Congenital Malformations of the Nose and Paranasal Sinuses

  • Ceren Günel
  • Sema Başak
  • Elina Toskala


Congenital anomalies occur because of errors during embryogenesis. For this reason, in order to understand the malformations of the nose and sinuses, normal development of these structures should be known well. Depending on the affected period during development, various malformations, ranging from the absence of full development of sinonasal structures to a malformation with mild severity, may be observed. At the third to fourth weeks of fetal life, frontal process and one pair of maxillary and mandibular processes begin to appear. Maxillary and mandibular processes are separated from each other by stomodeum, which is the primitive mouth. Nasal placodes of ectodermal origin migrate caudally and form the lateral and medial nasal processes. At the following weeks, lateral and medial nasal processes unite to form the nasal cavity. Nasal cavity is separated from the oral cavity by bucconasal membrane. The fusion of medial nasal processes (MNP) is the precursor of philtrum, columella, upper lip, nasal bone, and cartilage. At the sixth week of intrauterine life, by the fusion of maxillary and medial nasal processes, palatal structure begins to develop. The frontal nasal process is the precursor of septum and is created in the ninth week by the persistence of neural crest cells. At the tenth week, palatal structures unite with an inferior septum. At 11th and 12th weeks, the developments of uvula and soft palate are completed. Bucconasal membrane which separates posterior nasal cavity from the oral cavity regresses in the fifth to sixth months of intrauterine life. With canalization of the nasal cavity, the epithelial plugs filling the cavity are resorbed within the following weeks. In this chapter, congenital malformations of the nose and paranasal sinuses are presented.


Congenital malformations Nose Paranasal sinuses Nasal cavity Fetal life Malformations 


