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Eur J Pediatr. 2014 Dec;173(12):1577-80. doi: 10.1007/s00431-013-2103-7. Epub 2013 Aug 14.

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

Author information

1
Department of Cardiology, The Children's Memorial Health Institute, Dzieci Polskich 20, 04-730, Warsaw, Poland, kasiulapl@yahoo.com.

Abstract

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.

PMID:
23942743
DOI:
10.1007/s00431-013-2103-7
[Indexed for MEDLINE]
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