Song Chunze,Jiang Guoping,Ye Jingjing,et al.Clinical analysis of tuberous sclerosis complex complicated by cardiac rhabdomyomas in children[J].Journal of Clinical Pediatric Surgery,,18():767-770.[doi:10.3969/j.issn.1671-6353.2019.09.012]
Clinical analysis of tuberous sclerosis complex complicated by cardiac rhabdomyomas in children
- Keywords:
- Tuberous Sclerosis; Heart; Rhabdomyoma; Ultrasonography; Child
- CLC:
- R732.1;R445.1
- Abstract:
- Objective To analysis the clinical features of tuberous sclerosis complex (TSC) complicated by cardiac rhabdomyomas (CRM) in children.Methods The initial clinical symptoms of children with TSC complicated by CRM confirmedin our hospital from January 2008 to November 2017.Pediatric echocardiography was performed to examine the heart from multiple directions and angles in the subxiphoid process,the apex of the heart,the side of the sternum and the superior thoracic fossa.The location,number,shape,size,activity and echo of the tumor were mainly observed after cardiac tumor was discovered.And finally,TSC was further confirmed by head MRI/CT,electrocardiogram,electroencephalogram and other examinations.Tumor resection was performed for cardiac tumors with surgical indications,and ultrasonic follow-up was performed for cardiac tumors without surgical indications.Results 34 cases of clinical definite cases,23 cases of epileptic seizure (68%); It was found that after cardiac tumor was first diagnose,that TSC 11 cases (32%) were confirmed after clinical and other examination.21 child patients were followed up,7 cases were further confirmed CRM by operation and pathology,and 8 cases shrank spontaneously,2 cases regressed completely,while another 4 cases had no obvious changes.Conclusion Epileptic seizure is the first symptom of TSC,so this kind of children should be routinely examined by echocardiography.The presence of TSC should be alert to the presence of multiple hyperechoic tumors in the left ventricle.Clinical combined with echocardiography is helpful for early diagnosis and treatment of this disease.
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Memo
收稿日期:2018-03-07。
通讯作者:蒋国平,Email:jiangguoping.168@zju.edu.cn