Congenital and Pediatric Frontal Pathology

  • Joseph S. Schwartz
  • Nithin D. Adappa


Congenital midline nasal masses (nasal dermoids, nasal gliomas, and congenital encephaloceles) represent a rare group of lesions arising from faulty development of the anterior neuropore. Pediatric rhinosinusitis is a disease entity commonly encountered by pediatric otolaryngologists with rare instances of associated complications. Frontal sinusitis in this patient population represents an important risk factor for intracranial infectious complications given its close relationship to the cranial vault. The goal of this chapter is to provide a comprehensive review of both congenital midline nasal masses and complications of pediatric frontal sinusitis with particular attention given to the underlying pathophysiology, clinical approach, and treatment strategies of these disease entities.


Nasal dermoid Dermoid cyst Nasal glioma Sincipital encephalocele Basal encephalocele Congenital encephalocele Congenital midline mass 



None for either author.


  1. 1.
    Krakovitz PR, Koltai PJ. Neonatal nasal obstruction. NeoReviews. 2007;8:e199–205.CrossRefGoogle Scholar
  2. 2.
    Baxter DJ, Shroff M. Congenital midface abnormalities. Neuroimaging Clin N Am. 2011;21(3):563–84. vii-viiiCrossRefPubMedGoogle Scholar
  3. 3.
    Rohrich RJ, Lowe JB, Schwartz MR. The role of open rhinoplasty in the management of nasal dermoid cysts. Plast Reconstr Surg. 1999;104(5):1459–66; quiz 67; discussion 68CrossRefPubMedGoogle Scholar
  4. 4.
    Pinheiro-Neto CD, Snyderman CH, Fernandez-Miranda J, Gardner PA. Endoscopic endonasal surgery for nasal dermoids. Otolaryngol Clin N Am. 2011;44(4):981–7, ixCrossRefGoogle Scholar
  5. 5.
    Denoyelle F, Ducroz V, Roger G, Garabedian EN. Nasal dermoid sinus cysts in children. Laryngoscope. 1997;107(6):795–800.CrossRefPubMedGoogle Scholar
  6. 6.
    Zapata S, Kearns DB. Nasal dermoids. Curr Opin Otolaryngol Head Neck Surg. 2006;14(6):406–11.CrossRefPubMedGoogle Scholar
  7. 7.
    Wardinsky TD, Pagon RA, Kropp RJ, Hayden PW, Clarren SK. Nasal dermoid sinus cysts: association with intracranial extension and multiple malformations. Cleft Palate Craniofac J. 1991;28(1):87–95.CrossRefPubMedGoogle Scholar
  8. 8.
    Paradis J, Koltai PJ. Pediatric teratoma and dermoid cysts. Otolaryngol Clin N Am. 2015;48(1):121–36.CrossRefGoogle Scholar
  9. 9.
    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(8):1043–50.CrossRefPubMedGoogle Scholar
  10. 10.
    Sreetharan V, Kangesu L, Sommerlad BC. Atypical congenital dermoids of the face: a 25-year experience. J Plast Reconstr Aesthet Surg. 2007;60(9):1025–9.CrossRefPubMedGoogle Scholar
  11. 11.
    Bloom DC, Carvalho DS, Dory C, Brewster DF, Wickersham JK, Kearns DB. Imaging and surgical approach of nasal dermoids. Int J Pediatr Otorhinolaryngol. 2002;62(2):111–22.CrossRefPubMedGoogle Scholar
  12. 12.
    Szeremeta W, Parikh TD, Widelitz JS. Congenital nasal malformations. Otolaryngol Clin N Am. 2007;40(1):97–112, vi–viiCrossRefGoogle Scholar
  13. 13.
    Heywood RL, Lyons MJ, Cochrane LA, Hayward R, Hartley BE. Excision of nasal dermoids with intracranial extension – anterior small window craniotomy approach. Int J Pediatr Otorhinolaryngol. 2007;71(8):1193–6.CrossRefPubMedGoogle Scholar
  14. 14.
    Herrington H, Adil E, Moritz E, Robson C, Perez-Atayde A, Proctor M, et al. Update on current evaluation and management of pediatric nasal dermoid. Laryngoscope. 2016;126(9):2151–60.CrossRefPubMedGoogle Scholar
  15. 15.
    Hedlund G. Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol. 2006;36(7):647–62; quiz 726–7CrossRefPubMedGoogle Scholar
  16. 16.
    Pensler JM, Bauer BS, Naidich TP. Craniofacial dermoids. Plast Reconstr Surg. 1988;82(6):953–8.CrossRefPubMedGoogle Scholar
  17. 17.
    Huisman TA, Schneider JF, Kellenberger CJ, Martin-Fiori E, Willi UV, Holzmann D. Developmental nasal midline masses in children: neuroradiological evaluation. Eur Radiol. 2004;14(2):243–9.CrossRefPubMedGoogle Scholar
