Congenital calvarial defects are a group of disorders characterized by congenital calvarial bone defects that vary in severity.
CT with 3D shaded surface reformats is the best imaging tool as it demonstrates calvarial defects and bone margins:
- parietal foramina
- parietal fissure: small residual incomplete medial parietal bone suture
- sinus pericranii: communication between intra- and extra-cranial venous systems through calvarial defects
- arachnoid granulations: often multiple having irregular contour
- atretic cephalocele
- abnormally large fontanelle: secondary to increased intracranial pressure or skeletal dysplasia
- aplasia cutis congenita: congenital skin defect which may have underlying skull defect
- cleidocranial dysplasia: 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 with subsequent large midline skull defect
- amniotic band syndrome: large skull defects
- acalvaria: absent superior osseous cranial vault and dura matter. Normal skull base, brain and facial bones.
- acrania: partial or complete absence of cranial vault bones with abnormal cerebral hemisphere development.
Imaging differential considerations include:
- 1. Osborn AG, Salzman KL, Barkovich AJ. Diagnostic Imaging. Lippincott Williams & Wilkins. (2009) ISBN:1931884722. Read it at Google Books - Find it at Amazon
- 2. Perlyn CA, Schmelzer R, Govier D et-al. Congenital scalp and calvarial deficiencies: principles for classification and surgical management. Plast. Reconstr. Surg. 2005;115 (4): 1129-41. Pubmed citation
- 3. Pekçevik Y, Hasbay E, Pekçevik R. Three-dimensional CT imaging in pediatric calvarial pathologies. Diagn Interv Radiol. 2013;19 (6): 488-94. doi:10.5152/dir.2013.13140 - Pubmed citation