National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Bifid nose


Other Names:
Median fissure of nose; Nose, median cleft of
Categories:
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.[1][2] It is often associated with hypertelorbitism and midline clefts of the lip.[1] The airway usually is adequate despite the cosmetic appearance associated with the condition.[2] Both autosomal recessive and autosomal dominant inheritance of a bifid nose has been observed.[3] 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.[4] Treatment typically consists of surgical reconstruction to repair the malformation.[1]
Last updated: 7/1/2011

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
5%-29% of people have these symptoms
Hypertelorism
Wide-set eyes
Widely spaced eyes
[ more ] 		0000316
Percent of people who have these symptoms is not available through HPO
Abnormality of the kidney
Abnormal kidney
0000077
Abnormality of the skeletal system
Skeletal abnormalities
Skeletal anomalies
[ more ] 		0000924
Anteriorly placed anus 0001545
Autosomal dominant inheritance 0000006
Autosomal recessive inheritance 0000007
Bifid nose
Indentation or clefting of the nose
0011803
Bulbous nose 0000414
Cryptorchidism
Undescended testes
Undescended testis
[ more ] 		0000028
Midline defect of the nose 0004122
Ptosis
Drooping upper eyelid
0000508
Rectovaginal fistula
Abnormal connection between rectum and vagina
0000143
Short philtrum 0000322
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Last updated: 7/1/2020
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.[3][5] 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).[4] 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.[6]

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.
Last updated: 7/1/2011

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
    Bifid nose, autosomal recessive
    Bifid nose, autosomal dominant
    Bifid nose with or without anorectal and renal anomalies
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Bifid nose. Click on the link to view a sample search on this topic.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

  • I am 31 years old and my husband and I are considering starting a family. I was born with a bifid nose which was repaired as a child. I am wondering if you can explain the role of genetics and the bifid nose. See answer


  1. Miller PJ, Grinberg D, Wang TD. Midline cleft. Treatment of the bifid nose. Archives of Facial Plastic Surgery. July-Sept 1999; http://www.ncbi.nlm.nih.gov/pubmed/10937104. Accessed 7/1/2011.
  2. Paul W. Flint et al. Cummings Otolaryngology: Head & Neck Surgery. 5th ed. USA: Mosby Elsevier; 2010;
  3. Victor A. McKusick. Bifid Nose, Autosomal Recessive. OMIM. 1986; http://omim.org/210400. Accessed 7/1/2011.
  4. Marla J. F. O'Neill . FRONTONASAL DYSPLASIA 1; FND1. OMIM. June 17, 2010; http://omim.org/entry/136760. Accessed 7/1/2011.
  5. Victor A. McKusick . Bifid Nose, Autosomal Dominant. OMIM. 1986; http://omim.org/entry/109740?search=bifid%20nose&highlight=bifid%20noses%20nose%20bifids. Accessed 7/1/2011.
  6. Marla J. F. O'Neill . BIFID NOSE WITH OR WITHOUT ANORECTAL AND RENAL ANOMALIES; BNAR. OMIM. October 7, 2009; http://omim.org/entry/608980?search=bifid%20nose&highlight=bifid%20noses%20nose%20bifids. Accessed 7/1/2011.