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Bifid nose, autosomal dominant

MedGen UID:
414016
•Concept ID:
C2751431
•
Disease or Syndrome
Synonym: Bifid Nose, Autosomal Dominant
 
Monarch Initiative: MONDO:0007195
OMIM®: 109740

Professional guidelines

PubMed

Clinical features and orbital anomalies in Fraser syndrome and a review of management options.
Das D, Modaboyina S, Raj S, Agrawal S, Bajaj MS
Indian J Ophthalmol 2022 Jul;70(7):2559-2563. doi: 10.4103/ijo.IJO_2627_21. PMID: 35791156Free PMC Article

Recent clinical studies

Etiology

Reconstruction of the shortened columella in mild bifid nose using a propeller flap based on the nasal columella artery.
Wei J, Herrler T, Yu B, Chen X, Wang Z, Dong L, Chen A, Chen Q, Li Q, Dai C
J Plast Reconstr Aesthet Surg 2023 Jul;82:152-158. Epub 2023 Feb 10 doi: 10.1016/j.bjps.2023.02.011. PMID: 37167716
Clinical features and orbital anomalies in Fraser syndrome and a review of management options.
Das D, Modaboyina S, Raj S, Agrawal S, Bajaj MS
Indian J Ophthalmol 2022 Jul;70(7):2559-2563. doi: 10.4103/ijo.IJO_2627_21. PMID: 35791156Free PMC Article
Component columella augmentation in cleft nose rhinoplasty: a preliminary study.
Bohluli B, Varedi P, Sezavar M, Pakzad S, Bagheri SC
Int J Oral Maxillofac Surg 2017 May;46(5):548-553. Epub 2017 Jan 13 doi: 10.1016/j.ijom.2016.11.019. PMID: 28094096
Midline Cleft Lip and Bifid Nose Deformity: Description, Classification, and Treatment.
Kolker AR, Sailon AM, Meara JG, Holmes AD
J Craniofac Surg 2015 Nov;26(8):2304-8. doi: 10.1097/SCS.0000000000002229. PMID: 26594965
Manitoba-oculo-tricho-anal (MOTA) syndrome is caused by mutations in FREM1.
Slavotinek AM, Baranzini SE, Schanze D, Labelle-Dumais C, Short KM, Chao R, Yahyavi M, Bijlsma EK, Chu C, Musone S, Wheatley A, Kwok PY, Marles S, Fryns JP, Maga AM, Hassan MG, Gould DB, Madireddy L, Li C, Cox TC, Smyth I, Chudley AE, Zenker M
J Med Genet 2011 Jun;48(6):375-82. Epub 2011 Apr 20 doi: 10.1136/jmg.2011.089631. PMID: 21507892Free PMC Article

Diagnosis

Two novel mutations within FREM1 gene in patients with bifid nose.
Chen X, Yu B, Wang Z, Li Q, Dai C, Wei J
BMC Pediatr 2023 Dec 14;23(1):631. doi: 10.1186/s12887-023-04453-9. PMID: 38097983Free PMC Article
Clinical features and orbital anomalies in Fraser syndrome and a review of management options.
Das D, Modaboyina S, Raj S, Agrawal S, Bajaj MS
Indian J Ophthalmol 2022 Jul;70(7):2559-2563. doi: 10.4103/ijo.IJO_2627_21. PMID: 35791156Free PMC Article
Heterozygous intragenic deletions of FREM1 are not associated with trigonocephaly.
Dawson AJ, Hovanes K, Liu J, Marles S, Greenberg C, Mhanni A, Chudley A, Frosk P, Sahoo T, Schanze D, Zenker M
Clin Dysmorphol 2021 Apr 1;30(2):83-88. doi: 10.1097/MCD.0000000000000351. PMID: 33038106
Nasal fistula, epidermal cyst and hypernatremia in a girl presenting holoprosencephaly due to a rare ZIC2 point mutation.
de Almeida IG Jr, Kuratani DK, Gomes LM, Fiegenbaum M, Estima Correia EP, Gazzola Zen PR, Machado Rosa RF
Eur J Med Genet 2020 Feb;63(2):103641. Epub 2019 Mar 17 doi: 10.1016/j.ejmg.2019.03.005. PMID: 30894326
Diagnostic imaging features of congenital nose and nasal cavity lesions.
Ginat DT, Robson CD
Clin Neuroradiol 2015 Mar;25(1):3-11. Epub 2014 Aug 6 doi: 10.1007/s00062-014-0323-5. PMID: 25095909

