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Congenital Malformations of the Nose and Nasopharynx

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Congenital Malformations of the Head and Neck

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Abstract

This chapter provides embryologic and clinical descriptions, photographs, and illustrations of the more common congenital anomalies of the nose and nasopharynx. The authors present the topics in sections based on the underlying pathology. These sections include malformations that occur because the nose failed to form properly (such as arhinia, polyrhinia, and nasal colomboma), those that resulted in midline nasal and nasopharyngeal malformations (such as choanal atresia and craniopharyngioma), and those that resulted in congenital nasopharyngeal or nasal midline masses (such as epignathi, Thornwaldt cysts, dermoids, gliomas, and encephaloceles).

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References

  1. Hengerer AS, Wein RO. Congenital malformations of the nose and paranasal sinuses. In: Bluestone CD, Casselbrant ML, Stool SE, et al., editors. Pediatric otolaryngology. Philadelphia, PA: Saunders; 2003. p. 979–94.

    Google Scholar 

  2. Cohen MM. Nose. In: Stevenson RE, Hall JG, editors. Human malformations and related anomalies. New York, NY: Oxford University Press; 2006. p. 373–87.

    Google Scholar 

  3. Moore KL. The branchial apparatus and the head and neck. In: The developing human: clinically oriented embryology. Philadelphia, PA: W. B. Saunders Company; 1988. p. 170–206.

    Google Scholar 

  4. Moore KL. The developing human—clinically oriented embryology. Philadelphia, PA: W.B. Saunders Company; 1988.

    Google Scholar 

  5. Albernaz VS, Castillo M, Mukherji SK, Ihmeidan IH. Congenital arhinia. AJNR Am J Neuroradiol. 1996;17:1312–4.

    PubMed  CAS  Google Scholar 

  6. Weinberg A, Neuman A, Benmeir P, Lusthaus S, Wexler MR. A rare case of arhinia with severe airway obstruction: case report and review of the literature. Plast Reconstr Surg. 1993;91:146–9.

    Article  PubMed  CAS  Google Scholar 

  7. Cole RR, Myer 3rd CM, Bratcher GO. Congenital absence of the nose: a case report. Int J Pediatr Otorhinolaryngol. 1989;17:171–7.

    Article  PubMed  CAS  Google Scholar 

  8. Guion-Almeida ML, Machado-Paula LA, Richieri-Costa A. Newly recognized syndrome with heminasal aplasia and ocular anomalies or wider spectrum of heminasal aplasia/atypical clefting syndrome? Am J Med Genet A. 2004;129A:156–61.

    Article  PubMed  Google Scholar 

  9. Turkaslan T, Ozcan H, Genc B, Ozsoy Z. Combined intraoral and nasal approach to Tessier No: 0 cleft with bifid nose. Ann Plast Surg. 2005;54:207–10.

    Article  PubMed  CAS  Google Scholar 

  10. Miller PJ, Grinberg D, Wang TD. Midline cleft. Treatment of the bifid nose. Arch Fac Plast Surg. 1999;1:200–3.

    Article  CAS  Google Scholar 

  11. English GM. Congenital anomalies of the nose, nasopharynx, and paranasal sinuses. Philadelphia, PA: Lippincott, Williams, and Wilkins; 1998.

    Google Scholar 

  12. Thorne MC, Ruiz RE, Carvalho J, Lesperance MM. Proboscis lateralis: case report and review. Arch Otolaryngol Head Neck Surg. 2007;133:1051–3.

    Article  PubMed  Google Scholar 

  13. Eroglu L, Uysal OA. Proboscis lateralis: report of two cases. Br J Plast Surg. 2003;56:704–8.

    Article  PubMed  Google Scholar 

  14. Brown OE, Pownell P, Manning SC. Choanal atresia: a new anatomic classification and clinical management applications. Laryngoscope. 1996;106:97–101.

    Article  PubMed  CAS  Google Scholar 

  15. Harney MS, Russell J. Choanal atresia. In: Puri P, Hollward ME, editors. Pediatric surgery: diagnosis and management. Berlin: Springer-Verlag; 2009. p. 223–8.

    Google Scholar 

  16. Harris J, Robert E, Kallen B. Epidemiology of choanal atresia with special reference to the CHARGE association. Pediatrics. 1997;99:363–7.

