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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
- Nasopharyngeal
- Basal cephalocele = congenital extracranial herniation of meninges, CSF ± brain tissue through mesodermal defect in sphenoid, ethmoid, or basiocciput
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
- Pavanello M et al: A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management. Neurol Sci. ePub, 2021
- Thompson HM et al: Current management of congenital anterior cranial base encephaloceles. Int J Pediatr Otorhinolaryngol. 131:109868, 2020
- Keshri AK et al: Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci. 11(1):42-5, 2016
- Abele TA et al: Craniopharyngeal canal and its spectrum of pathology. AJNR Am J Neuroradiol. 35(4):772-7, 2014
- Morabito R et al: Pharyngeal enterogenous cyst associated with canalis basilaris medianus in a newborn. Pediatr Radiol. 43(4):512-5, 2013
- 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
- Borges A: Imaging of the central skull base. Neuroimaging Clin N Am. 19(4):669-96, 2009
- Castelnuovo P et al: Endoscopic endonasal management of encephaloceles in children: an eight-year experience. Int J Pediatr Otorhinolaryngol. 73(8):1132-6, 2009
- 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
- Lee TJ et al: Endoscopic treatment of traumatic basal encephaloceles: a report of 8 cases. J Neurosurg. 108(4):729-35, 2008
- 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
- Gupta DK et al: Transethmoidal transpharyngeal nasal encephalocele: neuroimaging. Pediatr Neurosurg. 42(5):335-7, 2006
- Kizilkilic O et al: Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol. 26(1):65-7, 2005
- 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
- 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
- Daniilidis J et al: Intrasphenoidal encephalocele and spontaneous CSF rhinorrhoea. Rhinology. 37(4):186-9, 1999
- Schick B et al: Occult malformations of the skull base. Arch Otolaryngol Head Neck Surg. 123(1):77-80, 1997
- Madeline LA et al: Postnatal development of the central skull base: normal variants. Radiology. 196(3):757-63, 1995
- Koch BL et al: Congenital malformations causing skull base changes. Neuroimaging Clin N Am. 4(3):479-98, 1994
- Naidich TP et al: Cephaloceles and related malformations. AJNR Am J Neuroradiol. 13(2):655-90, 1992
- Cohen R et al: Epignathic teratoma associated with craniopharyngeal canal. AJNR Am J Neuroradiol. 10(3):652, 1989
- Currarino G: Canalis basilaris medianus and related defects of the basiocciput. AJNR Am J Neuroradiol. 9(1):208-11, 1988
- Nager GT: Cephaloceles. Laryngoscope. 97(1):77-84, 1987
- Currarino G et al: Transsphenoidal canal (large craniopharyngeal canal) and its pathologic implications. AJNR Am J Neuroradiol. 6(1):39-43, 1985
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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
- Nasopharyngeal
- Basal cephalocele = congenital extracranial herniation of meninges, CSF ± brain tissue through mesodermal defect in sphenoid, ethmoid, or basiocciput
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
- Pavanello M et al: A rare triad of morning glory disc anomaly, moyamoya vasculopathy, and transsphenoidal cephalocele: pathophysiological considerations and surgical management. Neurol Sci. ePub, 2021
- Thompson HM et al: Current management of congenital anterior cranial base encephaloceles. Int J Pediatr Otorhinolaryngol. 131:109868, 2020
- Keshri AK et al: Transnasal endoscopic repair of pediatric meningoencephalocele. J Pediatr Neurosci. 11(1):42-5, 2016
- Abele TA et al: Craniopharyngeal canal and its spectrum of pathology. AJNR Am J Neuroradiol. 35(4):772-7, 2014
- Morabito R et al: Pharyngeal enterogenous cyst associated with canalis basilaris medianus in a newborn. Pediatr Radiol. 43(4):512-5, 2013
- 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
- Borges A: Imaging of the central skull base. Neuroimaging Clin N Am. 19(4):669-96, 2009
- Castelnuovo P et al: Endoscopic endonasal management of encephaloceles in children: an eight-year experience. Int J Pediatr Otorhinolaryngol. 73(8):1132-6, 2009
- 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
- Lee TJ et al: Endoscopic treatment of traumatic basal encephaloceles: a report of 8 cases. J Neurosurg. 108(4):729-35, 2008
- 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
- Gupta DK et al: Transethmoidal transpharyngeal nasal encephalocele: neuroimaging. Pediatr Neurosurg. 42(5):335-7, 2006
- Kizilkilic O et al: Hypothalamic hamartoma associated with a craniopharyngeal canal. AJNR Am J Neuroradiol. 26(1):65-7, 2005
- 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
- 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
- Daniilidis J et al: Intrasphenoidal encephalocele and spontaneous CSF rhinorrhoea. Rhinology. 37(4):186-9, 1999
- Schick B et al: Occult malformations of the skull base. Arch Otolaryngol Head Neck Surg. 123(1):77-80, 1997
- Madeline LA et al: Postnatal development of the central skull base: normal variants. Radiology. 196(3):757-63, 1995
- Koch BL et al: Congenital malformations causing skull base changes. Neuroimaging Clin N Am. 4(3):479-98, 1994
- Naidich TP et al: Cephaloceles and related malformations. AJNR Am J Neuroradiol. 13(2):655-90, 1992
- Cohen R et al: Epignathic teratoma associated with craniopharyngeal canal. AJNR Am J Neuroradiol. 10(3):652, 1989
- Currarino G: Canalis basilaris medianus and related defects of the basiocciput. AJNR Am J Neuroradiol. 9(1):208-11, 1988
- Nager GT: Cephaloceles. Laryngoscope. 97(1):77-84, 1987
- Currarino G et al: Transsphenoidal canal (large craniopharyngeal canal) and its pathologic implications. AJNR Am J Neuroradiol. 6(1):39-43, 1985
