Abstract

Chiari malformation type I (CM-I) is a well-known hindbrain disorder in which the cerebellar tonsils protrude through the foramen magnum. The soft tissues, including the transverse ligament and the tectorial membrane at the retro-odontoid space, can compress the cervicomedullary junction if they become hypertrophic. Twenty-two symptomatic patients with CM-I (aged 5-19 years) were treated between 2007 and 2017 at our institute. The retro-odontoid soft tissue was evaluated using T2-weighted magnetic resonance imaging. Anteroposterior (AP) distances and craniocaudal distances of the soft tissue were measured in patients with CM-I and 48 normal control children. Modified clivoaxial angles were also evaluated as the index of ventral compression of the cervicomedullary junction. Of the 18 patients treated with foramen magnum decompression, 16 patients improved postoperatively, whereas the condition of 2 remained unchanged. The AP distances in the CM-I group (6.0 mm) were significantly larger than those in the control group (3.5 mm), whereas there were no apparent differences in the craniocaudal distances. Modified clivoaxial angles were obviously smaller in the CM-I group (131.5°) than in the control group (146.9°). Moreover, the AP distances were significantly reduced postoperatively (5.5 mm), although the other parameters did not change significantly. The retro-odontoid soft tissue in symptomatic patients with CM-I can be hypertrophic enough to compress the cervicomedullary junction ventrally even if there are no combined osseous anomalies. Foramen magnum decompression works to reduce the hypertrophic changes significantly, suggesting that downward tonsil movement might participate in hypertrophic soft tissue formation at the retro-odontoid space.

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