Medicine:Bifid nose

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Bifid nose
Other namesBifid nose tip, cleft nose, cleft nose tip, median fissure of nose, median cleft of nose.
Individuals who have Snijders Blok-Campeau syndrome.jpg
Individuals with Snijders Blok-Campeau syndrome, one of which (Individual 8) has a noticeable bifid nose tip.
SpecialtyMedical genetics, plastic surgery
SymptomsHaving a cleft between the two nostrils of the nose.
ComplicationsUsually, there aren't any.
Usual onsetConception
DurationLife-long (unless cosmetic surgery is done)
CausesGenetics
Risk factorsHaving parents or close relatives with the disorder
Diagnostic methodPhysical evaluation
Preventionnone
Treatmentusually, none is necessary
PrognosisGood
FrequencyUncommon
Deathsnone

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:

  • 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.[22]
  • Autosomal recessive: Boo-Chai et al. (1965) describes 3 siblings from a continental Indian family.[23]

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

  • Cleft palate
  • Cleft lip
  • Cleft chin

References

  1. ↑ "Bifid Nose" (in en). http://www.dovemed.com/diseases-conditions/bifid-nose/. 
  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. doi:10.35434/rcmhnaaa.2021.141.885. https://www.scienceopen.com/document?vid=784d67ac-ab41-4186-af8a-bc3ee2ffe9a4. 
  3. ↑ Chauhan, Dinesh Singh; Guruprasad, Yadavalli (2014-01-01). "A rare occurrence of Tessier's 0 cleft in siblings" (in en). Medical Journal of Dr. D.Y. Patil University 7 (1): 104. doi:10.4103/0975-2870.122808. ISSN 0975-2870. https://www.mjdrdypu.org/article.asp?issn=0975-2870;year=2014;volume=7;issue=1;spage=104;epage=105;aulast=Chauhan;type=0. 
  4. ↑ "SNOMED CT - Bifid nose - Classes | NCBO BioPortal". https://bioportal.bioontology.org/ontologies/SNOMEDCT?p=classes&conceptid=787413007. 
  5. ↑ Miller, Philip J.; Grinberg, Daniel; Wang, Tom D. (1999-07-01). "Midline Cleft". Archives of Facial Plastic Surgery 1 (3): 200–203. doi:10.1001/archfaci.1.3.200. ISSN 1521-2491. PMID 10937104. https://www.liebertpub.com/doi/10.1001/archfaci.1.3.200. 
  6. ↑ "Bifid nose". https://rarediseases.org/gard-rare-disease/bifid-nose/. 
  7. ↑ "Bifid nose - About the Disease - Genetic and Rare Diseases Information Center" (in en). https://rarediseases.info.nih.gov/diseases/884/bifid-nose. 
  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" (in en). Journal of Fetal Medicine 1 (4): 187–188. doi:10.1007/s40556-015-0032-4. ISSN 2348-8859. 
  9. ↑ "Bifid nose". https://www.mybiosource.com/learn/bifid-nose/. 
  10. ↑ Mühlbauer, Wolfgang D. (1976-09-01). "A surgical approach to the bifid nose" (in en). Chirurgia Plastica 3 (3): 175–183. doi:10.1007/BF00274291. ISSN 1435-0130. https://doi.org/10.1007/BF00274291. 
  11. ↑ WEAVER, D. F.; BELLINGER, D. H. (1946-10-01). "Bifid Nose Associated with Midline Cleft of the Upper Lip". Archives of Otolaryngology 44 (4): 480–482. doi:10.1001/archotol.1946.00680060501012. ISSN 0276-0673. PMID 21002094. https://doi.org/10.1001/archotol.1946.00680060501012. 
  12. ↑ "Orphanet: Bifid nose". https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=2695. 
  13. ↑ "KEGG DISEASE: Bifid nose with or without anorectal and renal anomalies". https://www.genome.jp/dbget-bin/www_bget?H00685. 
  14. ↑ "Entry - #608980 - BIFID NOSE WITH OR WITHOUT ANORECTAL AND RENAL ANOMALIES; BNAR - OMIM" (in en-us). https://omim.org/entry/608980. 
  15. ↑ Alazami, Anas M.; Shaheen, Ranad; Alzahrani, Fatema; Snape, Katie; Saggar, Anand; Brinkmann, Bernd; Bavi, Prashant; Al-Gazali, Lihadh I. et al. (2009-11-13). "FREM1 Mutations Cause Bifid Nose, Renal Agenesis, and Anorectal Malformations Syndrome" (in English). The American Journal of Human Genetics 85 (5): 414–418. doi:10.1016/j.ajhg.2009.10.008. ISSN 0002-9297. PMID 19732862. 
  16. ↑ "What is a bifid nasal tip ?". https://www.jagchana.co.uk/blog/bifid-nasal-tip-rhinoplasty. 
  17. ↑ Tawfik, Ali; El-Sisi, Hossam Elsayed; Abd El-fattah, Ahmed Musaad (2016-07-01). "Surgical correction of bifid nose" (in en). International Journal of Pediatric Otorhinolaryngology 86: 72–76. doi:10.1016/j.ijporl.2016.04.004. ISSN 0165-5876. PMID 27260584. https://www.sciencedirect.com/science/article/pii/S0165587616300477. 
  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" (in en). Journal of Plastic, Reconstructive & Aesthetic Surgery 75 (9): 3457–3461. doi:10.1016/j.bjps.2022.06.060. ISSN 1748-6815. PMID 35941033. https://www.sciencedirect.com/science/article/pii/S1748681522003886. 
  19. ↑ "Cleft Nasal Deformity". https://www.rchsd.org/programs-services/plasticsurgery/conditions-treated/cleft-lip-and-palate/cleft-nasal-deformity/. 
  20. ↑ Wang, Xin; Wang, Huan; You, Jianjun; Zheng, Ruobing; Xu, Yihao; Zhang, Xulong; Guo, Junsheng; Fan, Fei (2021). "Morphological Analysis of Nose in Patients of Tessier No. 0 Cleft with a Bifid Nose in China". Frontiers in Pediatrics 9: 768176. doi:10.3389/fped.2021.768176. PMID 34912760. 
  21. ↑ "Symptoms: What are the signs and symptoms of autosomal recessive bifid nose? | ThinkGenetic". https://thinkgenetic.com/diseases/bifid-nose/symptoms. 
  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. doi:10.1002/ajmg.1320170303. ISSN 0148-7299. PMID 6711607. https://pubmed.ncbi.nlm.nih.gov/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. doi:10.1097/00006534-196512000-00007. ISSN 0032-1052. PMID 5845694. https://pubmed.ncbi.nlm.nih.gov/5845694/. 
  24. ↑ Lugo-Machado, Juan Antonio (2021). "Bifid Nose: Case presentation". https://alicia.concytec.gob.pe/vufind/Record/REVCMH_c57fed4f406553587103dbd0a12b9abc. 
  25. ↑ "Entry - 210400 - BIFID NOSE, AUTOSOMAL RECESSIVE - OMIM" (in en-us). https://omim.org/entry/210400#2. 
  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. doi:10.1111/j.1532-950X.2011.00880.x. PMID 22380669. 



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