Pediatric Chiari malformation type I: long‑term outcomes following small‑bone‑window posterior fossa decompression with autologous‑fascia duraplasty

Liu,Hai; Yang,Chenlong; Yang,Jun; Xu,Yulun

doi:10.3892/etm.2017.5211

Pediatric Chiari malformation type I: long‑term outcomes following small‑bone‑window posterior fossa decompression with autologous‑fascia duraplasty

Authors:
- Hai Liu
- Chenlong Yang
- Jun Yang
- Yulun Xu
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Affiliations: Department of Neurosurgery, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, P.R. China
Published online on: September 27, 2017 https://doi.org/10.3892/etm.2017.5211
Pages: 5652-5658

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Abstract

Chiari malformation type I (CM‑I) is a common hindbrain disorder that is associated with deformity and elongation of the cerebellar tonsils. Although CM‑I occurs in both pediatric and adult patients, its prevalence, clinical features and management in the pediatric population are not well defined. The current study evaluated a consecutive case series of 92 children (38 females and 54 males) who were diagnosed with congenital CM‑I. All patients underwent small‑bone‑window posterior fossa decompression with autologous‑fascia duraplasty. Clinical and radiological features were analyzed and long‑term follow‑up data were recorded. Risk factors associated with clinical outcomes were investigated using comprehensive statistical methods. Out of the 92 children, 11 (12.0%) were asymptomatic. Associated ventricular dilation was observed in 24 children (26.1%) and concomitant syringomyelia was observed in 72 children (78.3%). A total of 44 children (47.8%) showed scoliosis on plain films. Follow‑up data (mean duration, 88.6 months) were available for all patients. Syringomyelia was absent or markedly reduced in 56 patients (77.8%). Symptoms were alleviated in 66 patients, remained unchanged in 12 patients and progressed in 3 patients. Statistical analysis indicated that the cerebellar tonsillar descent (CTD) grade, basilar invagination and platybasia influenced the clinical outcome (P<0.05). In conclusion, early recognition and surgical treatment of CM‑I in pediatric patients can lead to good outcomes. The current results suggested that small‑bone‑window posterior fossa decompression with autologous‑fascia duraplasty was an effective safe treatment option with a low complication rate. High CTD grade, basilar invagination and platybasia were indicated to be predictors of poor clinical prognosis.

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