Management of Chiari malformation in syndromic and nonsyndromic craniosynostosis

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OBJECTIVE

Although the association between Chiari malformation (CM) and craniosynostosis is well recognized, management remains controversial. There are differences in the clinical course of CM in syndromic craniosynostosis (SC) patients and nonsyndromic craniosynostosis (NSC) patients. Still unclear is whether cranial expansion surgery, foramen magnum decompression (FMD), or both should be conducted and when the appropriate timing of surgery should be. Here, the authors retrospectively reviewed the clinical data at their institution to investigate the optimal management of CM associated with SC and NSC.

METHODS

The authors retrospectively analyzed the clinical records of 163 children with craniosynostosis who underwent surgical treatment at the National Center for Child Health and Development between April 2002 and May 2018.

RESULTS

Twelve (10%) of 119 children with NSC and 14 (31.8%) of 44 children with SC were radiologically diagnosed with CM. The median age at which CM was radiologically diagnosed was 7 months. Of the 12 NSC patients with CM, 1 (8%) was symptomatic with gait disturbance. This patient underwent FMD following expansion surgery. Of the 14 SC patients with CM, 8 (57.1%) were symptomatic with central sleep apnea. For SC patients with symptomatic CM, cranial expansion surgery alone was conducted in 2 cases, FMD was conducted after cranial expansion in 4 cases, and FMD was conducted first and additional expansion surgery was then conducted in 2 cases. In all cases, symptoms of CM improved after completing this series of surgeries. Regarding patients with asymptomatic CM, cranial expansion surgery alone was performed, and none of the patients showed symptoms of CM thereafter in both SC and NSC cases.

CONCLUSIONS

Multiple surgeries tend to be necessary for symptomatic CM in both SC and NSC, while no patient with asymptomatic CM became symptomatic after cranial expansion surgery.

ABBREVIATIONS CM = Chiari malformation; CSA = central sleep apnea; FMD = foramen magnum decompression; ICP = intracranial pressure; NSC = nonsyndromic craniosynostosis; SC = syndromic craniosynostosis; VPS = ventriculoperitoneal shunt.
Article Information

Contributor Notes

Correspondence Hideki Ogiwara: National Center for Child Health and Development, Tokyo, Japan. hideki_o@d5.dion.ne.jp.INCLUDE WHEN CITING Published online December 20, 2019; DOI: 10.3171/2019.9.PEDS19261.Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
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