Skip to main content

Naso-ethmoidal encephalocele with bilateral orbital extension: report of a case in a western country

Abstract

Introduction

Encephalocele is a rare congenital malformation of the central nervous system with protrusion of cranial content (meninges, brain, and ventricles in different combinations) beyond the normal confines of the skull. Anterior encephaloceles occur with a high frequency in Southeast Asia, while in the Western countries occipital encephaloceles prevail. The treatment of an anterior (naso-ethmoidal) encephalocele involves a neurosurgeon or a multidisciplinary team (neurosurgeon, maxillofacial surgeon, plastic surgeon, and ENT surgeon) dealing with craniofacial surgery. Goals of surgery include removal or repositioning of nonfunctional cerebral tissue, closure of the dura, and reconstruction of skeletal and cutaneous structures. The prognosis depends from the anatomical site, volume of neural contents, and the presence of coexisting malformations.

Case report

We report the case of an Italian child suffering from a naso-ethmoidal encephalocele with bilateral orbital extension. The surgical treatment was performed in two steps.

Conclusion

Sincipital encephalocele is a complex pathology without a unique standardized surgical treatment. Its low incidence in Western countries can make its management particularly tricky.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2

References

  1. Agthong S, Wiwanitkit V (2002) Encephalomeningocele cases over 10 years in Thailand: a case series. BMC Neurol 2:3

    PubMed  Article  Google Scholar 

  2. Boonvisut S, Ladpli S, Sujatanond M, Tandhavadhana C, Tisavipat N, Luxsuwong M, Nunta-aree S, Srimaharaja S, Panitphong T, Dulayajinda D, Areewattana S (1998) Morphologic study of 120 skull base defects in frontoethmoidal encephalomeningoceles. Plast Reconstr Surg 101(7):1784–1795

    PubMed  Article  CAS  Google Scholar 

  3. Boonvisut S, Ladpli S, Sujatanond M, Tisavipat N, Luxsuwong M, Nunta-aree S, Boonampol D, Srimaharaja S, Panitphong T, Dulayajinda D, Areewattana S (2001) A new technique for the repair and reconstruction of frontoethmoidal encephalomeningoceles by medial orbital composite-unit translocation. Br J Plast Surg 54(2):93–101. doi:10.1054/bjps.2000.3487

    PubMed  Article  CAS  Google Scholar 

  4. Brown MS, Sheridan-Pereira M (1992) Outlook for the child with a cephalocele. Pediatrics 90(6):914–919

    PubMed  CAS  Google Scholar 

  5. Bui CJ, Tubbs RS, Shannon CN, Acakpo-Satchivi L, Wellons JC 3rd, Blount JP, Oakes WJ (2007) Institutional experience with cranial vault encephaloceles. J Neurosurg 107(1 Suppl):22–25. doi:10.3171/PED-07/07/022

    PubMed  Google Scholar 

  6. Cohen MM Jr, Lemire RJ (1982) Syndromes with cephaloceles. Teratology 25(2):161–172. doi:10.1002/tera.1420250206

    PubMed  Article  Google Scholar 

  7. Date I, Yagyu Y, Asari S, Ohmoto T (1993) Long-term outcome in surgically treated encephalocele. Surg Neurol 40(2):125–130

    PubMed  Article  CAS  Google Scholar 

  8. David DJ (1993) Cephaloceles: classification, pathology, and management—a review. J Craniofac Surg 4(4):192–202

    PubMed  CAS  Google Scholar 

  9. David DJ, Sheffield L, Simpson D, White J (1984) Fronto-ethmoidal meningoencephaloceles: morphology and treatment. Br J Plast Surg 37(3):271–284

    PubMed  Article  CAS  Google Scholar 

  10. David DJ, Simpson DA (1987) Frontoethmoidal meningoencephaloceles. Clin Plast Surg 14(1):83–89

    PubMed  CAS  Google Scholar 

  11. Goldhammer Y, Smith JL (1975) Optic nerve anomalies in basal encephalocele. Arch Ophthalmol 93(2):115–118

    PubMed  Article  CAS  Google Scholar 

  12. Hemmy DC, David DJ (1985) Skeletal morphology of anterior encephaloceles defined through the use of three-dimensional reconstruction of computed tomography. Pediatr Neurosci 12(1):18–22

