The (extended) achondroplasia foramen magnum score has good observer reliability.
The (extended) achondroplasia foramen magnum score has good observer reliability.
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DOI：
10.1007/s00247-022-05348-0
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发表时间：
2022-07
影响因子：
2.3
通讯作者：
中科院分区：
文献类型：
--
作者：
软骨发育不全是最常见的骨骼发育不良。一个重要的并发症是枕骨大孔狭窄。严重时，脊髓受压可导致睡眠呼吸暂停、呼吸骤停和死亡。为避免并发症，对高危病例进行颅颈交界处手术减压。然而，各中心的做法各不相同。为了规范磁共振（MR）报告，软骨发育不全症大孔评分最近被开发出来。该评分的可靠性尚未得到评估。评估大孔软骨发育不全评分的观察者间可靠性。四名观察员使用软骨发育不全大孔评分对谢菲尔德儿童医院治疗的软骨发育不全儿童的颅底影像进行了回顾性和独立的回顾。双向随机效应类内系数（ICC）用于评估观察者之间和观察者内部的信度。纳入49例符合条件的病例和5例对照。其中10例评分正常，17例评分中位数为1分（轻度狭窄），11例评分中位数为2分（脑脊液淡化），10例评分中位数为3分（脊髓受压），6例评分中位数为4分（脊髓病变）。观察者间ICC为0.72（95%置信区间= 0.62-0.81）。观察员内部的ICC范围为0.60至0.86。读者不同意的原因包括血流空洞伪影，微妙的T2脊髓信号和与椎管狭窄不成比例的脊髓性T2脊髓改变。软骨发育不全大孔评分具有良好的观察者间可靠性。已经确定了导致观察者之间分歧的成像特征。需要进一步的研究来前瞻性地验证该评分与临床结果的关系。
Achondroplasia is the most common skeletal dysplasia. A significant complication is foramen magnum stenosis. When severe, compression of the spinal cord may result in sleep apnea, sudden respiratory arrest and death. To avoid complications, surgical decompression of the craniocervical junction is offered in at-risk cases. However, practice varies among centres. To standardize magnetic resonance (MR) reporting, the achondroplasia foramen magnum score was recently developed. The reliability of the score has not been assessed. To assess the interobserver reliability of the achondroplasia foramen magnum score. Base of skull imaging of children with achondroplasia under the care of Sheffield Children’s Hospital was retrospectively and independently reviewed by four observers using the achondroplasia foramen magnum score. Two-way random-effects intraclass coefficient (ICC) was used to assess inter- and intra-observer reliability. Forty-nine eligible cases and five controls were included. Of these, 10 were scored normal, 17 had a median score of 1 (mild narrowing), 11 had a median score of 2 (effacement of cerebral spinal fluid), 10 had a score of 3 (compression of cord) and 6 had a median score of 4 (cord myelopathic change). Interobserver ICC was 0.72 (95% confidence interval = 0.62–0.81). Intra-observer ICC ranged from 0.60 to 0.86. Reasons for reader disagreement included flow void artefact, subtle T2 cord signal and myelopathic T2 cord change disproportionate to canal narrowing. The achondroplasia foramen magnum score has good interobserver reliability. Imaging features leading to interobserver disagreement have been identified. Further research is required to prospectively validate the score against clinical outcomes.
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影响因子：
3.3
作者：
Becker, Anton S.;Cornelius, Alexander;Donati, Olivio F.
通讯作者：
Donati, Olivio F.
影响因子：
4.3
作者：
Berry, Richard B.;Budhiraja, Rohit;Tangredi, Michelle M.
通讯作者：
Tangredi, Michelle M.
影响因子：
4.1
作者：
ARYANPUR, J;HURKO, O;CARSON, B
通讯作者：
CARSON, B
影响因子：
4.1
作者：
Bagley, CA;Pindrik, JA;Carson, BS
通讯作者：
Carson, BS
影响因子：
5.2
作者：
Cheung, Moira S.;Irving, Melita;Thompson, Dominic
通讯作者：
Thompson, Dominic