Ainuz Bar Y, Wolfe Erin M, Wolfe S Anthony
Plastic and Reconstructive Surgery, Florida International University, Herbert Wertheim College of Medicine, Miami, USA.
Plastic and Reconstructive Surgery, University of Miami Miller School of Medicine, Miami, USA.
Cureus. 2021 Jan 20;13(1):e12821. doi: 10.7759/cureus.12821.
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.
额鼻发育异常(FND)是一种罕见的先天性颅面裂综合征,伴有一系列中线面部骨骼和软组织畸形。当出现时,FND的身体特征通常很明显,在出生时即可分类。由此产生的面部畸形有可能对心理社会健康和生活质量产生负面影响。幼儿期的重建性手术干预有助于恢复面部轮廓并减轻心理压力。在本报告中,一名14岁女性,患有先前未被诊断出的轻度FND,前来进行重建手术评估,并接受了包括滑动前移颏成形术、鼻甲黏膜下切除术、开放式鼻成形术和双侧经鼻内眦固定术等多项手术。患者随后因鼻尖持续饱满进行了鼻尖重塑。患者获得了可接受的美学效果,并对自己的外貌感到满意。该病例强调了轻度FND病例在较晚年龄才被诊断出的细微表现和重建手术选择,这与该综合征更严重的表型以及其他通常在幼儿期被诊断和治疗的常见颅面畸形不同。多学科颅面护理团队应该能够正确诊断并对症状较轻的FND患者实施适当的手术干预。