  18. 18.
    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(4):745–54; discussion 55–6CrossRefPubMedGoogle Scholar
  19. 19.
    Pollock RA. Surgical approaches to the nasal dermoid cyst. Ann Plast Surg. 1983;10(6):498–501.CrossRefPubMedGoogle Scholar
  20. 20.
    Kellman RM, Goyal P, Rodziewicz GS. The transglabellar subcranial approach for nasal dermoids with intracranial extension. Laryngoscope. 2004;114(8):1368–72.CrossRefPubMedGoogle Scholar
  21. 21.
    Goyal P, Kellman RM, Tatum SA 3rd. Transglabellar subcranial approach for the management of nasal masses with intracranial extension in pediatric patients. Arch Facial Plast Surg. 2007;9(5):314–7.CrossRefPubMedGoogle Scholar
  22. 22.
    Shlomi B, Chaushu S, Gil Z, Chaushu G, Fliss DM. Effects of the subcranial approach on facial growth and development. Otolaryngol Head Neck Surg. 2007;136(1):27–32.CrossRefPubMedGoogle Scholar
  23. 23.
    Khalili S, Palmer JN, Adappa ND. The expanded endonasal approach for the treatment of intracranial skull base disease in the pediatric population. Curr Opin Otolaryngol Head Neck Surg. 2015;23(1):65–70.PubMedGoogle Scholar
  24. 24.
    Rahbar R, Resto VA, Robson CD, Perez-Atayde AR, Goumnerova LC, McGill TJ, et al. Nasal glioma and encephalocele: diagnosis and management. Laryngoscope. 2003;113(12):2069–77.CrossRefPubMedGoogle Scholar
  25. 25.
    Tirumandas M, Sharma A, Gbenimacho I, Shoja MM, Tubbs RS, Oakes WJ, et al. Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst. 2013;29(5):739–44.CrossRefPubMedGoogle Scholar
  26. 26.
    Singh AK, Upadhyaya DN. Sincipital encephaloceles. J Craniofac Surg. 2009;20(Suppl 2):1851–5.CrossRefPubMedGoogle Scholar
  27. 27.
    Ajose-Popoola O, Lin HW, Silvera VM, Teot LA, Madsen JR, Meara JG, et al. Nasal glioma: prenatal diagnosis and multidisciplinary surgical approach. Skull Base Rep. 2011;1(2):83–8.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Lowe LH, Booth TN, Joglar JM, Rollins NK. Midface anomalies in children. Radiographics. 2000;20(4):907–22; quiz 1106–7, 12CrossRefPubMedGoogle Scholar
  29. 29.
    Adil E, Robson C, Perez-Atayde A, Heffernan C, Moritz E, Goumnerova L, et al. Congenital nasal neuroglial heterotopia and encephaloceles: an update on current evaluation and management. Laryngoscope. 2016;126(9):2161–7.CrossRefPubMedGoogle Scholar
  30. 30.
    Schlosser RJ, Faust RA, Phillips CD, Gross CW. Three-dimensional computed tomography of congenital nasal anomalies. Int J Pediatr Otorhinolaryngol. 2002;65(2):125–31.CrossRefPubMedGoogle Scholar
  31. 31.
    Dasgupta NR, Bentz ML. Nasal gliomas: identification and differentiation from hemangiomas. J Craniofac Surg. 2003;14(5):736–8.CrossRefPubMedGoogle Scholar
  32. 32.
    Woodworth BA, Schlosser RJ, Faust RA, Bolger WE. Evolutions in the management of congenital intranasal skull base defects. Arch Otolaryngol Head Neck Surg. 2004;130(11):1283–8.CrossRefPubMedGoogle Scholar
  33. 33.
    Keshri A, Shah S, Patadia S, Sahu R, Behari S. Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci. 2016;11(1):42–5. Scholar
  34. 34.
    Castelnuovo P, Bignami M, Pistochini A, Battaglia P, Locatelli D, Dallan I. Endoscopic endonasal management of encephaloceles in children: an eight-year experience. Int J Pediatr Otorhinolaryngol. 2009;73(8):1132–6.CrossRefPubMedGoogle Scholar
  35. 35.
    Hoving EW. Nasal encephaloceles. Childs Nerv Syst. 2000;16(10–11):702–6.CrossRefPubMedGoogle Scholar
  36. 36.
    Nogueira JF Jr, Stamm AC, Vellutini E, Santos FP. Endoscopic management of congenital meningo-encephalocele with nasal flaps. Int J Pediatr Otorhinolaryngol. 2009;73(1):133–7.CrossRefPubMedGoogle Scholar
  37. 37.
    Lang EE, Curran AJ, Patil N, Walsh RM, Rawluk D, Walsh MA. Intracranial complications of acute frontal sinusitis. Clin Otolaryngol Allied Sci. 2001;26(6):452–7.CrossRefPubMedGoogle Scholar
  38. 38.
