Bifid nose

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.{{Cite web |title=SNOMED CT - Bifid nose - Classes {{!}} NCBO BioPortal |url=https://bioportal.bioontology.org/ontologies/SNOMEDCT?p=classes&conceptid=787413007 |access-date=2022-08-25 |website=bioportal.bioontology.org}}{{Cite journal |last1=Miller |first1=Philip J. |last2=Grinberg |first2=Daniel |last3=Wang |first3=Tom D. |date=1999-07-01 |title=Midline Cleft |url=https://www.liebertpub.com/doi/10.1001/archfaci.1.3.200 |journal=Archives of Facial Plastic Surgery |volume=1 |issue=3 |pages=200–203 |doi=10.1001/archfaci.1.3.200 |pmid=10937104 |issn=1521-2491}} Individuals with this birth anomaly don't have any symptoms related to their bifid nose.

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.

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.{{cite web | url=https://rarediseases.org/gard-rare-disease/bifid-nose/ | title=Bifid nose }}{{Cite web |title=Bifid nose - About the Disease - Genetic and Rare Diseases Information Center |url=https://rarediseases.info.nih.gov/diseases/884/bifid-nose |access-date=2022-05-10 |website=rarediseases.info.nih.gov |language=en}}

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".{{Cite journal |last1=Padmanabhan |first1=Laxmi Devi |last2=Hamza |first2=V. Zareena |last3=Thampi |first3=Venugopalan Madhavan |last4=Govindankuttymenon |first4=Usha Madathiparambil |date=2014-12-01 |title=Double Barrel Sign: A Pointer for Prenatal Detection of Bifid Nose |journal=Journal of Fetal Medicine |language=en |volume=1 |issue=4 |pages=187–188 |doi=10.1007/s40556-015-0032-4 |s2cid=72797381 |issn=2348-8859|doi-access=free }}

This condition is caused due to incomplete fusion of both sides of the nose during embryonic life.{{cite web | url=https://www.mybiosource.com/learn/bifid-nose/ | title=Bifid nose }}{{Cite journal |last=Mühlbauer |first=Wolfgang D. |date=1976-09-01 |title=A surgical approach to the bifid nose |url=https://doi.org/10.1007/BF00274291 |journal=Chirurgia Plastica |language=en |volume=3 |issue=3 |pages=175–183 |doi=10.1007/BF00274291 |s2cid=37372814 |issn=1435-0130}}{{Cite journal |last1=WEAVER |first1=D. F. |last2=BELLINGER |first2=D. H. |title=Bifid Nose Associated with Midline Cleft of the Upper Lip |date=1946-10-01 |url=https://doi.org/10.1001/archotol.1946.00680060501012 |journal=Archives of Otolaryngology |volume=44 |issue=4 |pages=480–482 |doi=10.1001/archotol.1946.00680060501012 |pmid=21002094 |issn=0276-0673}}

This anomaly is highly hereditary: autosomal dominant and autosomal recessive inheritance patterns have been observed in multiple families.{{cite web | url=https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=2695 | title=Orphanet: Bifid nose }}

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{{Cite web |title=KEGG DISEASE: Bifid nose with or without anorectal and renal anomalies |url=https://www.genome.jp/dbget-bin/www_bget?H00685 |access-date=2022-08-25 |website=www.genome.jp}}{{Cite web |title=Entry - #608980 - BIFID NOSE WITH OR WITHOUT ANORECTAL AND RENAL ANOMALIES; BNAR - OMIM |url=https://omim.org/entry/608980 |access-date=2022-08-25 |website=omim.org |language=en-us}}) that is caused by autosomal recessive mutations in the FREM1 gene.{{Cite journal |last1=Alazami |first1=Anas M. |last2=Shaheen |first2=Ranad |last3=Alzahrani |first3=Fatema |last4=Snape |first4=Katie |last5=Saggar |first5=Anand |last6=Brinkmann |first6=Bernd |last7=Bavi |first7=Prashant |last8=Al-Gazali |first8=Lihadh I. |last9=Alkuraya |first9=Fowzan S. |date=2009-11-13 |title=FREM1 Mutations Cause Bifid Nose, Renal Agenesis, and Anorectal Malformations Syndrome |journal=The American Journal of Human Genetics |language=English |volume=85 |issue=5 |pages=414–418 |doi=10.1016/j.ajhg.2009.10.008 |pmid=19732862 |pmc=2771533 |issn=0002-9297}}

