婴幼儿卡-梅综合征的磁共振表现

MRI findings of Kasabach-Merritt syndrome in infants

目的探讨婴幼儿卡-梅综合征不同时期的磁共振表现,提高对此病的认识。方法选取确诊为卡-梅综合征的28例患儿的临床及影像资料。所有患儿实验室检查血小板均低于100×10^(9)/L。接受了磁共振检查的患儿治疗后缓解期共有28例。结果28例患儿中,男性15例,女性13例,年龄中位数为8个月。所有病例均临床诊断为卡-梅综合征,病灶均位于体表,头颈部8例、躯干11例、四肢9例。发病期患儿磁共振表现为病变部位巨大混杂信号肿块,境界模糊,增强扫描呈明显不均匀强化,病变皆有侵袭性表现(即真皮及皮下组织增厚,病灶向附近肌层浸润,周围皮肤水肿样图像),部分病变可见其供血动脉自正常动脉主干发出(5例),部分病变出现骨质破坏(4例)。治疗后稳定期复查磁共振,病变范围较前均有缩小,侵袭性表现消失,患儿血小板计数升高,均稳定在正常值范围内。结论婴幼儿卡-梅综合征具有典型的临床表现,病情进展迅速,疾病发展在不同时期,磁共振表现具有一定的特征性。

Objective In order to improve the understanding of this rare and critical disease by analyzing the magnetic resonance imaging(MRI)of Kasabach-Merritt syndrome in infants and young children at different stages combined with the clinical manifestations.Methods The clinical and imaging data of 28 children diagnosed as Kasabach-Merritt syndrome were retrospectively analyzed.The platelet count in all the children was lower than 100×10^(9)/L.A total of 20 children underwent MRI at the onset and 28 at the remission stage after treatment.Results Among the 28 cases,15 were male and 13 were female,with a median age of 8 months.All the cases were clinically diagnosed as Carmel syndrome,and the lesions were all located on the body surface,including 8 cases of head and neck,11 cases of trunk and 9 cases of limbs.At the onset stage,the MRI manifestations were huge mixed signal mass with vague boundary,the enhancement scan showed obvious inhomogeneous enhancement,and the lesions all had invasive manifestations(namely the thickening of the dermis and subcutaneous tissue,muscular lesion to nearby infiltration,the surrounding skin edema sample image),part of its blood supply artery lesion(5 cases)was issued by the normal artery,some lesions appeared bony destruction(4 cases).After treatment,MRI was rechecked at a stable period,and the lesion scope was smaller than before,the invasive expression disappeared,and the platelet count of the children was increased,all of which were stable within the normal range.Conclusion Infants with Kasabach-Merritt syndrome have typical clinical manifestations,rapid progress of the disease,disease development in different periods and magnetic resonance imaging has certain characteristics.