Wikipedia

Bifid nose

(Redirected from Bifid nose dominant)

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]

Bifid nose
Other namesBifid nose tip, cleft nose, cleft nose tip, median fissure of nose, median cleft of nose.
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

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

Signs and symptoms

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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

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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

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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

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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

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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

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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

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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

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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

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

See also

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References

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  1. ^ "Bifid Nose". DoveMed. Retrieved 2022-08-25.
  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. S2CID 236398187.
  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. 7 (1): 104. doi:10.4103/0975-2870.122808. ISSN 0975-2870.
  4. ^ "SNOMED CT - Bifid nose - Classes | NCBO BioPortal". bioportal.bioontology.org. Retrieved 2022-08-25.
  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.
  6. ^ "Bifid nose".
  7. ^ "Bifid nose - About the Disease - Genetic and Rare Diseases Information Center". rarediseases.info.nih.gov. Retrieved 2022-05-10.
  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. 1 (4): 187–188. doi:10.1007/s40556-015-0032-4. ISSN 2348-8859. S2CID 72797381.
  9. ^ "Bifid nose".
  10. ^ Mühlbauer, Wolfgang D. (1976-09-01). "A surgical approach to the bifid nose". Chirurgia Plastica. 3 (3): 175–183. doi:10.1007/BF00274291. ISSN 1435-0130. S2CID 37372814.
  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.
  12. ^ "Orphanet: Bifid nose".
  13. ^ "KEGG DISEASE: Bifid nose with or without anorectal and renal anomalies". www.genome.jp. Retrieved 2022-08-25.
  14. ^ "Entry - #608980 - BIFID NOSE WITH OR WITHOUT ANORECTAL AND RENAL ANOMALIES; BNAR - OMIM". omim.org. Retrieved 2022-08-25.
  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. 85 (5): 414–418. doi:10.1016/j.ajhg.2009.10.008. ISSN 0002-9297. PMC 2771533. PMID 19732862.
  16. ^ "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. 86: 72–76. doi:10.1016/j.ijporl.2016.04.004. ISSN 0165-5876. PMID 27260584.
  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. doi:10.1016/j.bjps.2022.06.060. ISSN 1748-6815. PMID 35941033. S2CID 249998871.
  19. ^ "Cleft Nasal Deformity". www.rchsd.org. Retrieved 2022-08-25.
  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. PMC 8668193. PMID 34912760.
  21. ^ "Symptoms: What are the signs and symptoms of autosomal recessive bifid nose? | ThinkGenetic". thinkgenetic.com. Retrieved 2022-08-25.
  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.
  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.
  24. ^ Lugo-Machado, Juan Antonio (2021). "Bifid Nose: Case presentation".
  25. ^ "Entry - 210400 - BIFID NOSE, AUTOSOMAL RECESSIVE - OMIM". omim.org. Retrieved 2022-08-25.
  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. S2CID 3377802.
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