Abstract
Chronic idiopathic hyperphosphatasia (CIH) is a rare generalised skeletal dysplasia in childhood. The clinical, radiographic and cerebral MR findings in a 5-year-old girl with the severe infantile form of CIH are reported. In spite of cranial enlargement, the intracranial space and the skull base were markedly reduced, the whole brain was compressed and a Chiari I malformation was present. Normal flow in the dural venous sinuses was documented. The patient showed no detectable cranial nerve involvement or hydrocephalus. Cranial MR in this patient enabled us to confirm that CIH involves the skull base and vault.
Similar content being viewed by others
Author information
Authors and Affiliations
Additional information
Received: 19 December 1997 Accepted: 17 April 1998
Rights and permissions
About this article
Cite this article
Mazzanti, L., Ambrosetto, P., Libri, R. et al. Involvement of the skull base and vault in chronic idiopathic hyperphosphatasia. Pediatric Radiology 29, 16–18 (1999). https://doi.org/10.1007/s002470050525
Issue Date:
DOI: https://doi.org/10.1007/s002470050525