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先天性颈椎椎弓根缺如:临床及影像学表现

Congenital absence of a cervical spine pedicle: clinical and radiologic findings.

作者信息

Wiener M D, Martinez S, Forsberg D A

机构信息

Department of Radiology, Duke University Medical Center, Durham, NC 27710.

出版信息

AJR Am J Roentgenol. 1990 Nov;155(5):1037-41. doi: 10.2214/ajr.155.5.2120932.

Abstract

Congenital absence of a cervical pedicle is an unusual disorder with potentially confusing clinical and radiologic manifestations. A review of the previously reported 42 cases as well as 13 new cases is presented. Congenital absence of a cervical pedicle was an incidental finding in 10 of the 55 total patients (age range, 3-65 years; mean 31 years). The remainder presented with an array of symptoms; the most common was cervical pain (31 patients), often after trauma (19/31). Neurologic examination was normal in the majority (65%) of patients. The most common neurologic finding, in an additional 19% of patients, was isolated diminished sensation in an upper extremity. Imaging included conventional radiographs, tomograms, myelograms, and CT. The typical radiologic triad of findings included (1) the false appearance of an enlarged neural foramen owing to the absent pedicle; (2) a dysplastic, dorsally displaced ipsilateral articular pillar and lamina; and (3) a dysplastic ipsilateral transverse process. The spectrum of this disorder also included absence of the ipsilateral pillar in four of 55 patients and absence of the entire ipsilateral neural arch in five of 55 patients. Other osseous anomalies were present in 28 (51%) of 55 cases. In 10 of 55 cases, initial misdiagnosis resulted in inappropriate therapy, with a poor clinical outcome after therapy in three cases. Conservative therapy was the only successful management in all cases in which follow-up information was available, with the exception of one patient who had partial improvement after surgery. Careful analysis of radiologic images and knowledge of the specific findings of absent cervical pedicle should decrease the potential for misdiagnosis and inappropriate, potentially harmful therapy.

摘要

先天性颈椎椎弓根缺如是一种罕见疾病,其临床和影像学表现可能令人困惑。本文回顾了先前报道的42例病例以及13例新病例。在55例患者(年龄范围3至65岁,平均31岁)中,有10例先天性颈椎椎弓根缺如是偶然发现。其余患者表现出一系列症状;最常见的是颈部疼痛(31例患者),常发生于外伤后(19/31)。大多数患者(65%)神经系统检查正常。另外19%的患者最常见的神经系统表现是上肢孤立性感觉减退。影像学检查包括传统X线片、断层扫描、脊髓造影和CT。典型的影像学三联征包括:(1)由于椎弓根缺如导致神经孔假性扩大;(2)发育不良、向背侧移位的同侧关节突和椎板;(3)发育不良的同侧横突。该疾病谱还包括55例患者中有4例同侧关节突缺如,55例患者中有5例同侧整个神经弓缺如。55例中有28例(51%)存在其他骨异常。55例中有10例最初误诊导致治疗不当,其中3例治疗后临床效果不佳。除1例患者术后有部分改善外,在所有有随访信息的病例中,保守治疗是唯一成功的治疗方法。仔细分析影像学图像并了解颈椎椎弓根缺如的具体表现应可减少误诊以及不恰当的、可能有害的治疗的可能性。

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