P641 Management of cardiac rhabdomyoma in neonates. (June 2019)

• Record Type: Journal Article
• Authors: Charfi, Manel; Zaghdoud, Rim; Hamad, Amel Ben; Regaieg, Chiraz; Bouraoui, Amira; Regaieg, Ridha; Hmida, Nedia; Abid, Dorra; Kammoun, Samir; Thabet, Afef Ben; Gargouri, Abdellatif
• Is Part Of: Archives of disease in childhood. Volume 104:(2019)Supplement 3
• Page Start: A406
• Page End: A406
• Publication Date: 2019-06
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2019-epa.972
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Introduction: Cardiac tumors are rare in infant. Most commonly it's a Rhabdomyoma with an incidence of 0.02% to 0.08%. These cardiac rhabdomyomas are usually multiple with a preferential location in the ventricular myocardium (up to 94%). We aim to evaluate the epidemiology, clinical features, management and outcome of this rare condition in neonates. Methods: It's a retrospective study of all cases of cardiac rhabdomyomas registered in the neonatal intensive care unit of Sfax between 2009 and 2018. Results: Six full term newborns were included: one boy and five girls. Two of them were twins from a monochorial and monamniotic pregnancy. Prenatal diagnosis was performed in all cases. After birth, all newborns were asymptomatic. Physical exam revealed associated hemangiomas of the forearm in one case and hypomelanotic macules in 4 cases. Postnatal echocardiography confirmed the prenatal findings in all cases. Multiple lesions were noted in 4 cases. Ventricular location was found in all cases. Associated interventricular septum tumor was found in one case as well as a right atrium tumor in one other case. The mean tumor size was 16 *10 mm. No hemodynamic disorders were noted in all cases. The ophthalmologic and renal assessment was normal for all newborns. Brain MRI was performed in 4 cases. It revealed white matter anomalies and subependymal nodules confirming then the diagnosis of tuberous sclerosis in two patients. During follow up one of them developed focal seizures fully controlled by antiepileptic drugs. The evolution was favorable for all patients. All tumors regressed spontaneously within an average of three years. Conclusion: Cardiac rhabdomyomas are benign tumors which tend to spontaneously regress during early childhood. However they may lead to arrhythmias or hemodynamic disorders due to ventricular inflow/outflow tract obstruction. Thus long term ultrasound follow up is needed. Moreover diagnosis of cardiac rhabdomyoma must lead clinician to look systematically for an associated tuberous sclerosis as it can be its earliest manifestation.