Abstract

A NEONATE WITH LEFT HEART TUMOUR AND CLINICAL SIGNS OF PIERRE - ROBIN SYNDROME - CASE REPORT AND REVIEW OF LITERATURE

Ramush Bejiqi*, Ragip Retkoceri, Hana Xhema-Bejiqi, Rinor Bejiqi,
Arlinda Maloku

Abstract

Cardiac tumours in infants and children are extremely rare, mostly are benign and differ in types when compared with those in adults. Their clinical manifestations vary widely from asymptomatic presentations to life-threatening cardiac events. Improvements in diagnostic techniques, such as those offered by echocardiography, have made early detection of cardiac masses possible, with or without the presence of clinical symptoms. Most of the benign tumours are rhabdomyoma, followed by teratoma, fibroma, oncocytic cardiomyopathy, vascular tumours and myxoma.[1] Pierre Robin syndrome is a congenital condition of facial abnormalities in humans. The three main features are cleft palate, retrognathia and glossoptosis. Rarely heart tumors are associated with syndromes, mostly are isolated. In this presentation we describe a 3-weeks old girl with Pierre-Robin syndrome and giant left ventricle tumour, diagnosed initially by transthoracic echocardiography. The purpose of this report is also to review the literature on the foetuses and neonate with cardiac tumours in an attempt to determine the various ways cardiac tumours differ clinically and morphologically in this age group from those occurring in older children and adults and to show that certain types of tumours have a better prognosis than others.

Keywords: Cardiac tumours, Pierre-Robin syndrome, echocardiography, prenatal diagnosis.