Frontal cranioplasty in fronto-metaphyseal dysplasia

Joly, Aline; Paré, Arnaud; Goga, D.; Laure, B.

doi:10.1016/j.jormas.2017.06.001

Cited by 3 publications

(1 citation statement)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…They described satisfactory surgical results with aesthetic and functional improvement without complications. Joly et al 15 in FMD, they found that the density and the quality of craniofacial bones were normal contrary to fibrous dysplasia 16 similarly as our histologic findings. The surgical impression is to be faced with phenotypic abnormalities related to a disorder of the periosteal envelope rather than a bone anomaly in itself.…”

Section: Discussionsupporting

confidence: 88%

Surgical Management of Craniomaxillofacial Features in the Otopalatodigital Spectrum Disorders

Roland-Billecart¹,

Schlund

Lauwers

et al. 2021

Journal of Craniofacial Surgery

View full text Add to dashboard Cite

Otopalatodigital syndrome spectrum disorders are caused by Filamin A (FLNA) gene mutations. Otopalatodigital syndrome spectrum disorders are a group of rare congenital skeletal dysplasia, with specific craniomaxillofacial features including otopalatodigital syndrome type 1 and type 2, Melnick-Needles syndrome, frontometaphyseal dysplasia, terminal osseous dysplasia with pigmentary defects. The authors describe cases of a young girl with Melnick-Needles syndrome and a young boy with frontometaphyseal dysplasia treated in the Oral and Maxillofacial Surgery Department. Both patients had FLNA gene mutation confirmed with molecular genetic analysis. The authors proposed a 4 step treatment of the malformations with good outcomes both aesthetically and functionally, without complication.

show abstract

Section: Discussionsupporting

confidence: 88%

Surgical Management of Craniomaxillofacial Features in the Otopalatodigital Spectrum Disorders

Roland-Billecart¹,

Schlund

Lauwers

et al. 2021

Journal of Craniofacial Surgery

View full text Add to dashboard Cite

show abstract

Multi-directional Cranial Distraction Osteogenesis for Treating Sagittal Synostosis with Frontometaphyseal Dysplasia: A Case Report

Morishita

Sunaga

Gomi

et al. 2021

Plastic and Reconstructive Surgery - Global Open

View full text Add to dashboard Cite

Summary: Frontometaphyseal dysplasia (FMD), also known as Gorlin-Cohen syndrome, is a rare genetic syndrome. This syndrome affects the skeletal system and connective tissue, and causes a wide spectrum of manifestations of the skull, tubular bones, cardiovascular system, urinary system, and/or gastrointestinal system. Craniofacial findings of FMD are characterized by protruding supraorbital ridge, broad nasal bridge, hypertelorism, down-slanting palpebral fissures, and/or micrognathia. We describe a case of a 2-year-old girl diagnosed with sagittal synostosis accompanied with FMD. She presents anterior sagittal synostosis cranial form, compressed cerebrospinal fluid space (which suggested increased intracranial pressure), and the supraorbital hyperostosis. She underwent multi-directional cranial distraction osteogenesis in the calvaria and shaving of the supraorbital ridges. Despite concerns about bone fragility associated with FMD, the surgery was accomplished as usual. The patient had no intra- and postoperative complications. After 6 months of follow-up, the cranial shape has improved and the cerebrospinal fluid space has widened, but the supraorbital ridge has protruded again. Re-protrusion of the supraorbital ridge appears to be due to age-appropriate vigorous osteogenesis. The multi-directional cranial distraction osteogenesis procedure has been useful for treating sagittal synostosis even concomitant with FMD.

show abstract

Combined Fronto-orbital Contouring and Orthognathic Surgery in Craniometaphyseal Dysplasia

Prado,

Cavalieri-Pereira

2024

Plastic and Reconstructive Surgery - Global Open

View full text Add to dashboard Cite

Summary: Craniometaphyseal dysplasia is a rare congenital sclerosing skeletal dysplasia that presents with facial dimorphism and is clinically described by prominent supraorbital bridges, severe retrognathia, and respiratory problems. Fronto-orbital cranioplasty is necessary to achieve satisfactory aesthetic outcomes. The supraorbital arches were exposed to the nasal bone through coronal access, with the help of a saw and a wear drill, and osteotomy of the frontal and orbital regions with hyperostosis was performed. Our case report describes a rare and difficult to diagnose case with a few publications of a 24-year-old female patient, who presented with metaphyseal dysplasia associated with mandibular retrognathia. As a treatment, fronto-orbital cranioplasty was performed in association with orthognathic surgery. The resolution of the case resulted in excellent functional and aesthetic results, indicating that frontal cranioplasty associated with orthognathic surgery is a plausible treatment for facial dimorphism through craniometaphyseal dysplasia.

show abstract

Frontal cranioplasty in fronto-metaphyseal dysplasia

Cited by 3 publications

References 10 publications

Surgical Management of Craniomaxillofacial Features in the Otopalatodigital Spectrum Disorders

Surgical Management of Craniomaxillofacial Features in the Otopalatodigital Spectrum Disorders

Multi-directional Cranial Distraction Osteogenesis for Treating Sagittal Synostosis with Frontometaphyseal Dysplasia: A Case Report

Combined Fronto-orbital Contouring and Orthognathic Surgery in Craniometaphyseal Dysplasia

Contact Info

Product

Resources

About