Abstract
Purpose of review
To describe the clinical presentation, diagnosis, and management of pediatric nasal encephaloceles.
Recent findings
Encephaloceles and meningoencephaloceles are on the differential diagnosis for congenital midline nasal masses. These lesions are treated surgically to prevent craniofacial deformities and meningitis and to address symptoms such as a nasal obstruction, feeding difficulties, and cerebrospinal fluid (CSF) rhinorrhea. With the advent of endoscopic skull base approaches and instruments, surgical treatment of nasal encephaloceles have transitioned from large bicoronal external approaches to more minimally invasive endonasal endoscopic approaches. Endoscopic surgical approaches are safe in pediatric patients.
Summary
Pediatric intranasal encephaloceles are more frequently managed endoscopically. However, due to the rarity of this condition, further multi-institutional research is needed to examine long-term outcomes for this surgical approach.
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Hoving EW. Nasal encephaloceles. Childs Nerv Syst. 2000;16(10–11):702–6. https://doi.org/10.1007/s003810000339.
Towfighi J. Cephaloceles. Medlink Neurology. 1995. Accessed October 23 2019.
Robbins KT, Thomas JR, Lesperance MM, Niparko JK, Flint PW, Haughey BH et al. Congenital malformation of the nose and nasopharynx. In: Elluru RG, editor. Cummings otolaryngology : head & neck surgery. 6th ed.: Elsevier/Saunders; 2015. p. 2944–55.
Hedlund G. Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol. 2006;36(7):647–62; quiz 726-7. https://doi.org/10.1007/s00247-005-0100-3.
Saettele M, Alexander A, Markovich B, Morelli J, Lowe LH. Congenital midline nasofrontal masses. Pediatr Radiol. 2012;42(9):1119–25. https://doi.org/10.1007/s00247-012-2409-z.
Suwanwela C, Suwanwela N. A morphological classification of sincipital encephalomeningoceles. J Neurosurg. 1972;36(2):201–11. https://doi.org/10.3171/jns.1972.36.2.0201.
Blumenfeld R, Skolnik EM. Intranasal encephaloceles. Arch Otolaryngol. 1965;82(5):527–31. https://doi.org/10.1001/archotol.1965.00760010529015.
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. https://doi.org/10.1016/j.ijporl.2009.04.023.
•• 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. https://doi.org/10.1007/s00381-012-1998-z This paper reviews the types of encephaloceles, treatment, and complications following repair.
Di Rocco F, Couloigner V, Dastoli P, Sainte-Rose C, Zerah M, Roger G. Treatment of anterior skull base defects by a transnasal endoscopic approach in children. J Neurosurg Pediatr. 2010;6(5):459–63. https://doi.org/10.3171/2010.8.PEDS09325.
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. https://doi.org/10.1097/00005537-200312000-00003.
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. https://doi.org/10.1001/archotol.130.11.1283.
Rahman NU. Nasal encephalocoele. Treatment by trans-cranial operation. J Neurol Sci. 1979;42(1):73–85. https://doi.org/10.1016/0022-510x(79)90153-9.
•• 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. https://doi.org/10.1002/lary.25864 This paper presents a case series and reviews the presentation of encephaloceles and current management options for them.
Woodworth B, Schlosser RJ. Endoscopic repair of a congenital intranasal encephalocele in a 23 months old infant. Int J Pediatr Otorhinolaryngol. 2005;69(7):1007–9. https://doi.org/10.1016/j.ijporl.2005.02.003.
Formica F, Iannelli A, Paludetti G, Di Rocco C. Transsphenoidal meningoencephalocele. Childs Nerv Syst. 2002;18(6–7):295–8. https://doi.org/10.1007/s00381-002-0578-z.
Eide JG, Walz P, Alden TD, Rastatter JC. Pediatric skull base surgery: encephaloceles and cerebrospinal fluid leaks. Oper Tech Otolaryngol Head Neck Surg. 2019;30(1):30–6. https://doi.org/10.1016/j.otot.2019.01.012.
