gms | German Medical Science

Objective: For years treatment of Chiari I malformation in children and young adults is a matter of controversy regarding both the diagnosis and the details of decompression. In this paper, differences in clinical presentation and diagnosis between children and adults are reported and results of decompression analysed.

Methods: Since 1991 all patients with spinal cord pathologies were entered into a spinal cord data base. Between 1991 and 2015 a total ot 725 patients with Chiari I malformation were encountered. Among these 60 children between 3 and 17 years presented (follow up 53.5+50.7 months). Patients were examined on admission, after surgery, 3 months after discharge and yearly thereafter. Clinical data were analyzed with a scoring system for individual symptoms. Long-term results were determined by Kaplan-Meier statistics to calculate rates for progression-free survival.

Results: By comparison to adults children complain significantly less about occipital headaches (55.9% vs. 79.1%, p < 0.0001), sensory disturbances (20.3% vs. 59.2%, p < 0.0001), dysesthesias (15.3% vs. 39.9%, p = 0.002), motor weakness (13.6% vs. 33.8%, p = 0.04) or gait ataxia (28,8% vs. 59.1%, p = 0.0003). Bony anomalies such as basilar invagination, however, tended to be more common in children (20.0% vs. 13.4%, p = 0.06). 45.0% of children and 52.0% of adults underwent foramen magnum decompression with duraplasty and inspection of the 4^th ventricle. Foramen Magendie was found to be occluded in 29.6% of children and 36.9% of adults (not significant). Postoperatively, 74.1% of children and 77.3% of adults reported improvement. The remainder considered their condition unchanged except 4.8% of adults, whose condition had worsened related to previous attempts of decompression. Progression-free survival for 10 years was achieved for 88.8% of children and 80.4% of adults (not significant).

Conclusion: Diagnosis of Chiari I malformation in childhood has to account for the physiologic tonsillar descent and differences in clinical symptoms compared to adults. A decompression should only be advised in patients with unequivocal symptoms and imaging results. Short- and long-term results of decompression with opening of the arachnoid and duraplasty tend to be slightly better compared to adults.