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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Enlarged parietal foramen (EPF), aplasia cutis congenita (ACC)
Definitions
- Parietal fissure (common)
- Small, residual, incomplete medial parietal bone suture
- Parietal foramina (common; 60-70% of normal skulls)
- Nonossification of medial parietal bone embryonal rest
- Emissary vein portal between superior sagittal sinus and extracranial scalp veins
- Enlarged parietal foramina (rare; prevalence 1:15,000-25,000)
- Delayed/incomplete ossification of membranous parietal bone → round or oval parietal bone defects
- Calvarial defect may be large, palpable
- Sinus pericranii
- Abnormal communication between intracranial, extracranial venous systems through calvarial defect
- Soft (often red or blue) scalp mass near superior sagittal or transverse dural sinuses
- ↑ size with ↑ intracranial pressure (ICP) (Valsalva, dependent positioning)
- Craniolacunia (venous lakes)
- Patulous calvarial diploic veins
- Irregular, geographic, well-demarcated contour
- Variable size and number
- Arachnoid (pacchionian) granulations
- Located within 3 cm of superior sagittal sinus
- Often multiple; irregular contour
- Abnormally large fontanelle
- Secondary to ↑ ICP (suture spreading) or skeletal dysplasia
- Search for ventriculomegaly, skeletal anomalies
- Cleidocranial dysplasia (uncommon)
- Defective membranous and endochondral bone formation → delayed skull ossification
- Enlarged sagittal and metopic sutures, wide anterior and posterior fontanelles, broad cranial diameter, multiple wormian bones along lambdoid sutures
- Cranium bifidum occultum (cleft skull)
- Delayed ossification of parietal bones → large midline skull defects
- Progressive parietal bone ossification fills defects; may persist as parietal foramina
- Cranium bifidum and parietal foramina may be continuum differentiated by time of detection
- Persistence in adulthood rare (< 1%)
- Brain covered by dura, intact scalp
- Amniotic band syndrome
- Occurs when inner amnion membrane ruptures or tears without disruption of outer chorion
- Strands of amniotic sac (bands) from ruptured amnion entangle digits, limbs, or other fetal parts
- ACC (uncommon)
- Congenital skin malformation; may have underlying skull defect
- Skin defects most frequent on midline scalp > trunk, face, limbs
- Distribution usually symmetrical
- Acalvaria (rare)
- Absent superior osseous cranial vault, dura mater
- Normal skull base, facial bones, brain (usually)
- Acrania (rare)
- Partial or complete absence of cranial vault bones, abnormal cerebral hemisphere development
- Parietal fissure (common)
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Jirapinyo C et al: Cleidocranial dysplasia: management of the multiple craniofacial and skeletal anomalies. J Craniofac Surg. ePub, 2020
- Pons Escoda A et al: Imaging of skull vault tumors in adults. Insights Imaging. 11(1):23, 2020
- Kang S et al: Pulsating lid: a rare presentation of sinus pericranii. Can J Ophthalmol. ePub, 2019
- Alalade AF et al: Fossa navicularis in a pediatric patient: anatomical skull base variant with clinical implications. J Neurosurg Pediatr. 1-5, 2018
- Flouty OE et al: Infantile cranial fasciitis: case-based review and operative technique. Childs Nerv Syst. 33(6):899-908, 2017
- Sanders FH et al: Extremely large sinus pericranii with involvement of the torcular and associated with Crouzon's syndrome. Childs Nerv Syst. 33(9):1445-9, 2017
- Singh I et al: Growing skull fractures: guidelines for early diagnosis and surgical management. Childs Nerv Syst. 32(6):1117-22, 2016
