Management of Chiari malformation in syndromic and nonsyndromic craniosynostosis

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>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.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>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.</jats:p></jats:sec>

Minor Suture Fusion is Associated With Chiari Malformation in Nonsyndromic Craniosynostosis

“…Second, erroneously diagnosed SC cases could have been included among the NSC cases as genetic testing at our clinic is only performed in patients with suspected SC. Therefore, the proportion of NSC in the total craniosynostosis was higher than in previous reports, and it is possible that the proportion of CM complications in NSC was higher than in previous reports because SC is more likely to be complicated by CM 22 . However, as SC is usually associated with syndactyly and facial bone deformities, erroneous diagnoses were unlikely.…”

“…Therefore, the proportion of NSC in the total craniosynostosis was higher than in previous reports, and it is possible that the proportion of CM complications in NSC was higher than in previous reports because SC is more likely to be complicated by CM. 22 However, as SC is usually associated with syndactyly and facial bone deformities, erroneous diagnoses were unlikely. Another limitation is that PFA can be affected by anterior cranial fossa deformation.…”

“…Chiari malformation was characterized as cerebellar tonsillar herniation more than 5 mm below the foramen magnum, as observed on MRI sagittal images. 8,[21][22][23][24]…”

Are Patients With Syndromic Craniosynostosis at Greater Risk for Abnormal Speech and Language Development Than Patients With Non-syndromic Craniosynostosis?

“…According to Matsuhashi et al, the prevalence of CM among patients with SCS and NSCS is 31.8% and 10%, respectively. 31 CM is thought to develop among patients with SCS due to ICH and an exceedingly small posterior cranial fossa that cannot keep up with the hindbrain's growth. 32,33 Several surgical interventions can correct CM among patients with CS.…”