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Skull Base Cephalocele
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Basal cephaloceles
            • "Occult" cephaloceles
          • Definitions

            • Basal cephalocele = congenital extracranial herniation of meninges, CSF ± brain tissue through mesodermal defect in sphenoid, ethmoid, or basiocciput
              • Nasopharyngeal
                • Transethmoid
                • Sphenoethmoid
                • Sphenonasopharyngeal = transsphenoid (large craniopharyngeal canal)
                • Basioccipital-nasopharyngeal (transbasioccipital)
              • Sphenoorbital
              • Sphenomaxillary

          IMAGING

          • General Features

            • Imaging Recommendations

              • CT Findings

                • MR Findings

                  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

                                      • Reporting Tips

                                        Selected References

                                        1. Keshri AK et al: Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci. 11(1):42-5, 2016
                                        2. Abele TA et al: Craniopharyngeal canal and its spectrum of pathology. AJNR Am J Neuroradiol. 35(4):772-7, 2014
                                        3. Morabito R et al: Pharyngeal enterogenous cyst associated with canalis basilaris medianus in a newborn. Pediatr Radiol. 43(4):512-5, 2013
                                        4. Lohman BD et al: Not the typical Tornwaldt's cyst this time? A nasopharyngeal cyst associated with canalis basilaris medianus. Br J Radiol. 84(1005):e169-71, 2011
                                        5. Borges A: Imaging of the central skull base. Neuroimaging Clin N Am. 19(4):669-96, 2009
                                        6. Castelnuovo P et al: Endoscopic endonasal management of encephaloceles in children: an eight-year experience. Int J Pediatr Otorhinolaryngol. 73(8):1132-6, 2009
                                        7. Lesavoy MA et al: Nasopharyngeal encephalocele: report of transcranial and transpalatal repair with a 25-year follow-up. J Craniofac Surg. 20(6):2251-6, 2009
                                        8. Lee TJ et al: Endoscopic treatment of traumatic basal encephaloceles: a report of 8 cases. J Neurosurg. 108(4):729-35, 2008
                                        9. Schuknecht B et al: Nontraumatic skull base defects with spontaneous CSF rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients. AJNR Am J Neuroradiol. 29(3):542-9, 2008
                                        10. Gupta DK et al: Transethmoidal transpharyngeal nasal encephalocele: neuroimaging. Pediatr Neurosurg. 42(5):335-7, 2006
                                        11. Kizilkilic O et al: Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol. 26(1):65-7, 2005
                                        12. Ekinci G et al: Transsphenoidal (large craniopharyngeal) canal associated with a normally functioning pituitary gland and nasopharyngeal extension, hyperprolactinemia, and hypothalamic hamartoma. AJR Am J Roentgenol. 180(1):76-7, 2003
                                        13. Blustajn J et al: Dysgenesis of the internal carotid artery associated with transsphenoidal encephalocele: a neural crest syndrome? AJNR Am J Neuroradiol. 20(6):1154-7, 1999
                                        14. Daniilidis J et al: Intrasphenoidal encephalocele and spontaneous CSF rhinorrhoea. Rhinology. 37(4):186-9, 1999
                                        15. Schick B et al: Occult malformations of the skull base. Arch Otolaryngol Head Neck Surg. 123(1):77-80, 1997
                                        16. Madeline LA et al: Postnatal development of the central skull base: normal variants. Radiology. 196(3):757-63, 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. Cohen R et al: Epignathic teratoma associated with craniopharyngeal canal. AJNR Am J Neuroradiol. 10(3):652, 1989
                                        20. Currarino G: Canalis basilaris medianus and related defects of the basiocciput. AJNR Am J Neuroradiol. 9(1):208-11, 1988
                                        21. Nager GT: Cephaloceles. Laryngoscope. 97(1):77-84, 1987
                                        22. Currarino G et al: Transsphenoidal canal (large craniopharyngeal canal) and its pathologic implications. AJNR Am J Neuroradiol. 6(1):39-43, 1985
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Basal cephaloceles
                                                  • "Occult" cephaloceles
                                                • Definitions

