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Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications

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Abstract

Introduction

Encephaloceles are considered by most to be a type of neural tube defect characterized by a herniation of the brain and meninges through structural weaknesses in the bony structures of the skull.

Discussion

Many different types of encephaloceles have been classified according to the location of the bony defect. Basal and frontoethmoidal encephaloceles constitute a nasal subclass of encephaloceles, which are herniations from the skull base and ethmoid bone, respectively. Basal encephaloceles are usually occult and can herniate into nasal structures causing obstruction. Frontoethmoidal encephaloceles usually present as a protrusion that is visible at birth and enlarges during crying. Both of these entities are rare with an incidence ranging from 1 in 5,000 to 1 in 40,000 live births around the world with the majority of cases localized in Southeast Asia.

Conclusion

Although cases have been recorded since the sixteenth century, there is still a scarcity of knowledge on the exact causes and factors associated with the development of the disease. Many studies have determined these to be caused by a combination of genetic and environmental factors. Most cases are nonlife threatening and the preferred method of treatment is surgical removal after diagnosis is confirmed with computerized tomography or magnetic resonance imaging. Prognosis is generally positive, especially in the subset of patients with frontoethmoidal encephaloceles.

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References

  1. Abdel-Aziz M, El-Bosraty H, Qotb M, El-Hamamsy M, El-Sonbaty M, Abdel-Badie H, Zynabdeen M (2010) Nasal encephalocele: endoscopic excision with anesthetic consideration. Int J Pediatr Otorhinolaryngol 74:869–873

    Article  PubMed  Google Scholar 

  2. Albright AL, Adelson PD, Pollack IF (2008) Principles and practice of pediatric neurosurgery, 2nd edn. Thieme, New York

    Google Scholar 

  3. Baradaran N, Nejat F, Baradaran N, El Khashab M (2009) Cephalocele: report of 55 cases over 8 years. Pediatr Neurosurg 45:461–466

    Article  PubMed  Google Scholar 

  4. Barnes L (2001) Surgical pathology of the head and neck, vol 3. Marcel Dekker, New York

    Google Scholar 

  5. Chen CS, David D, Hanieh A (2004) Morning glory syndrome and basal encephalocele. Childs Nerv Syst 20:87–90

    Article  PubMed  Google Scholar 

  6. Cinalli G, Maixner WJ, Sainte-Rose C (2004) Pediatric hydrocephalus. Springer, Milan

    Google Scholar 

  7. Fatterpekar GM, Naidich TP, Som PM (2012) The teaching files: brain and spine imaging. Elsevier/Saunders, Philadelphia

    Google Scholar 

  8. Formica F, Iannelli A, Paludetti G, Di Rocco C (2002) Transsphenoidal meningo-encephalocele. Child’s Nerv Syst 18:295–298

    CAS  Google Scholar 

  9. Franco D, Alonso N, Ruas R, da Silva FR, Franco T (2009) Transsphenoidal meningoencephalocele associated with cleft lip and palate: challenges for diagnosis and surgical treatment. Childs Nerv Syst 25:1455–1458

    Article  PubMed  Google Scholar 

  10. Goodrich JT (2008) Pediatric neurosurgery. Thieme, New York

    Google Scholar 

  11. Greenberg AM, Prein J (2002) Craniomaxillofacial reconstructive and corrective bone surgery: principles of internal fixation using the AO/ASIF technique. Springer, New York

    Book  Google Scholar 

  12. Hoving EW (2000) Nasal encephaloceles. Childs Nerv Syst 16:702–706

    Article  PubMed  CAS  Google Scholar 

  13. Chen 9: Kindler P (1970) Morning glory syndrome: unusual congenital optic disc anomaly. Am J Ophthalmol 69:376–384

    Google Scholar 

  14. Leelanukrom R, Wacharasint P, Kaewanuchit A (2007) Perioperative management for surgical correction of frontoethmoidal encephalomeningocele in children: a review of 102 cases. Pediatr Anesthes 17:856–862

    Article  Google Scholar 

  15. Mahajan C, Rath GP, Dash HH, Bithal PK (2011) Perioperative management of children with encephalocele: an institutional experience. J Neurosurg Anesthesiol 23:352–356

    Article  PubMed  Google Scholar 

  16. Mahapatra AK, Suri A (2002) Anterior encephaloceles: a study of 92 cases. Pediatr Neurosurg 36:113–118

    Article  PubMed  CAS  Google Scholar 

  17. Morina A, Kelmendi F, Morina Q, Dragusha S, Ahmeti F, Morina D (2011) Treatment of anterior encephaloceles over 24 years in Kosova. Med Arh 65:122–124