    Article  PubMed  CAS  Google Scholar 

  17. Leclerc JE, Fearon B. Choanal atresia and associated anomalies. Int J Pediatr Otorhinolaryngol. 1987;13:265–72.

    Article  PubMed  CAS  Google Scholar 

  18. Freng A. Surgical treatment of congenital choanal atresia. Ann Otol Rhinol Laryngol. 1978;87:346–50.

    PubMed  CAS  Google Scholar 

  19. Bunin GR, Surawicz TS, Witman PA, Preston-Martin S, Davis F, Bruner JM. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998;89:547–51.

    Article  PubMed  CAS  Google Scholar 

  20. Savas A, Erdem A, Tun K, Kanpolat Y. Fatal toxic effect of bleomycin on brain tissue after intracystic chemotherapy for a craniopharyngioma: case report. Neurosurgery. 2000;46:213–6. discussion 6-7.

    Article  PubMed  CAS  Google Scholar 

  21. Sanford RA, Muhlbauer MS. Craniopharyngioma in children. Neurol Clin. 1991;9:453–65.

    PubMed  CAS  Google Scholar 

  22. Weiner HL, Wisoff JH, Rosenberg ME, et al. Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery. 1994;35:1001–10. discussion 10–1.

    Article  PubMed  CAS  Google Scholar 

  23. Hasegawa T, Kondziolka D, Hadjipanayis CG, Lunsford LD. Management of cystic craniopharyngiomas with phosphorus-32 intracavitary irradiation. Neurosurgery. 2004;54:813–20. discussion 20–2.

    Article  PubMed  Google Scholar 

  24. Broggi G, Franzini A. Bleomycin for cystic craniopharyngioma. J Neurosurg. 1996;84:1080–1.

    PubMed  CAS  Google Scholar 

  25. Cavalheiro S, Sparapani FV, Franco JO, da Silva MC, Braga FM. Use of bleomycin in intratumoral chemotherapy for cystic craniopharyngioma. Case report. J Neurosurg. 1996;84:124–6.

    Article  PubMed  CAS  Google Scholar 

  26. Belden CJ, Mancuso AA, Schmalfuss IL. CT features of congenital nasal piriform aperture stenosis: initial experience. Radiology. 1999;213:495–501.

    Article  PubMed  CAS  Google Scholar 

  27. Hui Y, Friedberg J, Crysdale WS. Congenital nasal pyriform aperture stenosis as a presenting feature of holoprosencephaly. Int J Pediatr Otorhinolaryngol. 1995;31:263.

    Article  PubMed  CAS  Google Scholar 

  28. Brown OE, Myer CM, Manning SC. Congenital nasal pyriform aperture stenosis. Laryngoscope. 1989;99:86.

    Article  PubMed  CAS  Google Scholar 

  29. Shikowitz MJ. Congenital nasal piriform aperture stenosis: diagnosis and treatment. Int J Pediatr Otorhinolaryngol. 2003;67:635–9.

    Article  PubMed  Google Scholar 

  30. Haghighi K, Milles M, Cleveland D, Ziccardi V. Epignathus teratoma with bifid tongue and median glossal salivary mass: report of a Case. J Oral Maxillofac Surg. 2004;62:379–84.

    Article  PubMed  Google Scholar 

  31. Hatzihaberis F, Stamatis D, Staurinos D. Giant epignathus. J Pediatr Surg. 1978;13:517–8.

    Article  PubMed  CAS  Google Scholar 

  32. Mayer AFCJ. Bursa seu Cystis tubae eustachianae bei eingen saugethieren. Neue Notizen aus dem Gebiete der Nature Heilkunde von Froeiep. 1840;14:1.

    Google Scholar 

  33. Dohman G, Sjovall A. A large epignathus teratoma, successfully operated on immediately after birth. Glassgow Med J. 1953;34(3):123–5.

    Google Scholar 

  34. Tornwaldt GL. Uber die Bedeutung der bursa pharygea fur die erkennung und behandlung gewisser nasenrauchenraum. Krankheiten. Weisbaden: J.F. Bergmann; 1885.

    Google Scholar 

  35. Battino RA, Khangure MS. Is that another Thornwaldt's cyst on M.R.I.? Australas Radiol. 1990;34:19–23.

    Article  PubMed  CAS  Google Scholar 

  36. Ford WJ, Brooks BS, el Gammal T. Thornwaldt cyst: an incidental MR diagnosis. Am J Neuroradiol. 1987;8:922–3.

    PubMed  CAS  Google Scholar 

  37. Moody MW, Chi DH, Mason JC, et al. Tornwaldt’s cyst: incidence and a case report. Ear Nose Throat J. 2007;86(3):129.

    Google Scholar 

  38. Kelly A, Bough Jr ID, Luft JD, Conard K, Reilly JS, Tuttle D. Hairy polyp of the oropharynx: case report and literature review. J Pediatr Surg. 1996;31:704–6.

