Bifid nose explained

Bifid nose
Synonyms:Bifid nose tip, cleft nose, cleft nose tip, median fissure of nose, median cleft of nose.
Symptoms:Having a cleft between the two nostrils of the nose.
Complications:Usually, there aren't any.
Onset:Conception
Duration:Life-long (unless cosmetic surgery is done)
Risks:Having parents or close relatives with the disorder
Diagnosis:Physical evaluation
Prevention:none
Treatment:usually, none is necessary
Prognosis:Good
Frequency:Uncommon
Deaths:none

A bifid nose (also known as cleft nose) is an uncommon congenital malformation which is characterized by the presence of a cleft between the two nostrils of the nose.[1] It is the result of a disturbance during embryological nose development.[2]

It is part of the Tessier classification for craniofacial clefts.[3]

Signs and symptoms

The visibility of this malformation varies from person to person; from a barely noticeable "line" in the middle of the nose to the complete clefting of the nose which results in two "half noses", the airway is usually adequate.[4] [5] Individuals with this birth anomaly don't have any symptoms related to their bifid nose.

Complications

This condition is merely cosmetic, and the severity of it doesn't affect a person with the disorder (health-wise) since usually there is a normal and adequate nasal airway.

Diagnosis

This condition can be diagnosed by physical examination.

This difference can serve as a diagnostic method since it occurs alongside other malformations, such as frontonasal dysplasia, hypertelorbitism and cleft lips.[6] [7]

Bifid noses can also be diagnosed prenatally via a coronal view of the face under ultrasonography, they typically have a broad appearance with "a cleavage between the nostrils resembling a double barrel gun".[8]

Causes

This condition is caused due to incomplete fusion of both sides of the nose during embryonic life.[9] [10] [11]

This anomaly is highly hereditary: autosomal dominant and autosomal recessive inheritance patterns have been observed in multiple families.[12]

If the bifid nose is accompanied by abnormalities of the anorectal and renal systems, it may be part of a different separate genetic disorder (which is characterized by overlapping toes, renal adysplasia and variable anorectal anomalies[13] [14]) that is caused by autosomal recessive mutations in the FREM1 gene.[15]

Treatment

Generally, sutures and rhinoplasty can be done in order to get rid of a bifid nose tip (though what surgery should be done depends on the severity of the cleft nose).[16] [17] Open W-shaped surgical incisions have also proven to be effective.[18] Surgery is usually done before the age of 5.[19]

Prevalence

This birth anomaly affects less than 0.0008% of people worldwide, making it the most common midline craniofacial cleft.[20]

8% of people with bifid nose also have hypertelorism.[21]

Bifid nose as a hereditary trait

Two forms of inheritance pattern for bifid noses have been described: autosomal recessive and autosomal dominant.

One case per inheritance pattern follows:

History

This condition was first described in medical literature in 1939[24] by Esser et al. when they described 5 children (4 siblings and a male first-cousin of theirs) from a single family.[25]

Occurrence in animals

This condition can also occur in animals, such as dogs.[26]