- Bouali S et al: Clinical and imaging findings in a rare case of sinus pericranii. Childs Nerv Syst. 31(9):1429-32, 2015
- Pavanello M et al: Sinus pericranii: diagnosis and management in 21 pediatric patients. J Neurosurg Pediatr. 15(1):60-70, 2015
- Ramdurg SR et al: Pediatric encephaloceles: a series of 20 cases over a period of 3 years. J Pediatr Neurosci. 10(4):317-20, 2015
- Hawasli AH et al: Acalvaria. J Neurosurg Pediatr. 14(2):200-2, 2014
- Singh S et al: Cleidocranial dysplasia: a case report illustrating diagnostic clinical and radiological findings. J Clin Diagn Res. 8(6):ZD19-20, 2014
- Griessenauer CJ et al: Enlarged parietal foramina: a review of genetics, prognosis, radiology, and treatment. Childs Nerv Syst. 29(4):543-7, 2013
- Lee KE et al: RUNX2 mutations in cleidocranial dysplasia. Genet Mol Res. 12(4):4567-74, 2013
- Vij R et al: Cleidocranial dysplasia: complete clinical, radiological and histological profiles. BMJ Case Rep. 2013
- Kayserili H et al: Mild nasal malformations and parietal foramina caused by homozygous ALX4 mutations. Am J Med Genet A. 158A(1):236-44, 2012
- Vedajallam S et al: Cranium bifidum occultum. Pediatr Neurosurg. 48(4):261-3, 2012
- Cardoso BM et al: Cleidocranial dysplasia with severe parietal bone dysplasia: a new (p.Val124Serfs) RUNX2 mutation. Clin Dysmorphol. 19(3):150-2, 2010
- Chung HY et al: Enlarged parietal foramina: findings on prenatal ultrasound and magnetic resonance imaging. Ultrasound Obstet Gynecol. 36(4):521-2, 2010
- Celik SE et al: Complete cranium bifidum without scalp abnormality. Case report. J Neurosurg Pediatrics. 1(3):258-60, 2008
- Mavrogiannis LA et al: Enlarged parietal foramina caused by mutations in the homeobox genes ALX4 and MSX2: from genotype to phenotype. Eur J Hum Genet. 14(2):151-8, 2006
- Mupparapu M et al: Hereditary cranium bifidum persisting as enlarged parietal foramina (Catlin marks) on cephalometric radiographs. Am J Orthod Dentofacial Orthop. 129(6):825-8, 2006
- Bianca S et al: Prenatal and postnatal findings of acrania. Arch Gynecol Obstet. 271(3):256-8, 2005
- Perlyn CA et al: Congenital scalp and calvarial deficiencies: principles for classification and surgical management. Plast Reconstr Surg. 115(4):1129-41, 2005
- Glass RB et al: The infant skull: a vault of information. Radiographics. 24(2):507-22, 2004
- Amaral L et al: MR imaging for evaluation of lesions of the cranial vault: a pictorial essay. Arq Neuropsiquiatr. 61(3A):521-32, 2003
- de Heer IM et al: Parietal bone agenesis and associated multiple congenital anomalies. J Craniofac Surg. 14(2):192-6, 2003
- Kortesis B et al: Surgical management of foramina parietalia permagna. J Craniofac Surg. 14(4):538-44, 2003
- Reimao R et al: Frontal foramina, Chiari II malformation, and hydrocephalus in a female. Pediatr Neurol. 29(4):341-4, 2003
- Tubbs RS et al: Parietal foramina are not synonymous with giant parietal foramina. Pediatr Neurosurg. 39(4):216-7, 2003
- Donati V et al: Reparation of a severe case of aplasia cutis congenita with engineered skin. Biol Neonate. 80(4):273-6, 2001
- Chandran S et al: Fetal acalvaria with amniotic band syndrome. Arch Dis Child Fetal Neonatal Ed. 82(1):F11-3, 2000
- Reddy AT et al: Enlarged parietal foramina: association with cerebral venous and cortical anomalies. Neurology. 54(5):1175-8, 2000
- Wilkie AO et al: Functional haploinsufficiency of the human homeobox gene MSX2 causes defects in skull ossification. Nat Genet. 24(4):387-90, 2000
- Kaplan SB et al: Radiographic manifestations of congenital anomalies of the skull. Radiol Clin North Am. 29(2):195-218, 1991
