What are the other Names for this Condition? (Also known as/Synonyms)

• Median Cleft of Nose
• Median Fissure of Nose

What is Bifid Nose? (Definition/Background Information)

• 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

(Source: Bifid Nose; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Bifid Nose? (Age and Sex Distribution)

• Bifid Nose is a rare congenital disorder. The presentation of symptoms may occur at birth
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Bifid Nose? (Predisposing Factors)

• A positive family history may be an important risk factor, since Bifid Nose can be inherited
• Presence of frontonasal dysplasia

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Bifid Nose? (Etiology)

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.

(Source: Bifid Nose; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

What are the Signs and Symptoms of Bifid Nose?

The signs and symptoms of Bifid Nose may include:

• Abnormality of the kidney
• Abnormality of the skeletal system
• Anteriorly placed anus
• Bifid Nose
• Bulbous nose
• Cryptorchidism
• Ptosis
• Rectovaginal fistula
• Short philtrum

Occasionally present symptoms in 5-29% of the cases: Hypertelorism

(Source: Bifid Nose; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Bifid Nose Diagnosed?

Bifid Nose is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Bifid Nose?

The complications of Bifid Nose may include:

• Facial deformity
• Involvement of other organs

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Bifid Nose Treated?

• Treatment of Bifid Nose typically consists of surgical reconstruction to repair the malformation

(Source: Bifid Nose; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How can Bifid Nose be Prevented?

Currently, Bifid Nose may not be preventable, since it is a genetic disorder.

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

What is the Prognosis of Bifid Nose? (Outcomes/Resolutions)

• The prognosis of Bifid Nose is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Bifid Nose:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/