Cobb综合征的影像学表现(2例报告结合文献复习)

The medical imaging findings in Cobb syndrome(report of 2 cases and literature review)

目的分析Cobb综合征的影像学表现。方法2例患者,均为男性,年龄分别为22和13岁。临床症状为下肢无力,查体见皮肤血管瘤、下肢肌力下降。均行MRI、CT、DSA检查并经手术治疗。结果MRI显示2例椎体多发血管瘤,T1WI呈高或略高信号,T2WI呈高信号,压脂像(STIR)呈低信号;相应节段椎管及肌肉内见不规则长T1、长T2信号,有流空现象;增强扫描椎管内病灶呈明显强化。CT平扫仅显示椎体血管瘤,表现为局部骨小梁增粗,呈网眼状,冠、矢状面重建可见栅栏征。DSA示肋间动脉、肋下动脉增粗、紊乱,周围静脉提前显影并增粗迂曲,多个椎体内可见紊乱血管团。结论MRI能明确显示Cobb综合征椎体、椎管、脊髓、肌肉等组织的病变及其范围,结合皮肤表现对该病有诊断价值。

Objective To analyze the imaging findings in Cobb syndrome. Methods Two male patients, aged 22 and 13 years respectively, had suffered from weakness of lower limbs. Clinical examination revealed a decline of muscle strength of lower extremities and dermal angiokeratomas of trunk. Both cases underwent CT, MRI, DSA, and operation. Results MRI showed hemangioma in vertebral bodies which was hyperintense or slight hyperintense on T_1WI, hyperintense on T_2WI, and hypointense on fat-suppressed images. There were some lesions with long T_1 and long T_2 signal in vertebral canal and muscles, where flow void effect was seen. Non-enhanced CT only demonstrated hemangiomas with mesh-like change and thickening of trabeculae of bone in multiple vertebral body lesions. “Palisade sign” was revealed on coronal and sagittal reconstruction images. DSA showed dilatation and disorderliness of the intercostal artery and subcostal artery in the corresponding levels, early opacification of dilated and tortuous draining veins, and multiple dense niduses within vertebral bodies. The findings during operation were corresponded with those of DSA. Conclusion MRI can show the lesions of Cobb syndrome exactly in vertebral body, vertebral canal, spinal cord and muscles. Combining with angiokeratomas of skin, MRI has important diagnostic value on Cobb syndrome.