  1. 1.
    Johnston MC, Bronsky PT. Prenatal craniofacial development: new insights on normal and abnormal mechanisms. Crit Rev Oral Biol Med. 1995;6:25–79.CrossRefGoogle Scholar
  2. 2.
    Albernaz VS, Castillo M, Mukherji SK, et al. Congenital arhinia. AJNR Am J Neuroradiol. 1996;17:1312–4.PubMedGoogle Scholar
  3. 3.
    McGlone L. Congenital arhinia. J Paediatr Child Health. 2003;39:474–6.CrossRefGoogle Scholar
  4. 4.
    Williams A, Pizzuto M, Brodsky L, et al. Supernumerary nostril: a rare congenital deformity. Int J Pediatr Otorhinolaryngol. 1998;44:161–7.CrossRefGoogle Scholar
  5. 5.
    Franco D, Medeiros J, Faveret P, et al. Supernumerary nostril: case report and review of the literature. J Plast Reconstr Aesthet Surg. 2008;61:442–6.CrossRefGoogle Scholar
  6. 6.
    Matsumura T, Hayashi A, Komuro Y. The supernumerary nostril. J Craniofac Surg. 2010;21:808–10.CrossRefGoogle Scholar
  7. 7.
    Gargano F, Szymanski K, Bosman M, et al. Tessier 1-13 atypical craniofacial cleft. Eplasty. 2015;15:ic32.PubMedPubMedCentralGoogle Scholar
  8. 8.
    Tessier P. Anatomical classification facial, cranio-facial and latero-facial clefts. J Maxillofac Surg. 1976;4:69–92.CrossRefGoogle Scholar
  9. 9.
    Martin S, Hogan E, Sorenson EP, et al. Proboscis lateralis. Childs Nerv Syst. 2013;29:885–91.CrossRefGoogle Scholar
  10. 10.
    Sakamoto Y, Miyamoto J, Nakajima H, et al. New classification scheme of proboscis lateralis based on a review of 50 cases. Cleft Palate Craniofac J. 2012;49:201–7.CrossRefGoogle Scholar
  11. 11.
    Brown OE, Pownell P, Manning SC. Choanal atresia: a new anatomic classification and clinical management applications. Laryngoscope. 1996;106:97–101.CrossRefGoogle Scholar
  12. 12.
    Samadi DS, Shah UK, Handler SD. Choanal atresia: a twenty-year review of medical comorbidities and surgical outcomes. Laryngoscope. 2003;113:254–8.CrossRefGoogle Scholar
  13. 13.
    Leclerc JE, Fearon B. Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol. 1987;13:265–72.CrossRefGoogle Scholar
  14. 14.
    Hengerer AS, Brickman TM, Jeyakumar A. Choanal atresia: embryologic analysis and evolution of treatment, a 30-year experience. Laryngoscope. 2008;118:862–6.CrossRefGoogle Scholar
  15. 15.
    Newman JR, Harmon P, Shirley WP, et al. Operative management of choanal atresia: a 15-year experience. JAMA Otolaryngol Head Neck Surg. 2013;139:71–5.CrossRefGoogle Scholar
  16. 16.
    Slovis TL, Renfro B, Watts FB, et al. Choanal atresia: precise CT evaluation. Radiology. 1985;155:345–8.CrossRefGoogle Scholar
  17. 17.
    Coniglio JU, Manzione JV, Hengerer AS. Anatomic findings and management of choanal atresia and the CHARGE association. Ann Otol Rhinol Laryngol. 1988;97:448–53.CrossRefGoogle Scholar
  18. 18.
    Brown OE, Myer CM 3rd, Manning SC. Congenital nasal pyriform aperture stenosis. Laryngoscope. 1989;99:86–91.CrossRefGoogle Scholar
  19. 19.
    Shikowitz MJ. Congenital nasal pyriform aperture stenosis: diagnosis and treatment. Int J Pediatr Otorhinolaryngol. 2003;67:635–9.CrossRefGoogle Scholar
  20. 20.
    Tabor MH, Desai KR, Respler DS. Symptomatic bilateral nasolacrimal duct cysts in a newborn. Ear Nose Throat J. 2003;82:90–2.CrossRefGoogle Scholar
  21. 21.
    Leonard DS, O’Keefe M, Rowley H, et al. Neonatal respiratory distress secondary to bilateral intranasal dacryocystocoeles. Int J Pediatr Otorhinolaryngol. 2008;72:1873–7.CrossRefGoogle Scholar
  22. 22.
    Hepler KM, Woodson GE, Kearns DB. Respiratory distress in the neonate. Sequela of a congenital dacryocystocele. Arch Otolaryngol Head Neck Surg. 1995;121:1423–5.CrossRefGoogle Scholar
  23. 23.
    Rahbar R, Resto VA, Robson CD, et al. Nasal glioma and encephalocele: diagnosis and management. Laryngoscope. 2003;113:2069–77.CrossRefGoogle Scholar
  24. 24.
    Abdel-Aziz M, El-Bosraty H, Qotb M, et al. Nasal encephalocele: endoscopic excision with anesthetic consideration. Int J Pediatr Otorhinolaryngol. 2010;74:869–73.CrossRefGoogle Scholar
  25. 25.
    Tirumandas M, Sharma A, Gbenimacho I, et al. Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst. 2013;29:739–44.CrossRefGoogle Scholar
  26. 26.
    Singh AK, Upadhyaya DN. Sincipital encephaloceles. J Craniofac Surg. 2009;20(Suppl 2):1851–5.CrossRefGoogle Scholar
  27. 27.
    Steven RA, Rothera MP, Tang V, et al. An unusual cause of nasal airway obstruction in a neonate: trans-sellar, trans-sphenoidal cephalocoele. J Laryngol Otol. 2011;125:1075–8.CrossRefGoogle Scholar
  28. 28.
    Narasimhan K, Coticchia J. Transsphenoidal encephalocele in a neonate. Ear Nose Throat J. 2006;85(420):422.Google Scholar
  29. 29.
    Bradley PJ, Singh SD. Nasal glioma. J Laryngol Otol. 1985;99:247–52.CrossRefGoogle Scholar
  30. 30.
    Frodel JL, Larrabee WF, Raisis J. The nasal dermoid. Otolaryngol Head Neck Surg. 1989;101:392–6.CrossRefGoogle Scholar
  31. 31.
    Gorenstein A, Kern EB, Facer GW, et al. Nasal gliomas. Arch Otolaryngol. 1980;106:536–40.CrossRefGoogle Scholar
  32. 32.
    Denoyelle F, Ducroz V, Roger G, et al. Nasal dermoid sinus cysts in children. Laryngoscope. 1997;107:795–800.CrossRefGoogle Scholar
  33. 33.
    Hanikeri M, Waterhouse N, Kirkpatrick N, et al. The management of midline transcranial nasal dermoid sinus cysts. Br J Plast Surg. 2005;58:1043–50.CrossRefGoogle Scholar
  34. 34.
    Hartley BE, Eze N, Trozzi M, et al. Nasal dermoids in children: a proposal for a new classification based on 103 cases at Great Ormond Street Hospital. Int J Pediatr Otorhinolaryngol. 2015;79:18–22.CrossRefGoogle Scholar
  35. 35.
    Rahbar R, Shah P, Mulliken JB, et al. The presentation and management of nasal dermoid: a 30-year experience. Arch Otolaryngol Head Neck Surg. 2003;129:464–71.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Ceren Günel
    • 1
  • Sema Başak
    • 1
  • Elina Toskala
    • 2
  1. 1.Department of Otorhinolaryngology-Head and Neck Surgery, School of MedicineAdnan Menderes UniversityAydınTurkey
  2. 2.Department of Otolaryngology-Head and Neck SurgeryLewis Katz School of Medicine, Temple UniversityPhiladelphiaUSA

Personalised recommendations