Brigitte Desprechins

Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity

Heterotaxy syndrome is a situs anomaly that comprises a large spectrum of cardiac and extracardiac malformations. Its association with intestinal malrotation is frequent. This later might be asymptomatic or manifest by signs of abdominal discomfort or of intestinal obstruction. We report on the case of a full term, small for gestational age neonate in whom heterotaxy syndrome with partial situs inversus abdominalis, intestinal malrotation, polysplenia and vena azygos continuity was diagnosed at prenatal echography. Due to the high risk of volvulus carried by the malrotation, Ladd’s procedure was performed at the age of two months. Follow-up examination at the age of four years shows excellent post-operative result and normal development of the child.

Paraspinal arterio-venous fistula in children: two more cases of an exceptional malformation

We report on the case of two toddlers who presented in the last 2 years with heart and vascular murmur, respectively, and in whom the diagnosis of paraspinal arterio-venous fistula was made. Paraspinal arterio-venous fistulae in children are extremely rare congenital or post-traumatic vascular malformations. In the rare case of connection with the spinal venous system, they might affect spinal vascularization due to potential venous congestion. Interventional embolization rather than surgery is the treatment of choice for such lesions. Up to now, there is no consensus about the indication of prophylactic closure of asymptomatic fistulae. However, close clinical follow-up with repeated spinal magnetic resonance imaging to exclude venous congestion is mandatory for young asymptomatic patients until treatment.

Right-sided pulmonary venous return to the inferior vena cava: an exceptional malformation.

Received 6 May 2016; revision accepted for publication 4 July 2016. An 8-year-old girl was examined for a third opinion because of a cardiac murmur. She complained about slightly reduced exercise tolerance. The first exam was described as normal. The second let suspect an atrial septum defect that could not be precisely located at echocardiography. Clinical examination and electrocardiography were suggestive of an atrial septum defect with a 2/6 systolic cardiac murmur of medium frequency over the pulmonary artery, a split second cardiac sound and electrical signs of right ventricular volume overload, respectively. Echocardiography showed normal situs. The right pulmonary veins could not be identified. The left pulmonary veins were correctly connected to the left atrium. The superior vena cava was normal. The inferior vena cava was dilated, as were the supra-hepatic veins. Right atrium, right ventricle, pulmonary trunk and pulmonary arteries were enlarged. The atrial septum was intact. In the sub-xyphoïdal view, colour Doppler examination allowed to follow a large vessel arising from the right lung that crossed the diaphragm and drained into Right-sided pulmonary venous return to the inferior vena cava: an exceptional malformation

Exercise-triggered chest pain as an isolated symptom of myocarditis in children

In childhood, chest pain occurring at exercise is a common complaint. A cardiac etiology for it is exceptionally found, explaining that most children do not undergo systematic cardiological investigation. However, chest pain at exercise may manifest as the unique symptom of a viral myocarditis. Recognizing this form of myocardial injury, however, might help to avoid clinical deterioration by providing adequate care. In this paper, we report on two children presenting with the unique clinical symptom of chest pain related to physical activity and in whom laboratory and cardiac investigations suggested transient myocardial damage related to myocarditis.