    Article  PubMed  CAS  Google Scholar 

  39. Sexton M. Hairy polyp of the oropharynx: a case report with speculation on nosology. Am J Dermatopathol. 1990;12:294–8.

    Article  PubMed  CAS  Google Scholar 

  40. Yossuck P, Williams HJ, Polak MJ, Vaughan R. Oropharyngeal tumor in the newborn: a case report. Neonatology. 2007;91:69–72.

    Article  PubMed  Google Scholar 

  41. Chang SS, Halushka M, Meer JV, Goins M, Francis HW. Nasopharyngeal hairy polyp with recurrence in the middle ear. Int J Pediatr Otorhinolaryngol. 2008;72(2):261–4.

    Article  PubMed  Google Scholar 

  42. Roh JL. Transoral endoscopic resection of nasopharyngeal hairy polyp. Int J Pediatr Otorhinolaryngol. 2004;68:1087–90.

    Article  PubMed  Google Scholar 

  43. Burns BV, Axon PR, Pahade A. Hairy polyp of the pharynx in association with an ipsilateral branchial sinus: evidence that the “hairy polyp” is a second branchial arch malformation. J Laryngol Otol. 2001;115:145–8.

    PubMed  CAS  Google Scholar 

  44. Crawford JK, Webster JP. Congenital dermoid cyst of the nose. Plast Reconstr Surg. 1952;9:235–40.

    Article  CAS  Google Scholar 

  45. Kearns DB, Zapata S. Nasal dermoids. Curr Opin Otolaryngol Head Neck Surg. 2006;14:406–11.

    Article  PubMed  Google Scholar 

  46. Ma KJ, Cheung KL. Nasal glioma. Hong Kong Med J. 2006;12:477–9.

    PubMed  CAS  Google Scholar 

  47. Pratt LW. Midline cyst of the nasal dorsum: Embryologic origin and treatment. Laryngoscope. 1985;75:968–75.

    Article  Google Scholar 

  48. Denoyelle F, Ducroz V, Roger G, et al. Nasal dermoid sinus cysts in children. Laryngoscope. 1997;107:795–800.

    Article  PubMed  CAS  Google Scholar 

  49. Wardinsky T, Pagon R, Kropp R, Hayden P, Clarren S. Nasal dermoid sinus cyst: association with intracranial extension and multiple malformations. Cleft Pal Craniofac J. 1991;28:87–95.

    Article  CAS  Google Scholar 

  50. Hanikeri M, Waterhouse N, Kirkpatrick N, Peterson D, Macleod I. The management of midline transcranial nasal dermoid sinus cysts. Br J Plast Surg. 2005;58:1043–50.

    Article  PubMed  CAS  Google Scholar 

  51. Posnick JC, Bortoluzzi P, Armstrong DC, Drake JM. Intracranial nasal dermoid sinus cysts: computed tomographic scan findings and surgical results. Plast Reconstr Surg. 1994;93:745–54.

    Article  PubMed  CAS  Google Scholar 

  52. Goyal P, Kellman RM, Tatum SA. Transglabellar subcranial approach for the management of nasal masses with intracranial extension in pediatric patients. Arch Facial Plast Surg. 2007;9:314–7.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Division of Pediatric Otolaryngology, The Children’s Hospital of Philadelphia, 3400 Civic Center Boulevard, Wood Building, 1st Floor, Philadelphia, PA, 19104, USA

    Mark D. Rizzi M.D. & Brian P. Dunham M.D.

  2. Department of Otolaryngology: Head and Neck Surgery, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA

    Mark D. Rizzi M.D. & Brian P. Dunham M.D.

Authors
  1. Mark D. Rizzi M.D.
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  2. Brian P. Dunham M.D.
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Corresponding author

Correspondence to Mark D. Rizzi M.D. .

Editor information

Editors and Affiliations

  1. Pediatric Otolaryngology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

    Lisa M. Elden

  2. Pediatric Otolaryngology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

    Karen B. Zur

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Rizzi, M.D., Dunham, B.P. (2014). Congenital Malformations of the Nose and Nasopharynx. In: Elden, L., Zur, K. (eds) Congenital Malformations of the Head and Neck. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-1714-0_3

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  • DOI: https://doi.org/10.1007/978-1-4419-1714-0_3

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4419-1713-3

  • Online ISBN: 978-1-4419-1714-0

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