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Chiari malformation in craniosynostosis

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Abstract

Introduction

Chiari malformation (CM) is a frequent finding in multisutural and syndromic craniosynostosis, occurring in 70% of patients with Crouzon’s syndrome, 75% with oxycephaly, 50% with Pfeiffer’s syndrome and 100% with the Kleeblattschädel deformity. The pathogenesis of this condition and rationale for treatment are still controversial.

Discussion

Since its first description in 1972, several factors have been cited to play a role in inducing CM. In the light of recent publications, the roles of premature fusion of cranial vault and cranial base sutures, of congenital anomalies of the cerebellum and brain stem, of raised intracranial pressure, of venous hypertension and of hydrocephalus are reviewed. Evaluation and management of CM are also discussed.

Conclusion

Chiari malformation appears to be an acquired and progressive condition that develops in the first months of life, because of a disproportion between hindbrain growth and an abnormally small posterior fossa, a consequence of the premature fusion of lambdoid and cranial base sutures. Venous hypertension caused by stenosis of the jugular foramen can also be present in these patients, resulting in intracranial hypertension and/or hydrocephalus. Careful MRI evaluation is recommended for the forms of craniosynostosis at a high risk of developing hindbrain herniation. The selection of posterior cranial vault expansion as the first surgical procedure is advocated. In selected cases, treatment of the posterior cranial deformity by occipital vault remodelling and treatment of the Chiari-like deformity by suboccipital decompression can be carried out using the same surgical procedure.

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Acknowledgements

All the patients described in this paper were evaluated and treated in the Department of Pediatric Neurosurgery of the Hôpital Necker-Enfants Malades, Paris, France.

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Authors and Affiliations

  1. Department of Pediatric Neurosurgery, Santobono-Pausilipon Children’s Hospital, Naples, Italy

    Giuseppe Cinalli, Pietro Spennato & Ferdinando Aliberti

  2. Via Gennaro Serra n. 75, 80132, Naples, Italy

    Giuseppe Cinalli

  3. Department of Pediatric Neuroradiology, Santobono-Pausilipon Children’s Hospital, Naples, Italy

    Emilio Cianciulli

  4. Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, Paris, France

    Christian Sainte-Rose, Eric Arnaud & Dominique Renier

  5. Department of Pediatric Radiology, Hôpital Necker-Enfants Malades, Paris, France

    Francis Brunelle

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  1. Giuseppe Cinalli
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Correspondence to Giuseppe Cinalli.

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Cinalli, G., Spennato, P., Sainte-Rose, C. et al. Chiari malformation in craniosynostosis. Childs Nerv Syst 21, 889–901 (2005). https://doi.org/10.1007/s00381-004-1115-z

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