto-metaphyseal dysplasia.

INTRODUCTION

Fronto-metaphyseal dysplasia (FMD), also called Gorlin-Cohen syndrome, is a rare syndrome initially described in 1969 by Gorlin and Cohen. Patients present skeletal dysplasia, craniofacial malformations and digit abnormalities. Craniofacial phenotype of FMD is characterized by supraorbital hyperostosis, hypertelorism, down-slanting palpebral fissures, broad nasal bridge and micrognathia. Here, we report the first adult case of craniofacial reconstruction with frontal cranioplasty in a patient with FMD.

OBSERVATION: A 21-year-old male patient presented with aesthetic requests related to his facial abnormalities. The patient underwent a fronto-orbital cranioplasty using a coronal approach. Orbital, frontal and nasal hyperostoses were contoured in order to obtain a symmetric result. The patient had no postoperative complication. Aesthetic results were satisfactory and stable after 6 months of follow-up.

DISCUSSION: The density and the quality of craniofacial bones were normal and this may account for the stability of cranioplasty results over time. Because bone was normal, cranioplasty is safety and stable in FMD.