See also

Notes and References

  1. Web site: Bifid Nose . 2022-08-25 . DoveMed . en.
  2. Lugo-Machado . Juan Antonio . Quintero-Bauman . Alejandra . Barragán-Márquez . Fernanda . Vargas-Cárdenas . Luis Gerardo . Sepúlveda-Martínez . Mario . Jiménez-Rodríguez . Martha . Yocupicio-Hernández . Dalia . Gutiérrez-Pérez . Martha Lucia . 2021-01-01 . Nariz bífida: Presentación de caso Translated title: Bifid Nose: Case presentation . Revista del Cuerpo Médico Hospital Nacional Almanzor Aguinaga Asenjo . 10.35434/rcmhnaaa.2021.141.885. 236398187 . free .
  3. Chauhan . Dinesh Singh . Guruprasad . Yadavalli . 2014-01-01 . A rare occurrence of Tessier's 0 cleft in siblings . Medical Journal of Dr. D.Y. Patil University . en . 7 . 1 . 104 . 10.4103/0975-2870.122808 . 0975-2870. free .
  4. Web site: SNOMED CT - Bifid nose - Classes NCBO BioPortal . 2022-08-25 . bioportal.bioontology.org.
  5. Miller . Philip J. . Grinberg . Daniel . Wang . Tom D. . 1999-07-01 . Midline Cleft . Archives of Facial Plastic Surgery . 1 . 3 . 200–203 . 10.1001/archfaci.1.3.200 . 10937104 . 1521-2491.
  6. Web site: Bifid nose .
  7. Web site: Bifid nose - About the Disease - Genetic and Rare Diseases Information Center . 2022-05-10 . rarediseases.info.nih.gov . en.
  8. Padmanabhan . Laxmi Devi . Hamza . V. Zareena . Thampi . Venugopalan Madhavan . Govindankuttymenon . Usha Madathiparambil . 2014-12-01 . Double Barrel Sign: A Pointer for Prenatal Detection of Bifid Nose . Journal of Fetal Medicine . en . 1 . 4 . 187–188 . 10.1007/s40556-015-0032-4 . 72797381 . 2348-8859. free .
  9. Web site: Bifid nose .
  10. Mühlbauer . Wolfgang D. . 1976-09-01 . A surgical approach to the bifid nose . Chirurgia Plastica . en . 3 . 3 . 175–183 . 10.1007/BF00274291 . 37372814 . 1435-0130.
  11. WEAVER . D. F. . BELLINGER . D. H. . Bifid Nose Associated with Midline Cleft of the Upper Lip . 1946-10-01 . Archives of Otolaryngology . 44 . 4 . 480–482 . 10.1001/archotol.1946.00680060501012 . 21002094 . 0276-0673.
  12. Web site: Orphanet: Bifid nose .
  13. Web site: KEGG DISEASE: Bifid nose with or without anorectal and renal anomalies . 2022-08-25 . www.genome.jp.
  14. Web site: Entry - #608980 - BIFID NOSE WITH OR WITHOUT ANORECTAL AND RENAL ANOMALIES; BNAR - OMIM . 2022-08-25 . omim.org . en-us.
  15. Alazami . Anas M. . Shaheen . Ranad . Alzahrani . Fatema . Snape . Katie . Saggar . Anand . Brinkmann . Bernd . Bavi . Prashant . Al-Gazali . Lihadh I. . Alkuraya . Fowzan S. . 2009-11-13 . FREM1 Mutations Cause Bifid Nose, Renal Agenesis, and Anorectal Malformations Syndrome . The American Journal of Human Genetics . English . 85 . 5 . 414–418 . 10.1016/j.ajhg.2009.10.008 . 19732862 . 2771533 . 0002-9297.
  16. Web site: What is a bifid nasal tip ? .
  17. Tawfik . Ali . El-Sisi . Hossam Elsayed . Abd El-fattah . Ahmed Musaad . 2016-07-01 . Surgical correction of bifid nose . International Journal of Pediatric Otorhinolaryngology . en . 86 . 72–76 . 10.1016/j.ijporl.2016.04.004 . 27260584 . 0165-5876.
  18. Wei . Jiao . Herrler . Tanja . Yu . Baofu . Chen . Xiaoxue . Dai . Chuanchang . 2022-06-23 . Correction of severe bifid nose deformity using an open W-shaped incision . Journal of Plastic, Reconstructive & Aesthetic Surgery . 75 . 9 . 3457–3461 . en . 10.1016/j.bjps.2022.06.060 . 35941033 . 249998871 . 1748-6815.
  19. Web site: Cleft Nasal Deformity . 2022-08-25 . www.rchsd.org.
  20. 10.3389/fped.2021.768176 . free . Morphological Analysis of Nose in Patients of Tessier No. 0 Cleft with a Bifid Nose in China . 2021 . Wang . Xin . Wang . Huan . You . Jianjun . Zheng . Ruobing . Xu . Yihao . Zhang . Xulong . Guo . Junsheng . Fan . Fei . Frontiers in Pediatrics . 9 . 768176 . 34912760 . 8668193 .
  21. Web site: Symptoms: What are the signs and symptoms of autosomal recessive bifid nose? ThinkGenetic . 2022-08-25 . thinkgenetic.com.
  22. Anyane-Yeboa . K. . Raifman . M. A. . Berant . M. . Frogel . M. P. . Travers . H. . 1984-03-01 . Dominant inheritance of bifid nose . American Journal of Medical Genetics . 17 . 3 . 561–563 . 10.1002/ajmg.1320170303 . 0148-7299 . 6711607.
  23. Boo-Chai . K. . 1965-12-01 . The bifid nose. With a report of 3 cases in siblings . Plastic and Reconstructive Surgery . 36 . 6 . 626–628 . 10.1097/00006534-196512000-00007 . 0032-1052 . 5845694.
  24. Web site: Bifid Nose: Case presentation . 2021 . Lugo-Machado . Juan Antonio .
  25. Web site: Entry - 210400 - BIFID NOSE, AUTOSOMAL RECESSIVE - OMIM . 2022-08-25 . omim.org . en-us.
  26. Arzi . B. . Verstraete . F. . 2011 . Repair of a bifid nose combined with a cleft of the primary palate in a 1-year-old dog. . Veterinary Surgery . 40 . 7 . 865–869 . 10.1111/j.1532-950X.2011.00880.x. 22380669 . 3377802 .

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