Cobb综合征的临床表现和比较影像学分析(附4例报告并文献复习)

The clinical features and comparative imaging studies on Cobb syndrome (report of 4 cases and review the literature)

目的:分析Cobb综合征的临床和影像学表现,并进行影像学方法比较。方法:4例患者临床表现为双下肢无力或瘫痪,查体主要见下肢肌力减退和皮肤血管瘤。4例均行64排螺旋CT和DSA检查,其中3例行MRI检查。结果:CT增强扫描可显示病变相应节段的髓内、髓外椎管内、椎旁及肌肉和皮下软组织内的血管畸形。多排螺旋CT血管造影可显示畸形血管团及其主要供血动脉和引流静脉,并可立体观察畸形血管与邻近结构的关系。MRI平扫可见髓内异常信号,T1WI呈低或等信号,T2WI呈高信号,椎管内外可见大量流空信号,呈迂曲状;肌肉和皮下软组织内可见相似病灶,增强后病变明显强化。DSA行选择性双侧椎动脉、甲状颈干和/或相应节段的肋间动脉及腰动脉造影,可清楚显示病变节段内畸形血管团、增粗扩张的供血动脉和粗大的引流静脉。结论:多排螺旋CT血管造影和MRI能无创、准确地显示Cobb综合征髓内、椎管内外、椎体和软组织内病变的形态及范围,结合皮肤表现可对该病做出确定诊断。

Objective：To analyze the clinical and imaging findings on Cobb syndrome, and compare the differences among CT, MRI and DSA. Methods：Two female patients and two male patients, with mean age of 20 years old, suffered from weakness or paralysis of lower limbs. Clinical examination revealed decline of muscle strength of lower extremities and dermatoangiokeratomas. All patients underwent 64-row multi-detector CT （MDCT） and DSA, and 3 of them underwent MRI. Results： Enhanced CT images revealed the intramedullary, extramedullary, paravertebral and hypodermal vascular malformations in corresponding spinal segments. MDCT angiography could detect the masses of deformed vessels with their main feeding arteries and drainage veins. Furthermore, MDCT angiography could also show the relations among deformed vessels and their adjacent tissues. Non-enhanced MRI displayed abnormal intramedullary signals, which was hypointensity or isointensity on T1WI and hyperintensity on T2WI. There were lots of winding flow-void effect within and out of vertebral canal. Meanwhile, the same images could also be seen in muscles and hypodermal soft tissues, which could be obviously enhanced after contrast agent injected. Selective catheterization of bilateral vertebral arteries, thyrocervical thinks, and/or corresponding intercostal arteries, lumbar arteries were performed with Seldinger＇ s technique. DSA showed the masses of abnormal vessels, dilated feeding arteries and drainage veins clearly in corresponding segments. Conclusion： MDCT angiography and MRI can reveal the lesions of Cobb syndrome exactly and entirely, and combining with skin appearances, we can draw the definite diagnosis on it.