Antenatal sonographic features of aneurysmal dilatation of a vitelline vein.

We report a case of aneurysmal dilatation of a vitelline vein observed antenatally. Intra-abdominal vascular dilatation was diagnosed on ultrasound examination at 24 weeks' gestation. The relationship with the umbilicus and portal vein suggested the diagnosis of umbilical vein varix. Fetal tolerance remained excellent in spite of a gradual increase in the size of the dilated vein. Postnatal ultrasound examination revealed thrombosis of the aneurysm with gradual extension to the portal vein and the onset of serious coagulation problems. Operative findings on postnatal day 9 included the absence of intra-abdominal umbilical vein, and the presence of an abnormal, dilated and thrombosed vein connecting the umbilicus to the portal vein and following the trajectory of the right vitelline vein. Corrective surgery was attempted by resection of the aneurysm and portal thrombectomy, but this did not prevent the development of portal obstruction syndrome with cavernous hemangioma. This anomaly, in which the fetal venous return uses the vitelline vein in the absence of the umbilical vein, does not appear to have been described before. The mechanism in question could be anastomosis between the right vitelline vein and umbilical vein. Antenatal diagnosis should enable early surgical management before the formation of a portal thrombosis.

[Antenatal diagnosis and management of vein of Galen aneurysm: review illustrated by a case report]. / Diagnostic anténatal et prise en charge d'un anévrysme de la veine de Galien: revue de la littérature illustrée par une observation personnelle.

Vein of Galen aneurysm is a rare vascular congenital malformation. We report an antenatal diagnosis with duplex Doppler at 26 weeks gestation. Magnetic resonance imaging was used before and after delivery in order to determine neurological prognosis. The neonatal prognosis is poor if fetal cardiac insufficiency or cerebral lesions are present antenatally. Premature delivery does not improve the neonatal outcome. When the aneurysm is not life threatening, embolization of the malformation is at best delayed five months after birth.

Clinical, biochemical, magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (1H MRS) findings in a fourth case of combined D- and L-2 hydroxyglutaric aciduria.

We report the fourth case of combined D-and L-2-hydroxyglutaric aciduria presenting with neonatal encephalopathy and subependymal cysts.