title: Chiari I malformation associated with premature unilateral closure of the posterior intraoccipital synchondrosis in a preterm infant.
abstract: 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.
authors.affiliation: Unit of Pediatric Radiology, Department of Radiology, University Hospital of Geneva, Rue Willy Donzé 6, Switzerland. aikaterini.kanavaki@hcuge.ch
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Kanavaki
authors.forename: Benoit
authors.initials: B
authors.lastname: Jenny
authors.forename: Sylviane
authors.initials: S
authors.lastname: Hanquinet
mesh_terms: D001139:Arnold-Chiari Malformation / Q000000981:diagnostic imaging / Q000209:etiology*; D003388:Cranial Fossa, Posterior / Q000002:abnormalities; D003398:Craniosynostoses / Q000150:complications*; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology*; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D009777:Occipital Bone / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000254:growth & development; D055034:Osteochondrosis / Q000150:complications*; D019291:Skull Base / Q000002:abnormalities* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed