Extracranial extension of intracranial contents through defect in skull; categorized by contents & location
Congenital is more common than acquired
Congenital forms are typically sporadic, though chromosomal (trisomy 13 & 18), syndromic (Meckel-Gruber), & disruptive (amniotic band) etiologies have been described
Acquired forms may be due to trauma, surgery, infection, or Gorham-Stout
Contents
Cephalocele: Broad term for any focal extracranial extension of intracranial contents
Atretic cephalocele: Meningeal & neuroglial elements retain fibrous connection to intracranial compartment, often without significant protrusion through defect
Locations
Occipital: Most common location, up to 80% of cases
Supra- &/or infratentorial structures in cephalocele, including tentorium cerebelli & dural venous sinuses
Original Chiari 3 description: Upper cervical spinal dysraphism with protruding encephalocele containing herniated hindbrain
Associated intracranial findings of Chiari malformation, including "lemon" calvarium, callosal hypogenesis, tectal beaking
Frontoethmoidal/sincipital/anterior: 15% (↑ in Southeast Asia)
Nasoethmoidal: Most common form; defect between nasal bone & nasal cartilage (through foramen cecum)
Nasofrontal/frontonasal: Defect between frontal & nasal bones (through fonticulus frontalis)
Nasoorbital: Least common form; defect at junction of maxilla (anterior) & lacrimal bone with lamina papyracea of ethmoid bone (posterior)
Parietal: 10% (↑ in Japan at 38%)
Atretic parietal cephalocele is most common
Midline posterior parietal scalp mass over small bony defect, elevation of falx/tentorial junction, persistent falcine venous sinus, fibrous tract splits superior sagittal sinus
Majority have benign clinical course
Parietal meningoencephaloceles are uncommon
Usually associated with significant brain anomalies → poor prognosis
Basal/nasopharyngeal: Up to 10% of cases
Transethmoidal: Defect in ethmoids only with mass in anterior nasal cavity
Transsphenoidal/sphenopharyngeal: Defect in sphenoid bone only; usually extends into nasopharynx but can also be confined to sphenoid sinuses
Sphenoethmoidal: Through sphenoid & ethmoid bones with mass in posterior nasal cavity
Sphenoorbital: Defect through superior orbital fissure with unilateral exophthalmos; rare cases associated with neurofibromatosis type 1
Sphenomaxillary: Very rare, 1-2 case reports
Remaining calvarium: Rare
Interfrontal
Interparietal
Anterior fontanelle
Posterior fontanelle
Temporal (lateral)
Mastoid &/or petrous temporal bone: Rare
Cavum trigeminale: Rare
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
Reporting Tips
Selected References
Khodarahmi I et al: Imaging spectrum of calvarial abnormalities. Radiographics. 41(4):1144-63, 2021
Nagaraj UD et al: Prenatal evaluation of the Sakoda complex. Pediatr Radiol. 49(13):1843-7, 2019
Yucetas SC et al: A retrospective analysis of neonatal encephalocele predisposing factors and outcomes. Pediatr Neurosurg. 52(2):73-6, 2017
Demir MK et al: Atretic cephaloceles: a comprehensive analysis of historical cohort. Childs Nerv Syst. 32(12):2327-37, 2016
Boppel T et al: Excavating Meckel's cave: cavum-trigeminale-cephaloceles (CTCs). J Neuroradiol. 42(3):156-61, 2015
Morone PJ et al: Temporal lobe encephaloceles: a potentially curable cause of seizures. Otol Neurotol. 36(8):1439-42, 2015
Copp AJ et al: Neural tube defects: recent advances, unsolved questions, and controversies. Lancet Neurol. 12(8):799-810, 2013
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
Barkovich et al: Pediatric Neuroimaging: 5th edition. Lippincott Williams & Wilkins. 501-21, 2012
Bui CJ et al: Institutional experience with cranial vault encephaloceles. J Neurosurg. 107(1 Suppl):22-5, 2007
Rowland CA et al: Are encephaloceles neural tube defects? Pediatrics. 118(3):916-23, 2006
Sdano MT et al: Temporal bone encephaloceles. Curr Opin Otolaryngol Head Neck Surg. 13(5):287-9, 2005
Moron FE et al: Lumps and bumps on the head in children: use of CT and MR imaging in solving the clinical diagnostic dilemma. Radiographics. 24(6):1655-74, 2004
Lowe LH et al: Midface anomalies in children. Radiographics. 20(4):907-22; quiz 1106-7, 1112, 2000
Patterson RJ, et al: Atretic parietal cephaloceles revisited: an enlarging clinical and imaging spectrum? AJNR 19:791-5, 1998
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
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TERMINOLOGY
