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应用快速和标准 MRI 对横纹肌瘤和结节性硬化症进行产前和产后诊断。

Prenatal and Postnatal Diagnosis of Rhabdomyomas and Tuberous Sclerosis Complex by Ultrafast and Standard MRI.

机构信息

Department of Radiology, Shanghai Children's Medical Center, 1678 Dongfang Rd., Shanghai, 200127, China.

出版信息

Indian J Pediatr. 2018 Sep;85(9):729-737. doi: 10.1007/s12098-017-2592-x. Epub 2018 Jan 9.

Abstract

OBJECTIVE

To examine the features of cardiac rhabdomyomas and tuberous sclerosis in fetuses and infants using magnetic resonance imaging (MRI) and to determine whether MRI is an effective tool to facilitate early detection of tuberous sclerosis complex (TSC).

METHODS

Fifteen patients with TSC were evaluated by ultrafast or standard MRI between June 2005 and September 2016. Fifteen patients were divided into two groups. Group A included five cases in utero and followed in infancy with gestational ages from 26 + 1 to 38 + 2 wk. Group B included ten cases aged from 36 d to 18-mo-old.

RESULTS

There were 11 and 10 cardiac lesions of prenatal and postnatal period respectively in five subjects of Group A and 27 cardiac lesions in ten subjects of Group B. There were more than 31 prenatal brain lesions and 30 postnatal brain lesions in Group A and 169 lesions in Group B. Standard postnatal brain MRI confirmed the prenatal study of Group A. At 1 y follow up of Group A, there was partial regression of 2 cardiac lesions, complete regression of 1 cardiac lesion, no obvious regression of 8 cardiac lesions.

CONCLUSIONS

When one or multiple cardiac tumors are detected by ultrasound in fetal period or some specific clinical manifestations are presented in infancy, fetal ultrafast MRI or standard MRI is suggested to make early diagnosis of TSC.

摘要

目的

运用磁共振成像(MRI)研究胎儿和婴儿心脏横纹肌瘤和结节性硬化症的特征,探讨 MRI 是否有助于早期发现结节性硬化症复合征(TSC)。

方法

2005 年 6 月至 2016 年 9 月,对 15 例 TSC 患者进行了超快速或标准 MRI 检查。将这 15 例患者分为两组,A 组 5 例,在宫内和婴儿期进行评估,孕龄为 26+1 周至 38+2 周;B 组 10 例,年龄为 36 d 至 18 个月。

结果

A 组 5 例患者中,产前和产后分别有 11 例和 10 例心脏病变,B 组 10 例患者中有 27 例心脏病变。A 组产前有超过 31 例脑病变,产后有 30 例脑病变;B 组产前有 169 例脑病变,产后有 169 例脑病变。标准的产后脑 MRI 证实了 A 组的产前研究。A 组 1 年随访时,有 2 个心脏病变部分消退,1 个心脏病变完全消退,8 个心脏病变无明显消退。

结论

当胎儿期通过超声检查发现一个或多个心脏肿瘤或婴儿期出现某些特定的临床表现时,建议进行胎儿超快速 MRI 或标准 MRI 检查,以早期诊断 TSC。

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