    Oxford LE, McClay J. Complications of acute sinusitis in children. Otolaryngol Head Neck Surg. 2005;133(1):32–7.CrossRefPubMedGoogle Scholar
  39. 39.
    Patel NA, Garber D, Hu S, Kamat A. Systematic review and case report: intracranial complications of pediatric sinusitis. Int J Pediatr Otorhinolaryngol. 2016;86:200–12.CrossRefPubMedGoogle Scholar
  40. 40.
    Ong YK, Tan HK. Suppurative intracranial complications of sinusitis in children. Int J Pediatr Otorhinolaryngol. 2002;66(1):49.CrossRefPubMedGoogle Scholar
  41. 41.
    Reid JR. Complications of pediatric paranasal sinusitis. Pediatr Radiol. 2004;34(12):933–42.CrossRefPubMedGoogle Scholar
  42. 42.
    Herrmann BW, Forsen JW Jr. Simultaneous intracranial and orbital complications of acute rhinosinusitis in children. Int J Pediatr Otorhinolaryngol. 2004;68(5):619–25.CrossRefPubMedGoogle Scholar
  43. 43.
    Lerner DN, Choi SS, Zalzal GH, Johnson DL. Intracranial complications of sinusitis in childhood. Ann Otol Rhinol Laryngol. 1995;104(4 Pt 1):288–93.CrossRefPubMedGoogle Scholar
  44. 44.
    Kombogiorgas D, Seth R, Athwal R, Modha J, Singh J. Suppurative intracranial complications of sinusitis in adolescence. Single institute experience and review of literature. Br J Neurosurg. 2007;21(6):603–9.CrossRefPubMedGoogle Scholar
  45. 45.
    Glickstein JS, Chandra RK, Thompson JW. Intracranial complications of pediatric sinusitis. Otolaryngol Head Neck Surg. 2006;134(5):733–6.CrossRefPubMedGoogle Scholar
  46. 46.
    Quraishi H, Zevallos JP. Subdural empyema as a complication of sinusitis in the pediatric population. Int J Pediatr Otorhinolaryngol. 2006;70(9):1581–6.CrossRefPubMedGoogle Scholar
  47. 47.
    Brook I. Microbiology and antimicrobial treatment of orbital and intracranial complications of sinusitis in children and their management. Int J Pediatr Otorhinolaryngol. 2009;73(9):1183–6.CrossRefPubMedGoogle Scholar
  48. 48.
    Patel AP, Masterson L, Deutsch CJ, Scoffings DJ, Fish BM. Management and outcomes in children with sinogenic intracranial abscesses. Int J Pediatr Otorhinolaryngol. 2015;79(6):868–73.CrossRefPubMedGoogle Scholar
  49. 49.
    Jones RL, Violaris NS, Chavda SV, Pahor AL. Intracranial complications of sinusitis: the need for aggressive management. J Laryngol Otol. 1995;109(11):1061–2.CrossRefPubMedGoogle Scholar
  50. 50.
    Fenton JE, Smyth DA, Viani LG, Walsh MA. Sinogenic brain abscess. Am J Rhinol. 1999;13(4):299–302.CrossRefPubMedGoogle Scholar
  51. 51.
    Brook I, Frazier EH. Microbiology of subperiosteal orbital abscess and associated maxillary sinusitis. Laryngoscope. 1996;106(8):1010–3.CrossRefPubMedGoogle Scholar
  52. 52.
    Germiller JA, Monin DL, Sparano AM, Tom LW. Intracranial complications of sinusitis in children and adolescents and their outcomes. Arch Otolaryngol Head Neck Surg. 2006;132(9):969–76.CrossRefPubMedGoogle Scholar
  53. 53.
    Felsenstein S, Williams B, Shingadia D, Coxon L, Riordan A, Demetriades AK, et al. Clinical and microbiologic features guiding treatment recommendations for brain abscesses in children. Pediatr Infect Dis J. 2013;32(2):129–35.CrossRefPubMedGoogle Scholar
  54. 54.
    Garin A, Thierry B, Leboulanger N, Blauwblomme T, Grevent D, Blanot S, et al. Pediatric sinogenic epidural and subdural empyema: the role of endoscopic sinus surgery. Int J Pediatr Otorhinolaryngol. 2015;79(10):1752–60.CrossRefPubMedGoogle Scholar
  55. 55.
    DelGaudio JM, Evans SH, Sobol SE, Parikh SL. Intracranial complications of sinusitis: what is the role of endoscopic sinus surgery in the acute setting. Am J Otolaryngol. 2010;31(1):25–8.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Joseph S. Schwartz
    • 1
  • Nithin D. Adappa
    • 2
  1. 1.Department of Otolaryngology – Head and Neck SurgeryMcGill UniversityMontrealCanada
  2. 2.Department of Otorhinolaryngology – Head and Neck SurgeryUniversity of Pennsylvania, Perelman School of MedicinePhiladelphiaUSA

Personalised recommendations