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

Katarzyna Pregowska1, Elzbieta Jurkiewicz, Maria Miszczak-Knecht

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

European Journal of Pediatrics
|August 15, 2013
PubMed

Insights

Encephalocraniocutaneous lipomatosis (ECCL) can involve heart rhythm issues. Propafenone effectively treated supraventricular arrhythmia in a patient with ECCL, though neurological status declined over time.

Area of Science:

  • Pediatric Neurology
  • Clinical Genetics
  • Cardiology

Background:

  • Encephalocraniocutaneous lipomatosis (ECCL), also known as Haberland syndrome, is a rare congenital disorder characterized by malformations of the skin, eyes, bones, and central nervous system.
  • ECCL presents with a wide spectrum of clinical manifestations, often including neurological deficits.

Observation:

  • A 2-month-old boy diagnosed with ECCL exhibited mild physical and mental retardation.
  • Asymptomatic multifocal atrial tachycardia was detected via 24-hour Holter monitoring.
  • Initial treatment with digoxin and metoprolol failed to resolve the supraventricular arrhythmia.

Findings:

  • Propafenone administration successfully suppressed the supraventricular arrhythmia in the ECCL patient.
  • The patient maintained a sinus rhythm during a 3-year follow-up period.
  • Despite arrhythmia control, the patient's neurological status showed deterioration over the follow-up duration.

Implications:

  • Supraventricular arrhythmia is a potential comorbidity associated with Haberland syndrome.
  • Propafenone appears to be an effective therapeutic agent for managing supraventricular arrhythmia in ECCL.
  • This case highlights the complex interplay between neurological and cardiac manifestations in ECCL and the need for comprehensive management.
Abstract

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