CT, MRI and clinical manifestations of Kasabach-Merritt syndrome in children / 中华放射学杂志

ABSTRACT

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, right pericardial lesion,skin and soft tissue of left arm were shown as iso-,hypointensity on T1-weighted image,iso-,hyper-intensity on T_2-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 T_1-weighted image,hyperintensity on T_2-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 multifocal 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.