Definitions
Extracranial extension of intracranial contents through defect in skull; categorized by contents & location
Congenital is more common than acquired
Congenital forms are typically sporadic, though chromosomal (trisomy 13 & 18), syndromic (Meckel-Gruber), & disruptive (amniotic band) etiologies have been described
Acquired forms may be due to trauma, surgery, infection, or Gorham-Stout
Contents
Cephalocele: Broad term for any focal extracranial extension of intracranial contents
Atretic cephalocele: Meningeal & neuroglial elements retain fibrous connection to intracranial compartment, often without significant protrusion through defect
Locations
Occipital: Most common location, up to 80% of cases
Supra- &/or infratentorial structures in cephalocele, including tentorium cerebelli & dural venous sinuses
Original Chiari 3 description: Upper cervical spinal dysraphism with protruding encephalocele containing herniated hindbrain
Associated intracranial findings of Chiari malformation, including "lemon" calvarium, callosal hypogenesis, tectal beaking
Frontoethmoidal/sincipital/anterior: 15% (↑ in Southeast Asia)
Nasoethmoidal: Most common form; defect between nasal bone & nasal cartilage (through foramen cecum)
Nasofrontal/frontonasal: Defect between frontal & nasal bones (through fonticulus frontalis)
Nasoorbital: Least common form; defect at junction of maxilla (anterior) & lacrimal bone with lamina papyracea of ethmoid bone (posterior)
Parietal: 10% (↑ in Japan at 38%)
Atretic parietal cephalocele is most common
Midline posterior parietal scalp mass over small bony defect, elevation of falx/tentorial junction, persistent falcine venous sinus, fibrous tract splits superior sagittal sinus
Majority have benign clinical course
Parietal meningoencephaloceles are uncommon
Usually associated with significant brain anomalies → poor prognosis
Basal/nasopharyngeal: Up to 10% of cases
Transethmoidal: Defect in ethmoids only with mass in anterior nasal cavity
Transsphenoidal/sphenopharyngeal: Defect in sphenoid bone only; usually extends into nasopharynx but can also be confined to sphenoid sinuses
Sphenoethmoidal: Through sphenoid & ethmoid bones with mass in posterior nasal cavity
Sphenoorbital: Defect through superior orbital fissure with unilateral exophthalmos; rare cases associated with neurofibromatosis type 1
Sphenomaxillary: Very rare, 1-2 case reports
Remaining calvarium: Rare
Interfrontal
Interparietal
Anterior fontanelle
Posterior fontanelle
Temporal (lateral)
Mastoid &/or petrous temporal bone: Rare
Cavum trigeminale: Rare
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
Reporting Tips
Selected References
Khodarahmi I et al: Imaging spectrum of calvarial abnormalities. Radiographics. 41(4):1144-63, 2021
Nagaraj UD et al: Prenatal evaluation of the Sakoda complex. Pediatr Radiol. 49(13):1843-7, 2019
Yucetas SC et al: A retrospective analysis of neonatal encephalocele predisposing factors and outcomes. Pediatr Neurosurg. 52(2):73-6, 2017
Demir MK et al: Atretic cephaloceles: a comprehensive analysis of historical cohort. Childs Nerv Syst. 32(12):2327-37, 2016
Boppel T et al: Excavating Meckel's cave: cavum-trigeminale-cephaloceles (CTCs). J Neuroradiol. 42(3):156-61, 2015
Morone PJ et al: Temporal lobe encephaloceles: a potentially curable cause of seizures. Otol Neurotol. 36(8):1439-42, 2015
Copp AJ et al: Neural tube defects: recent advances, unsolved questions, and controversies. Lancet Neurol. 12(8):799-810, 2013
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
Barkovich et al: Pediatric Neuroimaging: 5th edition. Lippincott Williams & Wilkins. 501-21, 2012
Bui CJ et al: Institutional experience with cranial vault encephaloceles. J Neurosurg. 107(1 Suppl):22-5, 2007
Rowland CA et al: Are encephaloceles neural tube defects? Pediatrics. 118(3):916-23, 2006
Sdano MT et al: Temporal bone encephaloceles. Curr Opin Otolaryngol Head Neck Surg. 13(5):287-9, 2005
Moron FE et al: Lumps and bumps on the head in children: use of CT and MR imaging in solving the clinical diagnostic dilemma. Radiographics. 24(6):1655-74, 2004
Lowe LH et al: Midface anomalies in children. Radiographics. 20(4):907-22; quiz 1106-7, 1112, 2000
Patterson RJ, et al: Atretic parietal cephaloceles revisited: an enlarging clinical and imaging spectrum? AJNR 19:791-5, 1998
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