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Frontoethmoidal Cephalocele
Surjith Vattoth, MD, FRCR; Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Frontoethmoidal cephalocele (FECeph)
                  • Frontonasal cephalocele (FNCeph)
                  • Nasoethmoidal cephalocele (NECeph)
                  • Nasoorbital cephalocele (NOCeph)
              • Synonyms

                • Sincipital cephalocele
              • Definitions

                • Cephalocele: Outward herniation of CNS contents through defect in cranium
                  • Term includes meningocele and meningoencephalocele
                • Congenital herniation of meninges and CSF (meningocele) + brain tissue (meningoencephalocele)
                • Through mesodermal defect in anterior skull/skull base presenting as extranasal, intranasal, or medial orbital mass
                • Do not confuse for basal encephaloceles, which occur through cribriform plate or sphenoid defects

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Arifin M et al: Frontoethmoidal encephalocele: clinical presentation, diagnosis, treatment, and complications in 400 cases. Childs Nerv Syst. 34(6):1161-8, 2018
                                              2. Keshri AK et al: Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci. 11(1):42-5, 2016
                                              3. Castelnuovo P et al: Endoscopic endonasal management of encephaloceles in children: an eight-year experience. Int J Pediatr Otorhinolaryngol. 73(8):1132-6, 2009
                                              4. Kumar A et al: Correction of frontonasoethmoidal encephalocele: the HULA procedure. Plast Reconstr Surg. 123(2):661-9, 2009
                                              5. Arshad AR et al: Frontoethmoidal encephalocele: treatment and outcome. J Craniofac Surg. 19(1):175-83, 2008
                                              6. Holm C et al: Extracranial correction of frontoethmoidal meningoencephaloceles: feasibility and outcome in 52 consecutive cases. Plast Reconstr Surg. 121(6):386e-95e, 2008
                                              7. Lo BW et al: Clinical predictors of developmental outcome in patients with cephaloceles. J Neurosurg Pediatr. 2(4):254-7, 2008
                                              8. Hedlund G: Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol. 36(7):647-62; quiz 726-7, 2006
                                              9. Rahbar R et al: Nasal glioma and encephalocele: diagnosis and management. Laryngoscope. 113(12):2069-77, 2003
                                              10. Rojvachiranonda N et al: Frontoethmoidal encephalomeningocele: new morphological findings and a new classification. J Craniofac Surg. 14(6):847-58, 2003
                                              11. Mahapatra AK et al: Anterior encephaloceles: a study of 92 cases. Pediatr Neurosurg. 36(3):113-8, 2002
                                              12. Holmes AD et al: Frontoethmoidal encephaloceles: reconstruction and refinements. J Craniofac Surg. 12(1):6-18, 2001
                                              13. De Ponte FS et al: Surgical treatment of frontoethmoidal encephalocele: a case report. J Craniofac Surg. 11(4):342-5, 2000
                                              14. Songur E et al: Management of frontoethmoidal (sincipital) encephalocele. J Craniofac Surg. 10(2):135-9, 1999
                                              15. Boonvisut S et al: Morphologic study of 120 skull base defects in frontoethmoidal encephalomeningoceles. Plast Reconstr Surg. 101(7):1784-95, 1998
                                              16. Turgut M et al: Congenital nasal encephalocele: a review of 35 cases. J Craniomaxillofac Surg. 23(1):1-5, 1995
                                              17. Koch BL et al: Congenital malformations causing skull base changes. Neuroimaging Clin N Am. 4(3):479-98, 1994
                                              18. Naidich TP et al: Cephaloceles and related malformations. AJNR Am J Neuroradiol. 13(2):655-90, 1992
                                              19. Barkovich AJ et al: Congenital nasal masses: CT and MR imaging features in 16 cases. AJNR Am J Neuroradiol. 12(1):105-16, 1991
                                              20. David DJ et al: Cephaloceles: classification, pathology, and management. World J Surg. 13(4):349-57, 1989
                                              21. Vattoth S, Chapman PR. Anterior skull base. In: Watanabe K, et al., eds. Anatomy for Plastic Surgery of the Face, Head, and Neck.1st ed. New York: Thieme Publishers; 2016:13-9
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Frontoethmoidal cephalocele (FECeph)
                                                              • Frontonasal cephalocele (FNCeph)
                                                              • Nasoethmoidal cephalocele (NECeph)
                                                              • Nasoorbital cephalocele (NOCeph)
                                                          • Synonyms

