A bifid nose (also known as cleft nose) is an uncommon congenital malformation which is characterized by the presence of a cleft between the two nostrils of the nose.[1] It is the result of a disturbance during embryological nose development.[2]
It is part of the Tessier classification for craniofacial clefts.[3]
Signs and symptoms
The visibility of this malformation varies from person to person; from a barely noticeable "line" in the middle of the nose to the complete clefting of the nose which results in two "half noses", the airway is usually adequate.[4][5] Individuals with this birth anomaly don't have any symptoms related to their bifid nose.
Complications
This condition is merely cosmetic, and the severity of it doesn't affect a person with the disorder (health-wise) since usually there is a normal and adequate nasal airway.
Diagnosis
This condition can be diagnosed by physical examination.
Bifid noses can also be diagnosed prenatally via a coronal view of the face under ultrasonography, they typically have a broad appearance with "a cleavage between the nostrils resembling a double barrel gun".[8]
Causes
This condition is caused due to incomplete fusion of both sides of the nose during embryonic life.[9][10][11]
This anomaly is highly hereditary: autosomal dominant and autosomal recessive inheritance patterns have been observed in multiple families.[12]
If the bifid nose is accompanied by abnormalities of the anorectal and renal systems, it may be part of a different separate genetic disorder (which is characterized by overlapping toes, renal adysplasia and variable anorectal anomalies[13][14]) that is caused by autosomal recessive mutations in the FREM1 gene.[15]
Treatment
Generally, sutures and rhinoplasty can be done in order to get rid of a bifid nose tip (though what surgery should be done depends on the severity of the cleft nose).[16][17] Open W-shaped surgical incisions have also proven to be effective.[18] Surgery is usually done before the age of 5.[19]
Prevalence
This birth anomaly affects less than 0.0008% of people worldwide, making it the most common midline craniofacial cleft.[20]
8% of people with bifid nose also have hypertelorism.[21]
Bifid nose as a hereditary trait
Two forms of inheritance pattern for bifid noses have been described: autosomal recessive and autosomal dominant.
One case per inheritance pattern follows:
Autosomal dominant: Anyane-Yeboa et al. (1984) describes 5 women from a 3-generation American family. Karyotypes and skull X-rays done on the younger sisters gave normal results.[22]
Autosomal recessive: Boo-Chai et al. (1965) describes 3 siblings from a continental Indian family.[23]
History
This condition was first described in medical literature in 1939[24] by Esser et al. when they described 5 children (4 siblings and a male first-cousin of theirs) from a single family.[25]
Occurrence in animals
This condition can also occur in animals, such as dogs.[26]
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