Therapy

Surgical Correction of a Bifid Nose Deformity with a Split M-Shaped Flap.
Wang Y, Yu B, Dai C, Wei J
Facial Plast Surg Aesthet Med 2023 May-Jun;25(3):238-243. Epub 2022 Jul 19 doi: 10.1089/fpsam.2022.0077. PMID: 35856821
Surgical correction of bifid nose.
Tawfik A, El-Sisi HE, Abd El-Fattah AM
Int J Pediatr Otorhinolaryngol 2016 Jul;86:72-6. Epub 2016 Apr 20 doi: 10.1016/j.ijporl.2016.04.004. PMID: 27260584
Midline cleft. Treatment of the bifid nose.
Miller PJ, Grinberg D, Wang TD
Arch Facial Plast Surg 1999 Jul-Sep;1(3):200-3. doi: 10.1001/archfaci.1.3.200. PMID: 10937104

Prognosis

Novel FREM1 mutations expand the phenotypic spectrum associated with Manitoba-oculo-tricho-anal (MOTA) syndrome and bifid nose renal agenesis anorectal malformations (BNAR) syndrome.
Nathanson J, Swarr DT, Singer A, Liu M, Chinn A, Jones W, Hurst J, Khalek N, Zackai E, Slavotinek A
Am J Med Genet A 2013 Mar;161A(3):473-8. Epub 2013 Feb 8 doi: 10.1002/ajmg.a.35736. PMID: 23401257Free PMC Article
Correction of unilateral cleft lip nose deformity using nasal alar rim flap.
Wang H, Fan F, You J, Wang S
J Craniofac Surg 2012 Sep;23(5):1378-81. doi: 10.1097/SCS.0b013e318252fd09. PMID: 22948647
Inferior encephalocele: transpalatal repair using paired costal bone grafts with a 14-year follow-up.
Hassani E, Karimi H, Hassani A
J Pediatr Surg 2011 Oct;46(10):E9-13. doi: 10.1016/j.jpedsurg.2011.06.026. PMID: 22008362
Three-dimensional computed tomography of congenital nasal anomalies.
Schlosser RJ, Faust RA, Phillips CD, Gross CW
Int J Pediatr Otorhinolaryngol 2002 Sep 2;65(2):125-31. doi: 10.1016/s0165-5876(02)00147-7. PMID: 12176182
Midline cleft. Treatment of the bifid nose.
Miller PJ, Grinberg D, Wang TD
Arch Facial Plast Surg 1999 Jul-Sep;1(3):200-3. doi: 10.1001/archfaci.1.3.200. PMID: 10937104

Clinical prediction guides

Nasal fistula, epidermal cyst and hypernatremia in a girl presenting holoprosencephaly due to a rare ZIC2 point mutation.
de Almeida IG Jr, Kuratani DK, Gomes LM, Fiegenbaum M, Estima Correia EP, Gazzola Zen PR, Machado Rosa RF
Eur J Med Genet 2020 Feb;63(2):103641. Epub 2019 Mar 17 doi: 10.1016/j.ejmg.2019.03.005. PMID: 30894326
Rare Median Cleft-Lip Repair With Novel Surgical Procedures Using Bilateral Cleft Margin Flaps.
Kang S, Imai K, Masuoka T, Sakahara D, Kawamoto K, Tsumoto J
J Craniofac Surg 2019 Jan;30(1):19-22. doi: 10.1097/SCS.0000000000004891. PMID: 30418287
Recapitulation of Unilateral Cleft Lip Nasal Deformity on Normal Nasal Structure: A Finite Element Model Analysis.
Huang H, Luo X, Cheng X, Zhang Z, Ma G, Shi B, Li J
J Craniofac Surg 2018 Nov;29(8):2220-2225. doi: 10.1097/SCS.0000000000005024. PMID: 30339602
Potocki-Shaffer deletion encompassing ALX4 in a patient with frontonasal dysplasia phenotype.
Ferrarini A, Gaillard M, Guerry F, Ramelli G, Heidi F, Keddache CV, Wieland I, Beckmann JS, Jaquemont S, Martinet D
Am J Med Genet A 2014 Feb;164A(2):346-52. Epub 2013 Dec 13 doi: 10.1002/ajmg.a.36140. PMID: 24376213
Alar web in cleft lip nose deformity: study in adult unilateral clefts.
Agarwal R, Chandra R
J Craniofac Surg 2012 Sep;23(5):1349-54. doi: 10.1097/SCS.0b013e31826467e5. PMID: 22948620

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