Partial Rhombencephalosynapsis and Chiari Type II Malformation in a Child: a True Association Supported by DTI Tractography |
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Authors: | Laura Merlini Joel Fluss Christian Korff Sylviane Hanquinet |
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Institution: | 1. Unit of Pediatric Radiology, Geneva University Hospital (HUG), 6, Willy-Donzé, 1205, Geneva, Switzerland 2. Pediatric Neurology, Pediatric Subspecialty Service, University Hospital of Geneva, Geneva, Switzerland
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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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