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Cephalocele
Kevin R. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Definitions

              • Generic term for intracranial content protrusion through calvarial or skull base defect
                • Named for roof and floor of bone defect
                • Open or skin covered, dura attenuated or dehiscent
                • Congenital or post-traumatic
              • Cephalocele contents
                • Meningoencephalocele (encephalocele): Brain tissue, meninges, CSF
                • Meningocele: Meninges, CSF only
                • Atretic cephalocele: Cephalocele form fruste; dura, fibrous tissue, dysplastic brain tissue
                • Gliocele: Glial-lined cyst with CSF
              • Cephalocele types
                • Basal (10%)
                  • Midline basal cephalocele
                    • Sphenopharyngeal: Sphenoid body
                    • Sphenoethmoidal: Sphenoid, ethmoid bones
                    • Transethmoidal: Cribriform plate
                  • Lateral basal cephalocele
                    • Sphenomaxillary: Maxillary sinus, orbital fissure into pterygopalatine fossa
                    • Sphenoorbital: Sphenoid bone into orbit
                  • May include pituitary gland, hypothalamus, optic nerves/chiasm, anterior 3rd ventricle
                • Frontoethmoidal (sincipital) (10-15%)
                  • Midface, dorsum of nose, orbits, forehead
                  • Subtypes (may be mixed 10%)
                    • Frontonasal (40-60%): Foramen cecum, fonticulus frontalis into glabella
                    • Nasoethmoidal (30%): Foramen cecum into nasal cavity
                    • Nasoorbital: Maxilla, lacrimal bone into orbit
                • Nasopharyngeal (very uncommon)
                  • Occult cephalocele through ethmoid, sphenoid, or basiocciput → nasal cavity or pharynx
                • Occipital (75%)
                  • Occipitocervical: Occipital bone, foramen magnum, upper cervical posterior arches
                  • Low occipital: With foramen magnum
                  • High occipital: Without foramen magnum
                  • May include supratentorial &/or infratentorial structures in cephalocele
                • Parietal (10%)
                  • Usually associated with significant brain anomalies → poor prognosis
                  • Atretic parietal cephalocele subgroup much better prognosis
                • Temporal bone
                  • Inferior extension through middle ear/mastoid, petrous apex
                • Cranial vault
                  • Anterior fontanelle, interfrontal, lateral (coronal or lambdoid sutures), temporal, interparietal, posterior fontanelle

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Ultrasonographic Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Da Silva SL et al: Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele. J Neurosurg Pediatr. 15(4):392-8, 2015
                                        2. Kasprian GJ et al: Prenatal imaging of occipital encephaloceles. Fetal Diagn Ther. 37(3):241-8, 2015
                                        3. Rawal RB et al: Endoscopic sinonasal meningoencephalocele repair: a 13-year experience with stratification by defect and reconstruction type. Otolaryngol Head Neck Surg. 152(2):361-8, 2015
                                        4. Abele TA et al: Craniopharyngeal canal and its spectrum of pathology. AJNR Am J Neuroradiol. 35(4):772-7, 2014
                                        5. Dhirawani RB et al: Frontoethmoidal encephalocele: Case report and review on management. Ann Maxillofac Surg. 4(2):195-7, 2014
                                        6. Copp AJ et al: Neural tube defects: recent advances, unsolved questions, and controversies. Lancet Neurol. 12(8):799-810, 2013
                                        7. Tirumandas M et al: Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst. 29(5):739-44, 2013
                                        8. Sather MD et al: Large supra- and infra-tentorial occipital encephalocele encompassing posterior sagittal sinus and torcular Herophili. Childs Nerv Syst. 25(7):903-6, 2009
                                        9. Alkhalidi H et al: Spontaneous middle-ear encephalocele: report of two cases and brief review. Clin Neuropathol. 27(5):357-60, 2008
                                        10. Valencia MP et al: Congenital and acquired lesions of the nasal septum: a practical guide for differential diagnosis. Radiographics. 28(1):205-24; quiz 326, 2008
                                        11. Bui CJ et al: Institutional experience with cranial vault encephaloceles. J Neurosurg. 107(1 Suppl):22-5, 2007
                                        12. Köhrmann M et al: Nasal meningoencephalocele, an unusual cause for recurrent meningitis. Case report and review of the literature. J Neurol. 254(2):259-60, 2007
                                        13. Hedlund G: Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol. 36(7):647-62; quiz 726-7, 2006
                                        14. Sdano MT et al: Temporal bone encephaloceles. Curr Opin Otolaryngol Head Neck Surg. 13(5):287-9, 2005
                                        15. Hunt JA et al: encephaloceles. Plast Reconstr Surg. 2003 Aug;112(2):606-15; quiz 616,722. Review.
                                        16. Hoving EW: Nasal encephaloceles. Childs Nerv Syst. 16(10-11):702-6, 2000
                                        17. Lowe LH et al: Midface anomalies in children. Radiographics. 20(4):907-22; quiz 1106-7, 1112, 2000
                                        18. Barkovich AJ et al: Congenital nasal masses: CT and MR imaging features in 16 cases. AJNR Am J Neuroradiol. 12(1):105-16, 1991
                                        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

