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


CONCLUSIONS
Supraventricular arrhythmia may be associated with Haberland syndrome. It seems that propafenone is most effective in this condition.
Department of Cardiology, The Children's Memorial Health Institute, Dzieci Polskich 20, 04-730, Warsaw, Poland, kasiulapl@yahoo.com.
Katarzyna
K
Pregowska
Elzbieta
E
Jurkiewicz
Maria
M
Miszczak-Knecht
Anna
A
Turska-Kmiec
Katarzyna
K
Bieganowska
D002675:Child, Preschool; D005128:Eye Diseases / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007223:Infant; D008068:Lipomatosis / Q000150:complications / Q000175:diagnosis*; D008297:Male; D020752:Neurocutaneous Syndromes / Q000150:complications / Q000175:diagnosis*; D013617:Tachycardia, Supraventricular / Q000175:diagnosis / Q000209:etiology*