Chiari 1 malformation: age-based outcomes in a paediatric surgical cohort
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ORIGINAL ARTICLE
Chiari 1 malformation: age-based outcomes in a paediatric surgical cohort Charles Yates 1
&
Robert Campbell 1 & Martin Wood 1 & Raymond Chaseling 1 & Gert Tollesson 1 & Norman Ma 1
Received: 30 December 2019 / Accepted: 27 February 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Purpose Substantial evidence exists describing differences between paediatric and adult Chiari 1 malformation (CM1) patients. Differences in clinical presentation between very young (0–6 years old) and older (7–18 years old) paediatric patients is similarly well-established. However, progression on these findings with regard to surgical outcomes is limited. We aimed to establish whether inter-paediatric age group modifies surgical outcome for CM1 decompression. Methods Retrospective chart review was conducted for 65 patients receiving posterior fossa decompression between 2006 and 2018. Presenting features, surgical management, and outcome were evaluated and stratified into very young patients (0–6 years) or older patients (7–18 years). Outcomes were assessed using the Chicago Chiari Outcome Scale (CCOS), a validated 16-point framework for comparison. Results Very young patients (21 patients) scored significantly lower in surgical outcome overall compared with older patients (44 patients) (12.1 ± 3.2/16 vs 14.2 ± 1.6/16, p = 0.011), and across 3/4 CCOS subscores: non-pain symptoms, functionality, and complications. Very young patients also returned to theatre more commonly (47.6% vs 13.6%, p = 0.003), primarily for re-do decompression (7/10 patients, 70%). Finally, the presentation of very young patients differed to older patients with significantly more oropharyngeal (38.1% vs 9.0%, p = 0.014) and motor symptoms (47.6% vs 22.7%, p = 0.042). Discussion Very young patients (0–6 years) do not appear to respond as well to standard posterior fossa decompression, as their older (7–18 years) paediatric counterparts, in the absence of several baseline cohort characteristic differences. We hypothesise underlying anatomical differences may contribute to this finding. Keywords Chiari 1 malformation . Posterior fossa decompression . Paediatric
Introduction * Charles Yates [email protected] Robert Campbell [email protected] Martin Wood [email protected] Raymond Chaseling [email protected] Gert Tollesson [email protected] Norman Ma [email protected] 1
Department of Neurosurgery, Queensland Children’s Hospital, Brisbane, Queensland, Australia
Chiari 1 malformation (CM1) presents a curious pathology, with significant ambiguity in pathogenesis, natural history, management, and prognosis. Traditionally, it is defined as a herniation of the cerebellar tonsils greater than 5 mm below the foramen magnum [7]; however, it is more accurately defined by a mechanical obstruction of cerebrospinal fluid (CSF) flow in the posterior fossa. The incidence by radiological diagnosis is 0.8–3.7%, of which approximately 1/3 are symptomatic
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