    PubMed  Article  Google Scholar 

  13. Hockley AD, Goldin JH, Wake MJ (1990) Management of anterior encephalocele. Childs Nerv Syst 6(8):444–446

    PubMed  Article  CAS  Google Scholar 

  14. Holm C, Thu M, Hans A, Martina M, Silvia GS, Moritz S, Wolfgang M (2008) Extracranial correction of frontoethmoidal meningoencephaloceles: feasibility and outcome in 52 consecutive cases. Plast Reconstr Surg 121(6):386e–395e. doi:10.1097/PRS.0b013e318170a78b

    PubMed  Article  Google Scholar 

  15. Holmes AD, Meara JG, Kolker AR, Rosenfeld JV, Klug GL (2001) Frontoethmoidal encephaloceles: reconstruction and refinements. J Craniofac Surg 12(1):6–18

    PubMed  Article  CAS  Google Scholar 

  16. Hoving EW (2000) Nasal encephaloceles. Childs Nerv Syst 16(10–11):702–706

    PubMed  Article  CAS  Google Scholar 

  17. Hoving EW, Vermeij-Keers C (1997) Frontoethmoidal encephaloceles, a study of their pathogenesis. Pediatr Neurosurg 27(5):246–256

    PubMed  Article  CAS  Google Scholar 

  18. Hoving EW, Vermeij-Keers C, Mommaas-Kienhuis AM, Hartwig NG (1990) Separation of neural and surface ectoderm after closure of the rostral neuropore. Anat Embryol (Berl) 182(5):455–463

    Article  CAS  Google Scholar 

  19. Humphreys RP (1994) Encephalocele and dermal sinuses. In: Cheek WR (ed) Pediatric neurosurgery: surgery of the developing nervous system, 3rd edn. W.B. Saunders, Philadelphia, pp 96–103

    Google Scholar 

  20. Jacob OJ, Rosenfeld JV, Watters DA (1994) The repair of frontal encephaloceles in Papua New Guinea. Aust N Z J Surg 64(12):856–860

    PubMed  Article  CAS  Google Scholar 

  21. Kumar A, Helling E, Guenther D, Crabtree T, Wexler AW, Bradley JP (2009) Correction of frontonasoethmoidal encephalocele: the HULA procedure. Plast Reconstr Surg 123(2):661–669. doi:10.1097/PRS.0b013e3181956633

    PubMed  Article  CAS  Google Scholar 

  22. Macfarlane R, Rutka JT, Armstrong D, Phillips J, Posnick J, Forte V, Humphreys RP, Drake J, Hoffman HJ (1995) Encephaloceles of the anterior cranial fossa. Pediatr Neurosurg 23(3):148–158

    PubMed  Article  CAS  Google Scholar 

  23. Mahapatra AK, Agrawal D (2006) Anterior encephaloceles: a series of 103 cases over 32 years. J Clin Neurosci 13(5):536–539. doi:10.1016/j.jocn.2005.05.016

    PubMed  Article  CAS  Google Scholar 

  24. Mahapatra AK, Tandon PN, Dhawan IK, Khazanchi RK (1994) Anterior encephaloceles: a report of 30 cases. Childs Nerv Syst 10(8):501–504

    PubMed  Article  CAS  Google Scholar 

  25. Mahatumarat C, Rojvachiranonda N, Taecholarn C (2003) Frontoethmoidal encephalomeningocele: surgical correction by the Chula technique. Plast Reconstr Surg 111(2):556–565. doi:10.1097/01.PRS.0000040523.57406.94, discussion 566–557

    PubMed  Article  Google Scholar 

  26. Mahatumarat C, Taecholarn C, Charoonsmith T (1991) One-stage extracranial repair and reconstruction for frontoethmoidal encephalomeningocele: a new simple technique. J Craniofac Surg 2(3):127–133, discussion 134

