Frontal cranioplasty in fronto-metaphyseal dysplasia

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 hyperosto...

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Published inJournal of stomatology, oral and maxillofacial surgery Vol. 118; no. 5; pp. 310 - 312
Main Authors Joly, A., Pare, A., Goga, D., Laure, B.
Format Journal Article
LanguageEnglish
Published France Elsevier Masson SAS 01.10.2017
Subjects
Craniofacial abnormalities
Craniofacial Abnormalities - diagnosis
Craniofacial Abnormalities - surgery
Craniofacial surgery
Facial Bones - surgery
Forehead - abnormalities
Forehead - surgery
Fronto-metaphyseal dysplasia
Humans
Hypertelorism - diagnosis
Hypertelorism - surgery
Male
Osteochondrodysplasias - diagnosis
Osteochondrodysplasias - surgery
Rare disease
Reconstructive Surgical Procedures - methods
Skull - abnormalities
Skull - surgery
Syndrome
Young Adult
Fronto-metaphyseal dysplasia
Craniofacial abnormalities
Rare disease
Craniofacial surgery
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Summary: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. 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. 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.
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ISSN:2468-7855
2468-7855
DOI:10.1016/j.jormas.2017.06.001