[Aneurysm of the vein of Galen and cardiac insufficiency. Current therapeutic approach]. / Anévrisme de l'ampoule de Galien et insuffisance cardiaque. Attitude thérapeutique actuelle.

ABSTRACT

Aneurysm of the ampulla of Galien is a rare but serious cause of cardiac failure in the neonate or child. The diagnosis is suspected on finding a continuous murmur on auscultation of the skull. It is an intracranial arterio-venous malformation which is sometimes responsible for a very important shunt between the arterial (carotid or vertebral) and venous systems. The vital and neurological prognosis of these children is classically very poor. The authors report the cases of the last three consecutive children aneurysms of the ampulla of Galien followed up at Grenoble Hospital in the last five years. The clinical presentations were very different, corresponding to the usual three forms described in the literature a reputedly very severe form with cardiac failure at the 3rd day of life requiring multiple percutaneous embolisations; a neonatal form revealed by an isolated macrocranium; and a form diagnosed late (4.5 years) in the investigation of effort dyspnoea which regressed after two sessions of embolisation. All three children are alive and have normal psychomotor development for their age. A multidisciplinary approach involving neonatal physicians, paediatric cardiologists and neurologists, with complementary non-invasive investigations such as transfontanellar ultrasonography, electroencephalography, MRI and echocardiography are necessary to optimise the management and limit the neurological sequellae in children with this type of malformation. Cerebral arteriography and percutaneous embolisation under general anaesthesia by a trained team gave very good medium-term results in two of these cases, lightening the usual pessimism surrounding this condition.