目的:颅骨交界处(CVJ)异常在粘多糖贮积症I型Hurler综合征(MPSIH)中很常见且有充分记载,常导致严重的椎管狭窄。然而,手术减压和/或融合的要求并不常见。尽管造血细胞移植(HCT)已被证明可以延长MPSIH患者的生命,其在阻止或逆转肌肉骨骼异常方面的作用尚不清楚。不幸的是,对于这些患者,目前尚无通用的影像学指南或手术干预指征.这项研究的目的是跟踪HCT后MPSIH患者的CVJ解剖结构的进展,并检查需要手术干预的患者的影像学特征。
方法:回顾性分析了2008年至2020年在明尼苏达大学接受同种异体HCT治疗的MPSIH患者。接受CVJ手术的患者通过图表审查进行鉴定。检查所有MPSIH宫颈扫描,和齿状突后屈角,顶轴角(CXA),运河宽度,和Grabb-Oakes距离(pB-C2)在HCT后的7年内每年进行测量。建立了基于测量的纵向模型。使用组内相关系数来衡量评估者间的可靠性。对9名没有MPSIH的儿童进行了对照CVJ测量。
结果:共检查了54例MPSIH患者的253次颈椎MRI扫描。研究队列中只有4名(7.4%)患者需要手术。其中3例进行后颅窝和C1减压,1个有C1-2融合。手术和非手术组之间检查的脊柱参数没有统计学上的显着差异。在测量中,不同颈部位置的患者的管宽度和CXA差异很大。随着年龄的增长,齿状突后屈角和CXA呈下降趋势。随着年龄的增长,运河宽度和pB-C2有增加的趋势。
结论:根据数据,作者观察到根管宽度和pB-C2增加,而随着HCT后患者的年龄增长,CXA和齿状突后屈角变得更加尖锐。从这些数据得出的纵向模型反映了没有MPSIH的儿童的发育。仅在MR图像上获得的脊柱测量不足以识别需要手术干预的患者。症状监测和临床检查,以及MRI上的病理性脊髓改变,在评估手术需求方面比获得连续成像更为重要。
OBJECTIVE: Craniovertebral junction (CVJ) abnormalities are common and well documented in mucopolysaccharidosis type I-Hurler syndrome (MPS IH), often causing severe spinal canal narrowing. However, the requirement for surgical decompression and/or fusion is uncommon. Although hematopoietic cell transplant (HCT) has been shown to prolong the lives of patients with MPS IH, its effect in halting or reversing musculoskeletal abnormalities is less clear. Unfortunately, there are currently no universal guidelines for imaging or indication for surgical interventions in these patients. The goal of this study was to track the progression of the CVJ anatomy in patients with MPS IH following HCT, and to examine radiographic features in patients who needed surgical intervention.
METHODS: Patients with MPS IH treated at the University of Minnesota with allogeneic HCT between 2008 and 2020 were retrospectively reviewed. Patients who underwent CVJ surgery were identified with chart review. All MPS IH cervical scans were examined, and the odontoid retroflexion angle, clivoaxial angle (CXA), canal width, and Grabb-Oakes distance (pB-C2) were measured yearly for up to 7 years after HCT. Longitudinal models based on the measurements were made. An intraclass correlation coefficient was used to measure interrater reliability. Nine children without MPS IH were examined for control CVJ measurements.
RESULTS: A total of 253 cervical spine MRI scans were reviewed in 54 patients with MPS IH. Only 4 (7.4%) patients in the study cohort required surgery. Three of them had posterior fossa and C1 decompression, and 1 had a C1-2 fusion. There was no statistically significant difference in the spinal parameters that were examined between surgery and nonsurgery groups. Among the measurements, canal width and CXA varied drastically in patients with different neck positions. Odontoid retroflexion angle and CXA tended to decrease with age. Canal width and pB-C2 tended to increase with age.
CONCLUSIONS: Based on the data, the authors observed an increase in canal width and pB-C2, whereas the CXA and odontoid retroflexion angle became more acute as the patients aged after HCT. The longitudinal models derived from these data mirrored the development in children without MPS IH. Spinal measurements obtained on MR images alone are not sufficient in identifying patients who require surgical intervention. Symptom monitoring and clinical examination, as well as pathological spinal cord changes on MRI, are more crucial in assessing the need for surgery than is obtaining serial imaging.