    PubMed  Google Scholar 

  18. Mukherjee D, Raza SM, Boahene K, Quinones-Hinojosa A (2010) Giant encephalocele. Br J Neurosurg 24:219–220

    Article  PubMed  Google Scholar 

  19. Narasimhan K, Coticchia J (2006) Transsphenoidal encephalocele in a neonate. Ear Nose Throat J 85(420):422

    Google Scholar 

  20. Oucheng N, Lauwers F, Gollogly J, Draper L, Joly B, Roux FE (2010) Frontoethmoidal meningoencephalocele: appraisal of 200 operated cases. J Neurosurg Pediatr 6:541–549

    Article  PubMed  Google Scholar 

  21. Pollack IF (1998) Management of encephaloceles and craniofacial problems in the neonatal period. Neurosurg Clin North Am 9:121–139

    CAS  Google Scholar 

  22. Rahbar R, Resto VA, Robson CD, Perez-Atayde AR, Goumnerova LC, McGill TJ, Healy GB (2003) Nasal glioma and encephalocele: diagnosis and management. Laryngoscope 113:2069–2077

    Article  PubMed  Google Scholar 

  23. Rathore YS, Sinha S, Mahapatra AK (2011) Transsellar transsphenoidal encephalocele: a series of four cases. Neurol India 59:289–292

    Article  PubMed  Google Scholar 

  24. Sano H, Matsuwaki Y, Kaito N, Joki T, Okushi T, Moriyama H (2011) A case of sphenoid sinus meningoencephalocele repaired by an image-guided endoscopic endonasal approach. Auris Nasus Larynx 38:632–637

    Article  PubMed  Google Scholar 

  25. Sindou M (2009) Practical handbook of neurosurgery from leading neurosurgeons, vol 3. Springer, Vienna

    Book  Google Scholar 

  26. Steven RA, Rothera MP, Tang V, Bruce IA (2011) An unusual cause of nasal airway obstruction in a neonate: trans-sellar, trans-sphenoidal cephalocoele. J Laryngol Otol 125:1075–1078

    Article  PubMed  CAS  Google Scholar 

  27. Suphapeetiporn K, Mahatumarat C, Rojvachiranonda N, Taecholarn C, Siriwan P, Srivuthana S, Shotelersuk V (2008) Risk factors associated with the occurrence of frontoethmoidal encephalomeningocele. Eur J Paediatr Neurol 12:102–107

    Article  PubMed  Google Scholar 

  28. Hoving 47: Tsutsumi K, Asano T, Shigeno T, Matsui T, Ito S, Kaizu H (1999) Transcranial approach for transphenoidal encephalocele: report of two cases. Surg Neurol 51:252–257

    Google Scholar 

  29. Wacharasindhu S, Asawutmangkul U, Srivuthana S (2005) Endocrine abnormalities in patients with frontoethmoidal encephalomeningocele. A preliminary study. Horm Res 64:64–67

    Article  PubMed  CAS  Google Scholar 

  30. Wang IJ, Lin SL, Tsou KI, Hsu MC, Chiu WT, Tsai SH, Lee LM, Lin TJ (2010) Congenital midline nasal mass: cases series and review of the literature. Turk J Pediatr 52:520–524

    PubMed  Google Scholar 

  31. Warf BC, Stagno V, Mugamba J (2011) Encephalocele in Uganda: ethnic distinctions in lesion location, endoscopic management of hydrocephalus, and survival in 110 consecutive children. J Neurosurg Pediatr 7:88–93

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Anatomical Sciences, School of Medicine, St. George’s University, St. George’s, Grenada, West Indies

    Madhuri Tirumandas, Amit Sharma, Ikechi Gbenimacho & Marios Loukas

  2. Pediatric Neurosurgery, Children’s Hospital, Birmingham, AL, USA

    Mohammadali M. Shoja, R. Shane Tubbs & W. Jerry Oakes

Authors
  1. Madhuri Tirumandas
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  2. Amit Sharma
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  3. Ikechi Gbenimacho
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  4. Mohammadali M. Shoja
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  5. R. Shane Tubbs
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  7. Marios Loukas
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Correspondence to Marios Loukas.

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Tirumandas, M., Sharma, A., Gbenimacho, I. et al. Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst 29, 739–744 (2013). https://doi.org/10.1007/s00381-012-1998-z

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  • DOI: https://doi.org/10.1007/s00381-012-1998-z

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