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

Case report

Aikaterini Kanavaki M.D.1, Benoit Jenny M.D., Ph.D.2, and Sylviane Hanquinet M.D.1
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  • 1 Unit of Pediatric Radiology, Department of Radiology, and
  • | 2 Department of Neurosurgery, University Hospital of Geneva, Switzerland
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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.

Abbreviation used in this paper:

CM = Chiari malformation.

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