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
Diagnostic Checklist
TERMINOLOGY
Abbreviations
- Enlarged parietal foramen (EPF), aplasia cutis congenita (ACC)
Definitions
- Parietal fissure (common)
- Small, residual, incomplete medial parietal bone suture
- Parietal foramina (common; 60-70% of normal skulls)
- Nonossification of medial parietal bone embryonal rest
- Emissary vein portal between superior sagittal sinus and extracranial scalp veins
- Enlarged parietal foramina (rare; prevalence 1:15,000-25,000)
- Delayed/incomplete ossification of membranous parietal bone → round or oval parietal bone defects
- Calvarial defect may be large, palpable
- Sinus pericranii
- Abnormal communication between intracranial, extracranial venous systems through calvarial defect
- Soft (often red or blue) scalp mass near superior sagittal or transverse dural sinuses
- ↑ size with ↑ intracranial pressure (ICP) (Valsalva, dependent positioning)
- Craniolacunia (venous lakes)
- Patulous calvarial diploic veins
- Irregular, geographic, well-demarcated contour
- Variable size and number
- Arachnoid (pacchionian) granulations
- Located within 3 cm of superior sagittal sinus
- Often multiple; irregular contour
- Abnormally large fontanelle
- Secondary to ↑ ICP (suture spreading) or skeletal dysplasia
- Search for ventriculomegaly, skeletal anomalies
- Cleidocranial dysplasia (uncommon)
- Defective membranous and endochondral bone formation → delayed skull ossification
- Enlarged sagittal and metopic sutures, wide anterior and posterior fontanelles, broad cranial diameter, multiple wormian bones along lambdoid sutures
- Cranium bifidum occultum (cleft skull)
- Delayed ossification of parietal bones → large midline skull defects
- Progressive parietal bone ossification fills defects; may persist as parietal foramina
- Cranium bifidum and parietal foramina may be continuum differentiated by time of detection
- Persistence in adulthood rare (< 1%)
- Brain covered by dura, intact scalp
- Amniotic band syndrome
- Occurs when inner amnion membrane ruptures or tears without disruption of outer chorion
- Strands of amniotic sac (bands) from ruptured amnion entangle digits, limbs, or other fetal parts
- ACC (uncommon)
- Congenital skin malformation; may have underlying skull defect
- Skin defects most frequent on midline scalp > trunk, face, limbs
- Distribution usually symmetrical
- Acalvaria (rare)
- Absent superior osseous cranial vault, dura mater
- Normal skull base, facial bones, brain (usually)
- Acrania (rare)
- Partial or complete absence of cranial vault bones, abnormal cerebral hemisphere development
- Parietal fissure (common)
IMAGING
General Features
CT Findings
MR Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Jirapinyo C et al: Cleidocranial dysplasia: management of the multiple craniofacial and skeletal anomalies. J Craniofac Surg. ePub, 2020
- Pons Escoda A et al: Imaging of skull vault tumors in adults. Insights Imaging. 11(1):23, 2020
- Kang S et al: Pulsating lid: a rare presentation of sinus pericranii. Can J Ophthalmol. ePub, 2019
- Alalade AF et al: Fossa navicularis in a pediatric patient: anatomical skull base variant with clinical implications. J Neurosurg Pediatr. 1-5, 2018
- Flouty OE et al: Infantile cranial fasciitis: case-based review and operative technique. Childs Nerv Syst. 33(6):899-908, 2017
- Sanders FH et al: Extremely large sinus pericranii with involvement of the torcular and associated with Crouzon's syndrome. Childs Nerv Syst. 33(9):1445-9, 2017
- Singh I et al: Growing skull fractures: guidelines for early diagnosis and surgical management. Childs Nerv Syst. 32(6):1117-22, 2016
