Primary normocephalic pancraniosynostosis detected incidentally after an accidental head injury: a case report and review of the literature

Ai Peng Tan

Majority of multi-suture craniosynostosis are related to single-gene disorders or chromosomal abnormalities. Children with craniosynostosis usually present at an early age due to the presence of an abnormal head shape, with the exception of a unique entity termed primary normocephalic pancraniosynostosis. The objective of this article is to describe an unusual case of primary normocephalic pancraniosynostosis, detected incidentally following an accidental head injury. A comprehensive review of the literature will also be included. To the best of our knowledge, only eight cases of primary normocephalic pancraniosynostosis have been reported thus far. A 3-year 2-month-old child presented to the emergency department after a fall with severe scalp swelling. The child was noted to have mild frontal bossing and bilateral exophthalmos. Head size was normal but bilateral mild papilloedema was noted. CT scan was performed and demonstrated pancraniosynostosis and diffuse subgaleal hematoma. Patient underwent fronto-orbital advancement and total cranial vault reconstruction with favorable outcome. Our reported case adds to the current limited knowledge of this rare entity and emphasized the importance of a high index of suspicion in children with apparently normal head size and shape but show subtle evidence of raised intracranial pressure.