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Disease Profile

Bifid nose

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.


US Estimated

Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

Median fissure of nose; Nose, median cleft of


Congenital and Genetic Diseases; Ear, Nose, and Throat Diseases; Mouth Diseases


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]


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.

Medical Terms Other Names
Learn More:
80%-99% of people have these symptoms
Bifid nose
Indentation or clefting of the nose
5%-29% of people have these symptoms
Wide-set eyes
Widely spaced eyes

[ more ]

1%-4% of people have these symptoms
Renal agenesis
Absent kidney
Missing kidney

[ more ]

Percent of people who have these symptoms is not available through HPO
Abnormality of the skeletal system
Skeletal abnormalities
Skeletal anomalies

[ more ]

Anteriorly placed anus
Autosomal dominant inheritance
Autosomal recessive inheritance
Bulbous nose
Undescended testes
Undescended testis

[ more ]

Midline defect of the nose
Drooping upper eyelid
Rectovaginal fistula
Abnormal connection between rectum and vagina
Short lingual frenulum
Short philtrum


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

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    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.


      1. Miller PJ, Grinberg D, Wang TD. Midline cleft. Treatment of the bifid nose. Archives of Facial Plastic Surgery. July-Sept 1999; https://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; https://omim.org/210400. Accessed 7/1/2011.
      4. Marla J. F. O'Neill . FRONTONASAL DYSPLASIA 1; FND1. OMIM. June 17, 2010; https://omim.org/entry/136760. Accessed 7/1/2011.
      5. Victor A. McKusick . Bifid Nose, Autosomal Dominant. OMIM. 1986; https://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; https://omim.org/entry/608980?search=bifid%20nose&highlight=bifid%20noses%20nose%20bifids. Accessed 7/1/2011.

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