A Clinical Report of the Complete Nasal Agenesis: Reconstruction of Congenital Arhinia and Review of the Literature

Abstract

Congenital absence of the nose or arhinia is an exceptionally rare craniofacial malformation, and the pathophysiology of the arhinia is still unknown. Most arhinia patients can have difficulties with breathing and feeding due to the absence of the nose, nasal cavities, and associated problems. A 38-day-old female patient was referred to our clinic with arhinia. Physical examination revealed the complete agenesis of nasal structures as the nasal bones and vestibulum nasi. The region of the absent nose was flat and firm at palpation. Congenital arhinia may occur with other associated malformations such as ocular, ear, palate, and gonadal. Therefore, it is recommended to evaluate computed tomography/magnetic resonance imaging in the postnatal period. Additionally, a radiological evaluation will help nasal reconstruction by documenting changes in nasal and maxillary anatomy over time. Due to the limited number of arhinia cases presented, the surgical management of this condition has not been standardized. We presented the pyramid-shaped cartilage grafts for the nasal framework and an expanded paramedian forehead flap for the skin coverage for reconstruction of arhinia.

Two Forehead Flaps for Nasal Reconstruction of a Patient with Arhinia

“…Boynuyogun et al presented a 38-day-old patient with arhinia. Pyramid-shaped cartilage grafts were used for the nasal framework and the skin coverage was done with an expanded paramedian forehead flap 11 . Spiller et al presented a multi-staged reconstructive approach to arhinia with stereolithography in an 11 year-old-patient 12 .…”