                                                            • Sincipital cephalocele
                                                          • Definitions

                                                            • Cephalocele: Outward herniation of CNS contents through defect in cranium
                                                              • Term includes meningocele and meningoencephalocele
                                                            • Congenital herniation of meninges and CSF (meningocele) + brain tissue (meningoencephalocele)
                                                            • Through mesodermal defect in anterior skull/skull base presenting as extranasal, intranasal, or medial orbital mass
                                                            • Do not confuse for basal encephaloceles, which occur through cribriform plate or sphenoid defects

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Ultrasonographic Findings

                                                                  • Imaging Recommendations

                                                                    DIFFERENTIAL DIAGNOSIS

                                                                      PATHOLOGY

                                                                      • General Features

                                                                        • Staging, Grading, & Classification

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Arifin M et al: Frontoethmoidal encephalocele: clinical presentation, diagnosis, treatment, and complications in 400 cases. Childs Nerv Syst. 34(6):1161-8, 2018
                                                                                          2. Keshri AK et al: Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci. 11(1):42-5, 2016
                                                                                          3. Castelnuovo P et al: Endoscopic endonasal management of encephaloceles in children: an eight-year experience. Int J Pediatr Otorhinolaryngol. 73(8):1132-6, 2009
                                                                                          4. Kumar A et al: Correction of frontonasoethmoidal encephalocele: the HULA procedure. Plast Reconstr Surg. 123(2):661-9, 2009
                                                                                          5. Arshad AR et al: Frontoethmoidal encephalocele: treatment and outcome. J Craniofac Surg. 19(1):175-83, 2008
                                                                                          6. Holm C et al: Extracranial correction of frontoethmoidal meningoencephaloceles: feasibility and outcome in 52 consecutive cases. Plast Reconstr Surg. 121(6):386e-95e, 2008
                                                                                          7. Lo BW et al: Clinical predictors of developmental outcome in patients with cephaloceles. J Neurosurg Pediatr. 2(4):254-7, 2008
                                                                                          8. Hedlund G: Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol. 36(7):647-62; quiz 726-7, 2006
                                                                                          9. Rahbar R et al: Nasal glioma and encephalocele: diagnosis and management. Laryngoscope. 113(12):2069-77, 2003
                                                                                          10. Rojvachiranonda N et al: Frontoethmoidal encephalomeningocele: new morphological findings and a new classification. J Craniofac Surg. 14(6):847-58, 2003
                                                                                          11. Mahapatra AK et al: Anterior encephaloceles: a study of 92 cases. Pediatr Neurosurg. 36(3):113-8, 2002
                                                                                          12. Holmes AD et al: Frontoethmoidal encephaloceles: reconstruction and refinements. J Craniofac Surg. 12(1):6-18, 2001
                                                                                          13. De Ponte FS et al: Surgical treatment of frontoethmoidal encephalocele: a case report. J Craniofac Surg. 11(4):342-5, 2000
                                                                                          14. Songur E et al: Management of frontoethmoidal (sincipital) encephalocele. J Craniofac Surg. 10(2):135-9, 1999
                                                                                          15. Boonvisut S et al: Morphologic study of 120 skull base defects in frontoethmoidal encephalomeningoceles. Plast Reconstr Surg. 101(7):1784-95, 1998
                                                                                          16. Turgut M et al: Congenital nasal encephalocele: a review of 35 cases. J Craniomaxillofac Surg. 23(1):1-5, 1995
                                                                                          17. Koch BL et al: Congenital malformations causing skull base changes. Neuroimaging Clin N Am. 4(3):479-98, 1994
                                                                                          18. Naidich TP et al: Cephaloceles and related malformations. AJNR Am J Neuroradiol. 13(2):655-90, 1992
                                                                                          19. Barkovich AJ et al: Congenital nasal masses: CT and MR imaging features in 16 cases. AJNR Am J Neuroradiol. 12(1):105-16, 1991
                                                                                          20. David DJ et al: Cephaloceles: classification, pathology, and management. World J Surg. 13(4):349-57, 1989
                                                                                          21. Vattoth S, Chapman PR. Anterior skull base. In: Watanabe K, et al., eds. Anatomy for Plastic Surgery of the Face, Head, and Neck.1st ed. New York: Thieme Publishers; 2016:13-9