What is the definition of Bifid Nose?

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. It is often associated with hypertelorbitism and midline clefts of the lip. The airway usually is adequate despite the cosmetic appearance associated with the condition. Both autosomal recessive and autosomal dominant inheritance of a bifid nose has been observed. It may also occur with frontonasal dysplasia (a condition in with several possible findings limited to the head and neck), for which several inheritance patterns have been reported. Treatment typically consists of surgical reconstruction to repair the malformation.

What are the alternative names for Bifid Nose?

  • Median fissure of nose
  • Nose, median cleft of

Is Bifid Nose an inherited disorder?

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.

Individuals interested in learning more about the genetics of a particular trait or condition, or their specific risk to have a child or other family member with a condition, should speak with a genetics professional.

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