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Partial Rhombencephalosynapsis and Chiari Type II Malformation in a Child: a True Association Supported by DTI Tractography

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Abstract

Partial rhombencephalosynapsis (PRECS) has been recently reported in association with Chiari II (CII). However, its existence as a true malformation is challenged due to the anatomical changes potentially induced by CII. The aim of this report was to investigate the contribution of midbrain/hindbrain tractography in this setting. A 13-year-old boy with a known CII malformation and operated myelomeningocele was referred for brain imaging after a first complex partial seizure. In addition to the classical features of CII, MRI showed partially fused cerebellar hemispheres and multiple supratentorial abnormalities. Diffusion tensor imaging (DTI) color map and tractography showed absent transverse fibers on the midsection of the cerebellum, scarce fibers of the middle cerebellar peduncle (MCP), absence of the middle pontine crossing tract, and fibers running vertically in the medial part of the cerebellum. Vertical mediocerebellar fibers are a feature of classical RECS and the paucity or absence of MCP fibers is mainly described in CII. In our patient, DTI and FT therefore demonstrated structural characteristics of both RECS and CII confirming their potential coexistence and suggesting possible shared embryological pathway.

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Conflict of Interest

The authors state that there is no conflict of interest (e.g., consultancies, stock ownership, equity interests, patent licensing arrangements), real or perceived. No honorarium, grant, or other form of payment was given to anyone to produce the manuscript.

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Authors and Affiliations

  1. Unit of Pediatric Radiology, Geneva University Hospital (HUG), 6, Willy-Donzé, 1205, Geneva, Switzerland

    Laura Merlini & Sylviane Hanquinet

  2. Pediatric Neurology, Pediatric Subspecialty Service, University Hospital of Geneva, Geneva, Switzerland

    Joel Fluss & Christian Korff

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  1. Laura Merlini
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  2. Joel Fluss
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  3. Christian Korff
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  4. Sylviane Hanquinet
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Correspondence to Laura Merlini.

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Merlini, L., Fluss, J., Korff, C. et al. Partial Rhombencephalosynapsis and Chiari Type II Malformation in a Child: a True Association Supported by DTI Tractography. Cerebellum 11, 227–232 (2012). https://doi.org/10.1007/s12311-011-0300-3

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