Surgical Management of a Mild Case of Frontonasal Dysplasia: A Case Report and Review of Literature.

Bar Y Ainuz,Erin M Wolfe,S Anthony Wolfe

doi:10.7759/cureus.12821

Abstract

Frontonasal dysplasia (FND) is a rare congenital craniofacial cleft syndrome associated with a spectrum of midline facial bone and soft-tissue malformations. When present, the physical features of FND are often obvious and classified at birth. The resultant facial deformities have the potential to negatively influence psychosocial health and quality of life. Reconstructive surgical intervention in early childhood can serve to restore facial contour and alleviate psychological stress. In this report, a case of a 14-year-old female with previously undiagnosed mild form of FND presented for reconstructive surgery evaluation and underwent several procedures including sliding advanced genioplasty, submucosal resection of the nasal turbinates, open rhinoplasty, and bilateral transnasal medial canthopexies. The patient had subsequent nasal tip recontouring for persistent supratip fullness. The patient achieved an acceptable esthetic outcome and was satisfied with her physical appearance. This case emphasizes the subtle presentation and reconstructive surgical options of a mild case of FND that was diagnosed at a later age, unlike the more severe phenotypes of the syndrome and other common craniofacial anomalies that are usually diagnosed and treated in early childhood. Multidisciplinary craniofacial care teams should be able to correctly diagnose and implement the appropriate surgical interventions in patients with milder forms of FND.

Highlights

Frontonasal dysplasia (FND) is a rare congenital orofacial cleft syndrome of early embryogenic development resulting in a broad phenotypic presentation of midline head and face malformations [1,2]
As the classification of craniofacial clefts changed over the years, FND adopted several names including Tessier cleft no. 0, frontonasal dysostosis, median cleft face syndrome, median facial dysplasia, and most recently median craniofacial hyperplasia [8,9]
Features of FND may be seen as part of broader syndromes associated with a constellation of anomalies not seen in isolated FND such as frontofacionasal and craniofrontonasal syndromes, which include further ocular and musculoskeletal anomalies, respectively [4,11]

Summary

Introduction

Frontonasal dysplasia (FND) is a rare congenital orofacial cleft syndrome of early embryogenic development resulting in a broad phenotypic presentation of midline head and face malformations [1,2]. Patients with FND characteristically have at least two of the following features: hypertelorism; midline nasal cleft or facial groove; broad nasal bridge and/or nasal tip deformity; central hair pattern such as a widow’s peak; and median cleft lip and palate [3]. Literature describing the reconstructive surgical management of FND is limited and encompasses various multistaged bony and soft-tissue interventions tailored to the craniofacial features expressed in each individual [3,7,12] These include invasive facial bipartition surgery for correction of hypertelorism, specialized rhinoplasty techniques for correction of the nasal deformity, cleft lip and palate repair, laser. On physical examination the patient had characteristic features classified by the senior author as FND including a low forehead with a central hair pattern resembling a widow’s peak, mild telecanthus, substantial retrogenia, and profound nasal deformity.

Discussion

Conclusions

Disclosures

DeMyer W

Tessier P