儿童Kasabach—Merritt综合征的临床和CT、MRI表现

CT, MRI and clinical manifestations of Kasabach-Merritt syndrome in children

目的提高对儿童 Kasabach-Merritt 综合征(KMS)的临床及其 CT 和(或)MRI 表现的认识。方法分析总结6例 KMS 患儿的临床表现及其 CT 和(或)MRI 特点。结果 6例患儿均有血小板减少等消耗性凝血功能异常。3例 MRI 分别显示后纵隔脊柱旁、右侧心包及左上臂皮肤及皮下软组织 T_1WI 等、低信号,T_2WI 等、高信号病灶,增强呈不均匀显著强化;1例脾脏异常增大,CT 平扫为均匀稍低密度,增强呈弥漫性不均匀强化,MR T_1WI 呈低信号,T_2WI 呈高信号,增强后呈不均匀显著强化;1例除多根长骨干骺端骨质稀疏、骨皮质变薄外,脾脏亦增大,CT 平扫见多个大小不等低密度灶,增强早期见病灶周边强化,延迟相部分病灶有对比剂逐渐充填;1例肝左叶巨大低密度肿块,增强早期病灶周边强化,延迟相部分病灶见对比剂逐渐充填。结论血小板减少及凝血功能障碍提示可能合并大面积血管瘤;脾脏增大伴密度或 MRI 信号弥漫、局灶性降低应考虑血管瘤可能。

Objective To improve the awareness of the CT and/or MRI appearances and clinical presentations of Kasabach-Merritt syndrome（ KMS）, 6 cases were reported. Methods The CT and/or MRI and clinical characters of 6 cases of KMS were reviewed and analyzed. Results Thrombocytopenia, and consumption coagulopathy were presented in all 6 cases. The paravertebral mass of posterior mediastinum, fight pericardial lesion, skin and soft tissue of left ann were shown as iso-, hypointensity on Tl-weighted image, iso-, hyper-intensity on T2-weighted image, and with heterogeneous enhancement after contrast administration in three cases. Splenomegaly was presented in one case, it showed homogenous hypoattenuation on unenhanced CT, and diffused heterogeneous enhancement after contrast administration. On MRI, spleen was shown as hypointensity on TI-weighted image, hyperintensity on T2-weighted image, and with heterogeneous enhancement after contrast administration. There were lesions in both bones and spleen in one case. Osteopenia and thinned cortex were shown on the metaphyses of upper and lower extremities. Spleen enlarged and showed muhifocal hypoattenuation lesions, the peripheral region enhanced on the early phase, and some of the lesions filled on the delayed phase after the contrast administration. One case showed huge hypoattenuation lesion in the left lobe of liver with gradually filling of the mass after peripheral enhancement. Conclusion Thrombocytopenia and consumption coagulopathy suggest the entity of extensive hemangioma. Splenomegaly with diffuse or focal hypoattenation or hypointensity in MRI infers the diagnosis of hemangioma,