Tan SH, Mun KS, Chandran PA, Manuel AM, Prepageran N, Waran V, et al. Combined transnasal and transoral endoscopic approach to a transsphenoidal encephalocele in an infant. Childs Nerv Syst. 2015;31(7):1165–9. https://doi.org/10.1007/s00381-015-2667-9.
Albano L, Parisi V, Acerno S, Barzaghi LR, Losa M, Mortini P. Sublabial transsphenoidal microsurgical technique to treat congenital transsphenoidal encephalocele: a technical note. Neurosurg Rev. 2019;42(2):571–5. https://doi.org/10.1007/s10143-018-01075-z.
Lee KJ. Essential otolaryngology : head & neck surgery. 11th ed: McGraw-Hill, Medical Pub. Division; 2016. p. 536–40.
Schlosser RJ, Bolger WE. Management of multiple spontaneous nasal meningoencephaloceles. Laryngoscope. 2002;112(6):980–5. https://doi.org/10.1097/00005537-200206000-00008.
Roxbury CR, Lobo BC, Kshettry VR, D'Anza B, Woodard TD, Recinos PF, et al. Perioperative management in endoscopic endonasal skull-base surgery: a survey of the North American Skull Base Society. Int Forum Allergy Rhinol. 2018;8(5):631–40. https://doi.org/10.1002/alr.22066.
Johans SJ, Burkett DJ, Swong KN, Patel CR, Germanwala AV. Antibiotic prophylaxis and infection prevention for endoscopic endonasal skull base surgery: our protocol, results, and review of the literature. J Clin Neurosci. 2018;47:249–53. https://doi.org/10.1016/j.jocn.2017.10.036.
Brown SM, Anand VK, Tabaee A, Schwartz TH. Role of perioperative antibiotics in endoscopic skull base surgery. Laryngoscope. 2007;117(9):1528–32. https://doi.org/10.1097/MLG.0b013e3180caa177.
Shah RN, Surowitz JB, Patel MR, Huang BY, Snyderman CH, Carrau RL, et al. Endoscopic pedicled nasoseptal flap reconstruction for pediatric skull base defects. Laryngoscope. 2009;119(6):1067–75. https://doi.org/10.1002/lary.20216.
• Tatreau JR, Patel MR, Shah RN, McKinney KA, Wheless SA, Senior BA, et al. Anatomical considerations for endoscopic endonasal skull base surgery in pediatric patients. Laryngoscope. 2010;120(9):1730–7. https://doi.org/10.1002/lary.20964 This paper uses CT scans to predict feasibility of transphenoidal endoscopic approaches in pediatric patients.
Baradaran N, Nejat F, Baradaran N, El Khashab M. Cephalocele: report of 55 cases over 8 years. Pediatr Neurosurg. 2009;45(6):461–6. https://doi.org/10.1159/000277622.
Bothwell MR, Piccirillo JF, Lusk RP, Ridenour BD. Long-term outcome of facial growth after functional endoscopic sinus surgery. Otolaryngol Head Neck Surg. 2002;126(6):628–34. https://doi.org/10.1067/mhn.2002.125607.
Coelho G, Chaves TMF, Goes AF, Del Massa EC, Moraes O, Yoshida M. Multimaterial 3D printing preoperative planning for frontoethmoidal meningoencephalocele surgery. Childs Nerv Syst. 2018;34(4):749–56. https://doi.org/10.1007/s00381-017-3616-6.
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Cynthia Wang declares that she has no conflict of interest. Clarice Brown declares that she has no conflict of interest. Ron Mitchell declares that he has no conflict of interest. Gopi Shah declares that he has no conflict of interest.
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Wang, C.S., Brown, C., Mitchell, R.B. et al. Diagnosis and Management of Pediatric Nasal CSF Leaks and Encephaloceles. Curr Treat Options Allergy 7, 326–334 (2020). https://doi.org/10.1007/s40521-020-00266-8
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DOI: https://doi.org/10.1007/s40521-020-00266-8