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Definitions

                                                    • Generic term for intracranial content protrusion through calvarial or skull base defect
                                                      • Named for roof and floor of bone defect
                                                      • Open or skin covered, dura attenuated or dehiscent
                                                      • Congenital or post-traumatic
                                                    • Cephalocele contents
                                                      • Meningoencephalocele (encephalocele): Brain tissue, meninges, CSF
                                                      • Meningocele: Meninges, CSF only
                                                      • Atretic cephalocele: Cephalocele form fruste; dura, fibrous tissue, dysplastic brain tissue
                                                      • Gliocele: Glial-lined cyst with CSF
                                                    • Cephalocele types
                                                      • Basal (10%)
                                                        • Midline basal cephalocele
                                                          • Sphenopharyngeal: Sphenoid body
                                                          • Sphenoethmoidal: Sphenoid, ethmoid bones
                                                          • Transethmoidal: Cribriform plate
                                                        • Lateral basal cephalocele
                                                          • Sphenomaxillary: Maxillary sinus, orbital fissure into pterygopalatine fossa
                                                          • Sphenoorbital: Sphenoid bone into orbit
                                                        • May include pituitary gland, hypothalamus, optic nerves/chiasm, anterior 3rd ventricle
                                                      • Frontoethmoidal (sincipital) (10-15%)
                                                        • Midface, dorsum of nose, orbits, forehead
                                                        • Subtypes (may be mixed 10%)
                                                          • Frontonasal (40-60%): Foramen cecum, fonticulus frontalis into glabella
                                                          • Nasoethmoidal (30%): Foramen cecum into nasal cavity
                                                          • Nasoorbital: Maxilla, lacrimal bone into orbit
                                                      • Nasopharyngeal (very uncommon)
                                                        • Occult cephalocele through ethmoid, sphenoid, or basiocciput → nasal cavity or pharynx
                                                      • Occipital (75%)
                                                        • Occipitocervical: Occipital bone, foramen magnum, upper cervical posterior arches
                                                        • Low occipital: With foramen magnum
                                                        • High occipital: Without foramen magnum
                                                        • May include supratentorial &/or infratentorial structures in cephalocele
                                                      • Parietal (10%)
                                                        • Usually associated with significant brain anomalies → poor prognosis
                                                        • Atretic parietal cephalocele subgroup much better prognosis
                                                      • Temporal bone
                                                        • Inferior extension through middle ear/mastoid, petrous apex
                                                      • Cranial vault
                                                        • Anterior fontanelle, interfrontal, lateral (coronal or lambdoid sutures), temporal, interparietal, posterior fontanelle

                                                  IMAGING

                                                  • General Features

                                                    • CT Findings

                                                      • MR Findings

                                                        • Ultrasonographic Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Da Silva SL et al: Risk factors for hydrocephalus and neurological deficit in children born with an encephalocele. J Neurosurg Pediatr. 15(4):392-8, 2015
                                                                              2. Kasprian GJ et al: Prenatal imaging of occipital encephaloceles. Fetal Diagn Ther. 37(3):241-8, 2015
                                                                              3. Rawal RB et al: Endoscopic sinonasal meningoencephalocele repair: a 13-year experience with stratification by defect and reconstruction type. Otolaryngol Head Neck Surg. 152(2):361-8, 2015
                                                                              4. Abele TA et al: Craniopharyngeal canal and its spectrum of pathology. AJNR Am J Neuroradiol. 35(4):772-7, 2014
                                                                              5. Dhirawani RB et al: Frontoethmoidal encephalocele: Case report and review on management. Ann Maxillofac Surg. 4(2):195-7, 2014
                                                                              6. Copp AJ et al: Neural tube defects: recent advances, unsolved questions, and controversies. Lancet Neurol. 12(8):799-810, 2013
                                                                              7. Tirumandas M et al: Nasal encephaloceles: a review of etiology, pathophysiology, clinical presentations, diagnosis, treatment, and complications. Childs Nerv Syst. 29(5):739-44, 2013
                                                                              8. Sather MD et al: Large supra- and infra-tentorial occipital encephalocele encompassing posterior sagittal sinus and torcular Herophili. Childs Nerv Syst. 25(7):903-6, 2009
                                                                              9. Alkhalidi H et al: Spontaneous middle-ear encephalocele: report of two cases and brief review. Clin Neuropathol. 27(5):357-60, 2008
                                                                              10. Valencia MP et al: Congenital and acquired lesions of the nasal septum: a practical guide for differential diagnosis. Radiographics. 28(1):205-24; quiz 326, 2008
                                                                              11. Bui CJ et al: Institutional experience with cranial vault encephaloceles. J Neurosurg. 107(1 Suppl):22-5, 2007
                                                                              12. Köhrmann M et al: Nasal meningoencephalocele, an unusual cause for recurrent meningitis. Case report and review of the literature. J Neurol. 254(2):259-60, 2007
                                                                              13. Hedlund G: Congenital frontonasal masses: developmental anatomy, malformations, and MR imaging. Pediatr Radiol. 36(7):647-62; quiz 726-7, 2006
                                                                              14. Sdano MT et al: Temporal bone encephaloceles. Curr Opin Otolaryngol Head Neck Surg. 13(5):287-9, 2005
                                                                              15. Hunt JA et al: encephaloceles. Plast Reconstr Surg. 2003 Aug;112(2):606-15; quiz 616,722. Review.
                                                                              16. Hoving EW: Nasal encephaloceles. Childs Nerv Syst. 16(10-11):702-6, 2000
                                                                              17. Lowe LH et al: Midface anomalies in children. Radiographics. 20(4):907-22; quiz 1106-7, 1112, 2000
                                                                              18. Barkovich AJ et al: Congenital nasal masses: CT and MR imaging features in 16 cases. AJNR Am J Neuroradiol. 12(1):105-16, 1991