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Scientific article
English

Chiari I malformation associated with premature unilateral closure of the posterior intraoccipital synchondrosis in a preterm infant

Published inJournal of neurosurgery. Pediatrics, vol. 11, no. 6, p. 658-660
Publication date2013
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.

Keywords
  • Arnold-Chiari Malformation/etiology/radiography
  • Cranial Fossa, Posterior/abnormalities
  • Craniosynostoses/complications
  • Humans
  • Hydrocephalus/etiology
  • Infant
  • Infant, Newborn
  • Infant, Premature
  • Infant, Premature, Diseases/diagnosis/pathology/radiography
  • Magnetic Resonance Imaging
  • Male
  • Occipital Bone/abnormalities/growth & development/radiography
  • Osteochondrosis/complications
  • Skull Base/abnormalities/radiography
  • Tomography, X-Ray Computed
Citation (ISO format)
KANAVAKI, Aikaterini, JENNY, Benoît John, HANQUINET, Sylviane. Chiari I malformation associated with premature unilateral closure of the posterior intraoccipital synchondrosis in a preterm infant. In: Journal of neurosurgery. Pediatrics, 2013, vol. 11, n° 6, p. 658–660. doi: 10.3171/2013.3.PEDS12549
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ISSN of the journal1933-0707
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