Complete congenital arhinia is a rare defect of embryogenesis leading to the absence of the external nose and airway. We report our novel multistaged reconstructive approach and literature review. Nasal methyl methacrylate prosthesis was created from a stereolithographic model for use as a temporary prosthesis and tissue expander. Lefort 1 with cannulization was utilized for midface advancement and airway formation. External framework was reconstructed with bilateral conchal bowl cartilage and rib osteocartilagenous grafts. Patient was pleased with the aesthetics and had safe decannulation with the ability to breathe through the nose and airway.
AkkuzuGAkkuzuBAydinEDerbentMOzluogluL. Congenital partial arhinia: a case report. J Med Case Rep. 2007;1:97.
2.
AlbernazVSCastilloMMukherjiSKIhmeidanIH. Congenital arhinia. AJNR Am J Neuroradiol. 1996;17(7):1312–1314.
3.
AliMJ. Bilateral lacrimal mucoceles in a setting of congenital arhinia. Ophthalmic Plast Reconstr Surg. 2014;30(6):e167.
4.
BergerMMartinC. Total arhinogenesis (congenital absence of the nose and the nasal fossae) apropos of an unusual case [in French]. Rev Laryngol Otol Rhinol (Bord). 1969;90(5):300–319.
5.
BorghiARuggieroFTenhagenMSchievanoSPonniahADunawayDO’HaraJOngJBrittoJA. Design and manufacturing of a patient-specific nasal implant for congenital arhinia: case report. JPRAS Open. 2019;21:28–34.
6.
BrusatiRDonatiVMarelliSFerrariM. Management of a case of arhinia. J Plast Reconstr Aesthet Surg. 2009;62(7):e206–e210.
7.
CesarettiCGentilinBBianchiVMelloniGBonaguroMRossiCMeazziniCBrusatiRLalattaF.Occurrence of complete arhinia in two siblings with a clinical picture of Treacher Collins syndrome negative for TCOF1, POLR1D and POLR1C mutations. Clin Dysmorphol. 2011;20(4):229–231.
CusickWSullivanCARojasBPooleAEPooleDA. Prenatal diagnosis of total arhinia. Ultrasound Obstet Gynecol. 2000;15(3):259–261.
12.
Das GuptaHKGuptaVGuptaM. Absent nose. Br J Plast Surg. 1979;32(2):85–86.
13.
DekabanA. Arhinencephaly in an infant born to a diabetic mother. J Neuropathol Exp Neurol. 1959;18:620–626.
14.
FakhraeeSHNarimanSTaghipourR.Congenital arhinia: case report of a rare congenital anomaly. Arch Iran Med. 2011;14(5):355–356.
15.
FeledyJAGoodmanCMTaylorTStalSSmithBHollierL. Vertical facial distraction in the treatment of arhinia. Plast Reconstr Surg. 2004;113(7):2061–2066.
16.
FernandesNvan den HeeverJSykesLKlugeH. Nasal reconstruction of a patient with complete congenital arhinia: a clinical report. J Prosthet Dent. 2016;116(6):924–927.
17.
FullerAKMcCraryHCGrahamMESkirkoJR. The case of the missing nose: congenital arhinia case presentation and management recommendations. Ann Otol Rhinol Laryngol. 2020:129(7):645–648.
18.
GiffordGHJSwansonLMacCollumDW. Congenital absence of the nose and anterior nasopharynx. Report of two cases. Plast Reconstr Surg. 1972;50(1):5–12.
19.
GoyalAAgrawalVRainaVKSharmaD. Congenital arhinia: a rare case. J Indian Assoc Pediatr Surg. 2008;13(4):153–154.
20.
HourJ-W. Congenital arhinia with de novo reciprocal translocation, t(3;12) (q13.2;p11.2). Am J Med Genet A. 2004;130A(2):200–203.
21.
JungJWHaD-HKimBYSeoBFHanHHKimDHRhieJWKimSWChoDW. Nasal reconstruction using a customized three-dimensional-printed stent for congenital arhinia: three-year follow-up. Laryngoscope. 2019;129(3):582–585.
22.
KaminkerCPDaínLLamasMASánchezJMOpitzJMReynoldsJF. Mosaic trisomy 9 syndrome with unusual phenotype. Am J Med Genet. 1985;22(2):237–241.
23.
KembleJV. The importance of the nasal septum in facial development. J Laryngol Otol. 1973;87(4):379–386.
24.
LaTrentaGSChoiHWWardRFHoffmanLNeidichJA. Complete nasal agenesis with bilateral microphthalmia and unilateral duplication of the thumb. Plast Reconstr Surg. 1995;95(6):1101–1104.
25.
LiXZhangLWangF. Prenatal diagnosis of total arhinia by MRI. Jpn J Radiol. 2015;33(10):672–674.
26.
LütolfU.Bilateral aplasia of the nose: a case report. J Maxillofac Surg. 1976;4(4):245–249.
27.
MathurNNDubeyNKKumarSBothraRChadhaA. Arhinia. Int J Pediatr Otorhinolaryngol. 2005;69(1):97–99.
Méndez-GallartRGarrido-ValenzuelaMBautista-CasasnovasAEstevez-MartínezE. Congenital partial arhinia with no associated malformations. Int J Pediatr Otorhinolaryngol Extra. 2009;4(4):162–164.
30.
MeyerR. Total external and internal construction in arhinia. Plast Reconstr Surg. 1997;99(2):534–542.
31.
MondalUPrasadR.Congenital arhinia: a rare case report and review of literature. Indian J Otolaryngol Head Neck Surg. 2016;68(4):537–539.
32.
MühlbauerWSchmidtAFairleyJ. Simultaneous construction of an internal and external nose in an infant with arhinia. Plast Reconstr Surg. 1993;91(4):720–725.
33.
Navarro-VilaCMatiasGCMartinezGCVerdaguerMJAceroSJPerezSVRodriguezA. Congenital absence of the nose and nasal fossae. J Craniomaxillofacial Surg. 1991;19(2):56–60.
34.
NgRLRajapathyKIshakZ. Congenital arhinia—first published case in Malaysia. Med J Malaysia. 2017;72(5):308–310.
35.
OlsenØEGjellandKReigstadHRosendahlK. Congenital absence of the nose: a case report and literature review. Pediatr Radiol. 2001;31(4):225–232.
36.
PalmerCRThomsonHG. Congenital absence of the nose: a case report. Can J Surg. 1967;10(1):83–86.
37.
RuprechtKWMajewskiF. Familiary arhinia combined with peters’ anomaly and maxilliar deformities, a new malformation syndrome (author’s transl) [in German]. Klin Monbl Augenheilkd. 1978;172(5):708–715.
38.
SatoDShimokawaOHaradaNOlsenOEHouJWMuhlbauerWBlinkenbergEOkamotoNKinoshitaAMatsumotoN, et al.Congenital arhinia: molecular-genetic analysis of five patients. Am J Med Genet A. 2007;143A(6):546–552.
39.
ShinoMChikamatsuKYasuokaYNagaiKFuruyaN.Congenital arhinia: a case report and functional evaluation. Laryngoscope. 2005;115(6):1118–1123.
40.
ShubichISanchezC. Nasal aplasia associated with meningocele and submucous cleft palate. Ear Nose Throat J. 1985;64(5):259–260.
41.
TakcıSKorkmazASimşek-KiperPOUtineGEBoduroğluKYurdakökM. Congenital partial arhinia: a rare malformation of the nose coexisting with holoprosencephaly. Turk J Pediatr. 2012;54(4):440–443.
42.
TessierPCiminelloFSWolfeSA. The arrhinias. Scand J Plast Reconstr Surg Hand Surg. 2009;43(4):177–196.
43.
ThieleHMusilANagelFMajewskiF. Familial arhinia, choanal atresia, and microphthalmia. Am J Med Genet. 1996;63(1):310–313.
44.
TryggestadJBLiSChernausekSD. Hypogonadotropic hypogonadism presenting with arhinia: a case report. J Med Case Rep. 2013;7:52.
45.
WeinbergANeumanABenmeirPLusthausSWexlerMR. A rare case of arhinia with severe airway obstruction: case report and review of the literature. Plast Reconstr Surg. 1993;91(1):146–149.
46.
ZhangMMHuYHHeWHuKK. Congenital arhinia: a rare case. Am J Case Rep. 2014;15:115–118.
