Pediatric complex Chiari I Malformation-how complex is it?

BACKGROUND

Pediatric complex Chiari I Malformation (CCM), defined by Chiari I with additional craniovertebral junction anomalies, has been associated with higher failure rates following foramen magnum decompression (FMD) alone, leading some to advocate occipitocervical fixation (OCF) or ventral decompression. This study evaluates a single-institution experience with isolated FMD in CCM.

METHODS

Patients meeting CCM criteria were identified from a prospective surgical database from March 2020 to December 2023. Patients with incomplete imaging or < 6-month follow-up were excluded. Pre- and postoperative imaging assessed tonsillar descent, brainstem crowding, CSF flow, and syringomyelia. Clinical outcome was recorded.

RESULTS

Sixty patients met the inclusion criteria for CCM, but nineteen patients were excluded. Forty-one CCM patients underwent FMD. Headache prevalence decreased from 73.2% preoperatively to 7.3% at last follow-up (p < 0.001). One patient (2.4%) required reoperation for inadequate decompression. Median tonsillar descent improved from 15.0 to 0.0 mm (p < 0.001), and obex ectopia from 7.0 to 0.0 mm (p < 0.001). Anterior and posterior CSF effacement significantly improved. Craniocervical angle increased from 122.0 to 130.0° (p = 0.007), and pb-C2 distance decreased from 7.0 to 5.0 mm (p < 0.001). No patient required OCF or ventral decompression during a mean 2.1-year follow-up.

CONCLUSION

Isolated FMD provides effective clinical and radiological improvement in most pediatric CCM patients. Routine addition of OCF or ventral decompression may be unnecessary, preserving cervical mobility while minimizing morbidity.