Objectives: Congenital frontonasal dysplasia (CFND) is a rare heterogeneous collection of facial deformities. Due to the range of complexity, surgical management is not standardized.
Methods: We present a severe case of CFND and approach to managing multiple defects with a focus on rhinoplasty.
Results: This infant was born full term with a large mass instead of a nose, a bilateral cleft lip and palate, and hypertelorbitism. Our primary concerns initially were to address communication with the intracranial cavity, preserve a nasal lining, and improve nasal appearance and airway function in the short term without interfering with subsequent rhinoplasty and adult nasal appearance.
Conclusions: This complex case of CFND is more severe than anything we encountered in our literature review and demonstrates the necessity for multidisciplinary approach to multiple craniofacial defects. Future plans for this patient include rhinoplasty with auricular graft, scar revision, and addressing tip support.
Keywords: craniofacial; frontonasal dysplasia; pediatric rhinoplasty.