- Bouali S et al: Clinical and imaging findings in a rare case of sinus pericranii. Childs Nerv Syst. 31(9):1429-32, 2015
- Pavanello M et al: Sinus pericranii: diagnosis and management in 21 pediatric patients. J Neurosurg Pediatr. 15(1):60-70, 2015
- Ramdurg SR et al: Pediatric encephaloceles: a series of 20 cases over a period of 3 years. J Pediatr Neurosci. 10(4):317-20, 2015
- Hawasli AH et al: Acalvaria. J Neurosurg Pediatr. 14(2):200-2, 2014
- Singh S et al: Cleidocranial dysplasia: a case report illustrating diagnostic clinical and radiological findings. J Clin Diagn Res. 8(6):ZD19-20, 2014
- Griessenauer CJ et al: Enlarged parietal foramina: a review of genetics, prognosis, radiology, and treatment. Childs Nerv Syst. 29(4):543-7, 2013
- Lee KE et al: RUNX2 mutations in cleidocranial dysplasia. Genet Mol Res. 12(4):4567-74, 2013
- Vij R et al: Cleidocranial dysplasia: complete clinical, radiological and histological profiles. BMJ Case Rep. 2013
- Kayserili H et al: Mild nasal malformations and parietal foramina caused by homozygous ALX4 mutations. Am J Med Genet A. 158A(1):236-44, 2012
- Vedajallam S et al: Cranium bifidum occultum. Pediatr Neurosurg. 48(4):261-3, 2012
- Cardoso BM et al: Cleidocranial dysplasia with severe parietal bone dysplasia: a new (p.Val124Serfs) RUNX2 mutation. Clin Dysmorphol. 19(3):150-2, 2010
- Chung HY et al: Enlarged parietal foramina: findings on prenatal ultrasound and magnetic resonance imaging. Ultrasound Obstet Gynecol. 36(4):521-2, 2010
- Celik SE et al: Complete cranium bifidum without scalp abnormality. Case report. J Neurosurg Pediatrics. 1(3):258-60, 2008
- Mavrogiannis LA et al: Enlarged parietal foramina caused by mutations in the homeobox genes ALX4 and MSX2: from genotype to phenotype. Eur J Hum Genet. 14(2):151-8, 2006
- Mupparapu M et al: Hereditary cranium bifidum persisting as enlarged parietal foramina (Catlin marks) on cephalometric radiographs. Am J Orthod Dentofacial Orthop. 129(6):825-8, 2006
- Bianca S et al: Prenatal and postnatal findings of acrania. Arch Gynecol Obstet. 271(3):256-8, 2005
- Perlyn CA et al: Congenital scalp and calvarial deficiencies: principles for classification and surgical management. Plast Reconstr Surg. 115(4):1129-41, 2005
- Glass RB et al: The infant skull: a vault of information. Radiographics. 24(2):507-22, 2004
- Amaral L et al: MR imaging for evaluation of lesions of the cranial vault: a pictorial essay. Arq Neuropsiquiatr. 61(3A):521-32, 2003
- de Heer IM et al: Parietal bone agenesis and associated multiple congenital anomalies. J Craniofac Surg. 14(2):192-6, 2003
- Kortesis B et al: Surgical management of foramina parietalia permagna. J Craniofac Surg. 14(4):538-44, 2003
- Reimao R et al: Frontal foramina, Chiari II malformation, and hydrocephalus in a female. Pediatr Neurol. 29(4):341-4, 2003
- Tubbs RS et al: Parietal foramina are not synonymous with giant parietal foramina. Pediatr Neurosurg. 39(4):216-7, 2003
- Donati V et al: Reparation of a severe case of aplasia cutis congenita with engineered skin. Biol Neonate. 80(4):273-6, 2001
- Chandran S et al: Fetal acalvaria with amniotic band syndrome. Arch Dis Child Fetal Neonatal Ed. 82(1):F11-3, 2000
- Reddy AT et al: Enlarged parietal foramina: association with cerebral venous and cortical anomalies. Neurology. 54(5):1175-8, 2000
- Wilkie AO et al: Functional haploinsufficiency of the human homeobox gene MSX2 causes defects in skull ossification. Nat Genet. 24(4):387-90, 2000
- Kaplan SB et al: Radiographic manifestations of congenital anomalies of the skull. Radiol Clin North Am. 29(2):195-218, 1991