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).{{cite web | url=https://www.jagchana.co.uk/blog/bifid-nasal-tip-rhinoplasty | title=What is a bifid nasal tip ? }}{{Cite journal |last1=Tawfik |first1=Ali |last2=El-Sisi |first2=Hossam Elsayed |last3=Abd El-fattah |first3=Ahmed Musaad |date=2016-07-01 |title=Surgical correction of bifid nose |url=https://www.sciencedirect.com/science/article/pii/S0165587616300477 |journal=International Journal of Pediatric Otorhinolaryngology |language=en |volume=86 |pages=72–76 |doi=10.1016/j.ijporl.2016.04.004 |pmid=27260584 |issn=0165-5876}} Open W-shaped surgical incisions have also proven to be effective.{{Cite journal |last1=Wei |first1=Jiao |last2=Herrler |first2=Tanja |last3=Yu |first3=Baofu |last4=Chen |first4=Xiaoxue |last5=Dai |first5=Chuanchang |date=2022-06-23 |title=Correction of severe bifid nose deformity using an open W-shaped incision |url=https://www.sciencedirect.com/science/article/pii/S1748681522003886 |journal=Journal of Plastic, Reconstructive & Aesthetic Surgery |volume=75 |issue=9 |pages=3457–3461 |language=en |doi=10.1016/j.bjps.2022.06.060 |pmid=35941033 |s2cid=249998871 |issn=1748-6815}} Surgery is usually done before the age of 5.{{Cite web |title=Cleft Nasal Deformity |url=https://www.rchsd.org/programs-services/plasticsurgery/conditions-treated/cleft-lip-and-palate/cleft-nasal-deformity/ |access-date=2022-08-25 |website=www.rchsd.org}}

This birth anomaly affects less than 0.0008% of people worldwide, making it the most common midline craniofacial cleft.{{cite journal | doi=10.3389/fped.2021.768176 | doi-access=free | title=Morphological Analysis of Nose in Patients of Tessier No. 0 Cleft with a Bifid Nose in China | year=2021 | last1=Wang | first1=Xin | last2=Wang | first2=Huan | last3=You | first3=Jianjun | last4=Zheng | first4=Ruobing | last5=Xu | first5=Yihao | last6=Zhang | first6=Xulong | last7=Guo | first7=Junsheng | last8=Fan | first8=Fei | journal=Frontiers in Pediatrics | volume=9 | page=768176 | pmid=34912760 | pmc=8668193 }}

8% of people with bifid nose also have hypertelorism.{{Cite web |title=Symptoms: What are the signs and symptoms of autosomal recessive bifid nose? {{!}} ThinkGenetic |url=https://thinkgenetic.com/diseases/bifid-nose/symptoms |access-date=2022-08-25 |website=thinkgenetic.com}}

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

One case per inheritance pattern follows:

• Autosomal dominant: Anyane-Yeboa et al. (1984) describes 5 women from a 3-generation American family. Karyotypes and skull X-rays done on the younger sisters gave normal results.{{Cite journal |last1=Anyane-Yeboa |first1=K. |last2=Raifman |first2=M. A. |last3=Berant |first3=M. |last4=Frogel |first4=M. P. |last5=Travers |first5=H. |date=1984-03-01 |title=Dominant inheritance of bifid nose |url=https://pubmed.ncbi.nlm.nih.gov/6711607/ |journal=American Journal of Medical Genetics |volume=17 |issue=3 |pages=561–563 |doi=10.1002/ajmg.1320170303 |issn=0148-7299 |pmid=6711607}}
• Autosomal recessive: Boo-Chai et al. (1965) describes 3 siblings from a continental Indian family.{{Cite journal |last=Boo-Chai |first=K. |date=1965-12-01 |title=The bifid nose. With a report of 3 cases in siblings |url=https://pubmed.ncbi.nlm.nih.gov/5845694/ |journal=Plastic and Reconstructive Surgery |volume=36 |issue=6 |pages=626–628 |doi=10.1097/00006534-196512000-00007 |issn=0032-1052 |pmid=5845694}}

This condition was first described in medical literature in 1939{{cite web | url=https://alicia.concytec.gob.pe/vufind/Record/REVCMH_c57fed4f406553587103dbd0a12b9abc | title=Bifid Nose: Case presentation | year=2021 | last1=Lugo-Machado | first1=Juan Antonio }} by Esser et al. when they described 5 children (4 siblings and a male first-cousin of theirs) from a single family.{{Cite web |title=Entry - 210400 - BIFID NOSE, AUTOSOMAL RECESSIVE - OMIM |url=https://omim.org/entry/210400#2 |access-date=2022-08-25 |website=omim.org |language=en-us}}

This condition can also occur in animals, such as dogs.{{Cite journal |last1=Arzi |first1=B. |last2=Verstraete |first2=F. |date=2011 |title=Repair of a bifid nose combined with a cleft of the primary palate in a 1-year-old dog. |journal=Veterinary Surgery |volume=40 |issue=7 |pages=865–869 |doi=10.1111/j.1532-950X.2011.00880.x|pmid=22380669 |s2cid=3377802 }}

• Cleft palate
• Cleft lip
• Cleft chin

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Category:Genetic diseases and disorders