Bifid nose

Other Names: Median fissure of nose; Nose, median cleft of

A bifid nose is a relatively uncommon malformation that is characterized by the nose being divided into two parts. There is a large degree of variability in the severity of the condition, ranging from a minimally noticeable groove down the center of the nasal tip to a complete clefting of the underlying bones and cartilage, resulting in 2 complete half noses.

Genetics

The role of genetics in being born with a bifid nose is not completely understood. There have been reports in the literature consistent with several different patterns of inheritance for a bifid nose. Inheritance patterns consistent with autosomal recessive inheritance and autosomal dominant inheritance have been reported both for individuals with only a bifid nose as well as for individuals with a bifid nose and additional abnormalities.

Ocular hypertelorism (widely spaced eyes) is occasionally associated with bifid nose but the genetics of the combination has been unclear. For frontonasal dysplasia, a condition that includes several potential abnormalities limited to the head and neck (including a bifid nose), both autosomal recessive and X-linked dominant inheritance has been observed, as well as sporadic cases (occurring in individuals with no history of the condition in the family).

For another condition called bifid nose with or without anorectal and renal anomalies, autosomal recessive inheritance has been suggested, and there has been evidence that mutations in the FREM1 gene cause this particular condition.

Symptoms

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Bifid nose(Indentation or clefting of the nose)

5%-29% of people have these symptoms

• Hypertelorism(Wide-set eyes)

1%-4% of people have these symptoms

• Renal agenesis(Absent kidney)

Treatment

Treatment typically consists of surgical reconstruction to repair the malformation.

NIH genetic and rare disease info

• NIH info on Bifid nose

Bifid nose is a rare disease.

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  Jason Bateman at Deauville 2011