The authors report a case of a preterm infant at 29 weeks of gestation who gradually developed a Chiari malformation Type I (CM-I) with hydrocephalus due to a premature unilateral fusion of the posterior intraoccipital synchondrosis. Brain ultrasonography results in the 1st week of life were normal. Follow-up ultrasonography showed progressive development of triventricular hydrocephalus. Brain MRI demonstrated the presence of a CM-I and a deformation of the occipital bone. A complementary CT scan was obtained, showing a closure of the right posterior intraoccipital synchondrosis, resulting in a deformation of the posterior cranial fossa. This case shows the close relationship between a malformation of the skull base and the secondary development of a brain malformation. The authors discuss the anatomy of the occipital bone and suggest a probable theory for the premature closure of this synchondrosis and the consequent development of a CM-I. The originality of this case lies in the observation of the natural history of a brain malformation in a preterm infant.
Address correspondence to: Aikaterini Kanavaki, M.D., Unit of Pediatric Radiology, Department of Radiology, University Hospital of Geneva, Rue Willy Donzé 6, Geneva 1205, Switzerland. email: firstname.lastname@example.org.
Please include this information when citing this paper: published online April 12, 2013; DOI: 10.3171/2013.3.PEDS12549.
NoudelR, , JoveninN, , EapC, , ScherpereelB, , PierotL, & RousseauxP: Incidence of basioccipital hypoplasia in Chiari malformation type I: comparative morphometric study of the posterior cranial fossa. Clinical article. J Neurosurg111:1046–1052, 2009
NoudelR, JoveninN, EapC, ScherpereelB, PierotL, RousseauxP: Incidence of basioccipital hypoplasia in Chiari malformation type I: comparative morphometric study of the posterior cranial fossa. Clinical article. J Neurosurg111:1046–1052, 2009)| false