Persistent multifocal atrial tachycardia in infant with encephalocraniocutaneous lipomatosis: a case report

被引:0
作者
Katarzyna Pregowska
Elzbieta Jurkiewicz
Maria Miszczak-Knecht
Anna Turska-Kmiec
Katarzyna Bieganowska
机构
[1] The Children’s Memorial Health Institute,Department of Cardiology
[2] The Children’s Memorial Health Institute,Department of Diagnostic Imaging
来源
European Journal of Pediatrics | 2014年 / 173卷
关键词
Multifocal atrial tachycardia; Haberland syndrome; Children; Arrhythmia;
D O I
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摘要
Encephalocraniocutaneous lipomatosis (ECCL, Haberland syndrome, Fishman syndrome) is a very rare congenital disorder, involving skin, eye, bone and central nervous system malformations. In this paper we present a case of a 2-month-old boy with encephalocraniocutaneous lipomatosis diagnosed on the basis of characteristic clinical manifestations and neuroimaging findings. Neurologically, the child presented only with mild physical and mental retardation. 24-h Holter monitoring revealed asymptomatic multifocal atrial tachycardia. Initial therapy with digoxin and metoprolol was not effective. Introduction of propafenone resulted in supression of supraventricular arrhythmia. During the 3- years follow-up, sinus rhythm persisted, but neurological status deteriorated. Conclusion: Supraventricular arrhythmia may be associated with Haberland syndrome. It seems that propafenone is most effective in this condition.
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页码:1577 / 1580
页数:3
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