Persistent multifocal atrial tachycardia in infant with encephalocraniocutaneous lipomatosis: a case report
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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.
Keywords
Multifocal atrial tachycardia Haberland syndrome Children ArrhythmiaNotes
Acknowledgments
The study was not sponsored or supported by any organization other than The Children's Memorial Health Institute, Warsaw, Poland.
Conflict of interest
There are no conflicts of interest related to this publication.
References
- 1.Bradley DJ, Fishbach PS, Law IH, Serwer GA, Dick M 2nd (2001) The clinical course of multifocal atrial tachycardia in infants and children. J Am Coll Cardiol 38(2):401–408PubMedCrossRefGoogle Scholar
- 2.Gawel J, Schwartz RA, Jóźwiak S (2003) Encephalocraniocutaneous lipomatosis. J Cutan Med Surg 7(1):61–65PubMedCrossRefGoogle Scholar
- 3.Gokhale NR, Mahajan PM, Belgaumkar VA, Pradhan SN, Uttarwar NS (2007) Encephalocraniocutaneous lipomatosis: a rare neurocutaneous syndrome. Indian J Dermatol Venereol Leprol 73(1):40–42PubMedCrossRefGoogle Scholar
- 4.Haberland C, Perou M (1970) Encephalocraniocutaneous lipomatosis. A new example of ectomesodermal dysgenesis. Arch Neurol 22(2):144–155PubMedCrossRefGoogle Scholar
- 5.Jóźwiak S, Pascual-Casroviejo I (2008) Encephalocraniocutaneous lipomatosis (Haberland syndrome). In: Ruggieri M, Pascual Castroviejo I, Di Rocco C (eds) Neurocutaneous disorders: Phakomatoses and hamartoneoplastic syndromes. Springer, Wien, pp 517–525Google Scholar
- 6.Southall DP, Johnson AM, Shinebourne EA, Johnston PG, Vulliamy DG (1981) Frequency and outcome of disorders of cardiac rhythm and conduction in a population of newborn infants. Pediatrics 68(1):58–66PubMedGoogle Scholar