ABSTRACT
Background:
In
patients with Chiari 1.5 malformation (CM1.5), a more aggressive
disease course and an increased
association with craniovertebral junction (CVJ) anomalies has been suggested. The best management of this subgroup of
patients is not clearly defined, also due to the lack of specific series elucidating this anomaly's peculiar characteristics.
Methods:
We evaluated a series of 33
patients (25
females, 8
males; mean age at
surgery 13 years) fulfilling the criteria for Chiari 1.5
diagnosis who underwent posterior fossa
decompression and duraplasty (PFDD) between 2006 and 2021.
Results:
Headache was present in all
children, five presented
central apnea, five had
dysphagia, and three had
rhinolalia.
Syringomyelia was present in 19 (58%)
children. Twenty
patients (61%) showed various CVJ anomalies, but only one
child presented instability requiring
arthrodesis. The mean
tonsil displacement below the
foramen magnum was 19.9 mm (range 12-30), without significant correlation with the severity of symptoms.
Syringomyelia recurred or was unchanged in three
patients, and one needed C1-C2 fixation. The
headache disappeared in 28
children (84%).
Arachnoid opening and
tonsil coagulation or resection was necessary for 19
children (58%).
Conclusions:
In our pediatric CM series, the need for
tonsil resection or coagulation was higher in CM1.5
children due to a more severe
crowding.
