[MIM 109 740, 210 400]

Rare congenital anomaly of the nose and cavum characterized by a nasal cleft that can vary from a barely visible groove in the columella to a complete cleft in the underlying bone and cartilage, dividing the nose into two halves. The upper airway is generally adequate. Hypertelorism and median cleft lip may be associated There is an autosomal dominant form, without hypertelorism [MIM 109 740], and an autosomal recessive form [MIM 210 400].

A bifid nose may also be observed in cases of 

-         frontonasal dysplasia (see this term)

-         holoprosencephaly (see this term)

-         Tessier cleft n°0 (see this term)

-         BNAR syndrome: bifid nose with or without rectal and renal anomalies (see this term)

-         cranio-fronto-nasal dysplasia [MIM 304 110], a very rare form of craniosynostosis due to a mutation of the EFNB1 gene (Xq13.1).

Anesthetic implications: 

check for any associated cerebral malformations; avoid nasal intubation

References : 

-        Wang X, Wang H, You J, Han R, Zheng R, Xu Y, Zhang X, Guo J, Fan  F.
A ten-year surgical experience in patients of Tessier No.0 cleft with a bifid nose.
Int J Pediatr Otorhinolaryngology 2023 ; 164 :  111399

Updated: March 2024