Ont Health Technol Assess Ser. 2015 Jul 1;15(13):1-24. eCollection 2015.
Ehlers-Danlos syndrome (EDS) is an inherited disorder affecting the connective tissue. EDS can manifest with symptoms attributable to the spine or craniovertebral junction (CVJ). In addition to EDS, numerous congenital, developmental, or acquired disorders can increase ligamentous laxity in the CVJ and cervical spine. Resulting abnormalities can lead to morbidity and serious neurologic complications. Appropriate imaging and diagnosis is needed to determine patient management and need for complex surgery. Some spinal abnormalities cause symptoms or are more pronounced while patients sit, stand, or perform specific movements. Positional magnetic resonance imaging (pMRI) allows imaging of the spine or CVJ with patients in upright, weight-bearing positions and can be combined with dynamic maneuvers, such as flexion, extension, or rotation. Imaging in these positions could allow diagnosticians to better detect spinal or CVJ abnormalities than recumbent MRI or even a combination of other available imaging modalities might allow.
To determine the diagnostic impact and clinical utility of pMRI for the assessment of (a) craniovertebral or spinal abnormalities among people with EDS and (b) major craniovertebral or cervical spine abnormalities among symptomatic people.
A literature search was performed using Ovid MEDLINE, Ovid MEDLINE In-Process and Other Non-Indexed Citations, Ovid Embase, and EBM Reviews, for studies published from January 1, 1998, to September 28, 2014.
Studies comparing pMRI to recumbent MRI or other available imaging modalities for diagnosis and management of spinal or CVJ abnormalities were reviewed. All studies of spinal or CVJ imaging in people with EDS were included as well as studies among people with suspected major CVJ or cervical spine abnormalities (cervical or craniovertebral spine instability, basilar invagination, cranial settling, cervical stenosis, spinal cord compression, Chiari malformation).
No studies were identified that met the inclusion criteria.
We did not identify any evidence that assessed the diagnostic impact or clinical utility of pMRI for (a) craniovertebral or spinal abnormalities among people with EDS or (b) major craniovertebral or cervical spine abnormalities among symptomatic people relative to currently available diagnostic modalities.
埃勒斯-当洛综合征（EDS）是一种影响结缔组织的遗传性疾病。EDS可表现出归因于脊柱或颅颈交界区（CVJ）的症状。除EDS外，许多先天性、发育性或后天性疾病可增加CVJ和颈椎的韧带松弛。由此产生的异常可导致发病和严重的神经并发症。需要进行适当的影像学检查和诊断，以确定患者的治疗方案以及是否需要进行复杂手术。一些脊柱异常在患者坐立、站立或进行特定动作时会引起症状或更加明显。体位磁共振成像（pMRI）可在患者直立、负重的体位下对脊柱或CVJ进行成像，并可与动态动作（如屈伸或旋转）相结合。在这些体位下进行成像，诊断医生可能比在仰卧位MRI甚至其他现有成像方式组合下，能更好地检测出脊柱或CVJ异常。
确定pMRI对评估（a）EDS患者的颅颈或脊柱异常以及（b）有症状患者的主要颅颈或颈椎异常的诊断影响和临床实用性。
使用Ovid MEDLINE、Ovid MEDLINE在研及其他未索引引文、Ovid Embase和循证医学综述进行文献检索，检索1998年1月1日至2014年9月28日发表的研究。
对比较pMRI与仰卧位MRI或其他现有成像方式用于诊断和管理脊柱或CVJ异常的研究进行综述。纳入所有关于EDS患者脊柱或CVJ成像的研究，以及疑似主要CVJ或颈椎异常（颈椎或颅颈椎不稳、基底凹陷、颅骨下沉、颈椎管狭窄、脊髓受压、Chiari畸形）患者的研究。
未发现符合纳入标准的研究。
我们未找到任何证据表明，相对于目前可用的诊断方式，pMRI对（a）EDS患者的颅颈或脊柱异常或（b）有症状患者的主要颅颈或颈椎异常的诊断影响或临床实用性如何。