    PubMed  Article  CAS  Google Scholar 

  27. Martinez-Lage JF, Poza M, Sola J, Soler CL, Montalvo CG, Domingo R, Puche A, Ramon FH, Azorin P, Lasso R (1996) The child with a cephalocele: etiology, neuroimaging, and outcome. Childs Nerv Syst 12(9):540–550

    PubMed  Article  CAS  Google Scholar 

  28. McLone DG (2000) Congenital malformations of the central nervous system. Clin Neurosurg 47:346–377

    PubMed  Article  CAS  Google Scholar 

  29. Mulliken JB, Kaban LB, Evans CA, Strand RD, Murray JE (1986) Facial skeletal changes following hypertelorbitism correction. Plast Reconstr Surg 77(1):7–16

    PubMed  Article  CAS  Google Scholar 

  30. Naidich TP, Altman NR, Braffman BH, McLone DG, Zimmerman RA (1992) Cephaloceles and related malformations. AJNR Am J Neuroradiol 13(2):655–690

    PubMed  CAS  Google Scholar 

  31. Peter JC, Fieggen G (1999) Congenital malformations of the brain—a neurosurgical perspective at the close of the twentieth century. Childs Nerv Syst 15(11–12):635–645

    PubMed  Article  CAS  Google Scholar 

  32. Pinzer T, Lauer G, Gollogly J, Schackert G (2006) A complex therapy for treatment of frontoethmoidal meningoencephalocele in a developing third world country: neurosurgical aspects. J Neurosurg 104(5 Suppl):326–331. doi:10.3171/ped.2006.104.5.326

    PubMed  Google Scholar 

  33. Pollock JA, Newton TH, Hoyt WF (1968) Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology 90(3):442–453

    PubMed  CAS  Google Scholar 

  34. Rapport RL 2nd, Dunn RC Jr, Alhady F (1981) Anterior encephalocele. J Neurosurg 54(2):213–219. doi:10.3171/jns.1981.54.2.0213

    PubMed  Article  Google Scholar 

  35. Richards CG (1992) Frontoethmoidal meningoencephalocele: a common and severe congenital abnormality in South East Asia. Arch Dis Child 67(6):717–719

    PubMed  Article  CAS  Google Scholar 

  36. Rojvachiranonda N, David DJ, Moore MH, Cole J (2003) Frontoethmoidal encephalomeningocele: new morphological findings and a new classification. J Craniofac Surg 14(6):847–858

    PubMed  Article  Google Scholar 

  37. Rojvachiranonda N, Mahatumarat C, Taecholarn C (2006) Correction of the frontoethmoidal encephalomeningocele with minimal facial incision: modified Chula technique. J Craniofac Surg 17(2):353–357

    PubMed  Article  Google Scholar 

  38. Simpson DA, David DJ, White J (1984) Cephaloceles: treatment, outcome, and antenatal diagnosis. Neurosurgery 15(1):14–21

    PubMed  Article  CAS  Google Scholar 

  39. Suwanwela C, Sukabote C, Suwanwela N (1971) Frontoethmoidal encephalomeningocele. Surgery 69(4):617–625

    PubMed  CAS  Google Scholar 

  40. Suwanwela C, Suwanwela N (1972) A morphological classification of sincipital encephalomeningoceles. J Neurosurg 36(2):201–211. doi:10.3171/jns.1972.36.2.0201

    PubMed  Article  CAS  Google Scholar 

  41. Zee CS, McComb JG, Segall HD, Tsai FY, Stanley P (1981) Lipomas of the corpus callosum associated with frontal dysraphism. J Comput Assist Tomogr 5(2):201–205

    PubMed  Article  CAS  Google Scholar 

Download references

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Armando Cama.

Rights and permissions

Reprints and Permissions

About this article

Cite this article

Secci, F., Consales, A., Merciadri, P. et al. Naso-ethmoidal encephalocele with bilateral orbital extension: report of a case in a western country. Childs Nerv Syst 29, 1947–1952 (2013). https://doi.org/10.1007/s00381-013-2125-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-013-2125-5

Keywords

  • Frontoethmoidal
  • Sincipital
  • Encephalocele
  • Skull defect