Management of a case of arhinia.

Abstract

Arhinia is a rare condition characterised by the congenital absence of nasal structures, with different patterns of presentation, and often associated with other craniofacial or somatic anomalies. To date, about 30 surviving cases have been reported. We report the case of a female patient aged 6 years, who underwent internal and external nose reconstruction using a staged procedure: a nasal airway was obtained through maxillary osteotomy and ostectomy, and lined with a local skin flap and split-thickness skin grafts; then the external nose was reconstructed with an expanded frontal flap, armed with an autogenous rib framework.