Nasal Bifidity: An Unusual Pediatric Congenital Anomaly and Review of the Literature

Abstract

Congenital nasal anomalies are rare, with an estimated incidence of 1/20,000 to 40,000 live births. Hyperplasia and duplication anomalies are the most uncommon, comprising about 1% of reported cases. The authors present the case of a 6-year-old girl who presented to our institution with an isolated congenital bifid nasal septum. Parents reported a visibly abnormal nose since birth, and it had been continuously monitored by the parents and pediatrician. She demonstrated no history of difficulty breathing or other nasal concerns and was otherwise growing and developing normally. On physical examination, she was breathing comfortably through her bilateral nasal airways. Her nasal examination revealed a widened mid-vault with deep dorsal grooving and a bifid tip. Magnetic resonance imaging demonstrated an isolated bifid nasal septum without other facial malformation or intracranial extension. She underwent an open septorhinoplasty. Intraoperatively, the authors identified an anomalous dorsal nasal bone extension with a resultant bifidity in the body and caudal portions of the septum. Ostectomy and cartilaginous repositioning allowed for an autogenous reconstruction without the need for grafting. She subsequently recovered well without postoperative complications and continues to have improved nasal appearance with maintenance of function. A review of recent literatures revealed 2 other cases that are similar in presentation. The authors proposed that embryologically there might have been a change in expression of bone morphogenetic protein in the frontonasal area leading to caudal extension of the nasal bone. This in turn interferes with the fusion of nasal septum resulting in the bifid septum and dual dome morphology.