                                                  • Basal cephalocele = congenital extracranial herniation of meninges, CSF ± brain tissue through mesodermal defect in sphenoid, ethmoid, or basiocciput
                                                    • Nasopharyngeal
                                                      • Transethmoid
                                                      • Sphenoethmoid
                                                      • Sphenonasopharyngeal = transsphenoid (large craniopharyngeal canal)
                                                      • Basioccipital-nasopharyngeal (transbasioccipital)
                                                    • Sphenoorbital
                                                    • Sphenomaxillary

                                                IMAGING

                                                • General Features

                                                  • Imaging Recommendations

                                                    • CT Findings

                                                      • MR Findings

                                                        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

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Keshri AK et al: Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci. 11(1):42-5, 2016
                                                                              2. Abele TA et al: Craniopharyngeal canal and its spectrum of pathology. AJNR Am J Neuroradiol. 35(4):772-7, 2014
                                                                              3. Morabito R et al: Pharyngeal enterogenous cyst associated with canalis basilaris medianus in a newborn. Pediatr Radiol. 43(4):512-5, 2013
                                                                              4. Lohman BD et al: Not the typical Tornwaldt's cyst this time? A nasopharyngeal cyst associated with canalis basilaris medianus. Br J Radiol. 84(1005):e169-71, 2011
                                                                              5. Borges A: Imaging of the central skull base. Neuroimaging Clin N Am. 19(4):669-96, 2009
                                                                              6. Castelnuovo P et al: Endoscopic endonasal management of encephaloceles in children: an eight-year experience. Int J Pediatr Otorhinolaryngol. 73(8):1132-6, 2009
                                                                              7. Lesavoy MA et al: Nasopharyngeal encephalocele: report of transcranial and transpalatal repair with a 25-year follow-up. J Craniofac Surg. 20(6):2251-6, 2009
                                                                              8. Lee TJ et al: Endoscopic treatment of traumatic basal encephaloceles: a report of 8 cases. J Neurosurg. 108(4):729-35, 2008
                                                                              9. Schuknecht B et al: Nontraumatic skull base defects with spontaneous CSF rhinorrhea and arachnoid herniation: imaging findings and correlation with endoscopic sinus surgery in 27 patients. AJNR Am J Neuroradiol. 29(3):542-9, 2008
                                                                              10. Gupta DK et al: Transethmoidal transpharyngeal nasal encephalocele: neuroimaging. Pediatr Neurosurg. 42(5):335-7, 2006
                                                                              11. Kizilkilic O et al: Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol. 26(1):65-7, 2005
                                                                              12. Ekinci G et al: Transsphenoidal (large craniopharyngeal) canal associated with a normally functioning pituitary gland and nasopharyngeal extension, hyperprolactinemia, and hypothalamic hamartoma. AJR Am J Roentgenol. 180(1):76-7, 2003
                                                                              13. Blustajn J et al: Dysgenesis of the internal carotid artery associated with transsphenoidal encephalocele: a neural crest syndrome? AJNR Am J Neuroradiol. 20(6):1154-7, 1999
                                                                              14. Daniilidis J et al: Intrasphenoidal encephalocele and spontaneous CSF rhinorrhoea. Rhinology. 37(4):186-9, 1999
                                                                              15. Schick B et al: Occult malformations of the skull base. Arch Otolaryngol Head Neck Surg. 123(1):77-80, 1997
                                                                              16. Madeline LA et al: Postnatal development of the central skull base: normal variants. Radiology. 196(3):757-63, 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. Cohen R et al: Epignathic teratoma associated with craniopharyngeal canal. AJNR Am J Neuroradiol. 10(3):652, 1989
                                                                              20. Currarino G: Canalis basilaris medianus and related defects of the basiocciput. AJNR Am J Neuroradiol. 9(1):208-11, 1988
                                                                              21. Nager GT: Cephaloceles. Laryngoscope. 97(1):77-84, 1987
                                                                              22. Currarino G et al: Transsphenoidal canal (large craniopharyngeal canal) and its pathologic implications. AJNR Am J Neuroradiol. 6(1):39-43, 1985