Vein of Galen malformation and interrupted aortic arch in a neonate: a previously unreported association.

Ann Saud Med. 2005;25(3):258–261 The arteriovenous malformation of the vein of Galen is a rare entity in neonates. Its association with other major cardiac anomalies is even more uncommon. We report the first case of interrupted aortic arch associated with the vein of Galen malformation in the English literature. e possible etiology, diagnosis and management of this rare combination are discussed. e location of vein of Galen is under the cerebral hemispheres. e function of the vein of Galen is the drainage of the anterior and central regions of the brain into the sinuses of the posterior cerebral fossa. Originating from the median vein of the prosencephalon, the vein of Galen is formed by the union of the two internal cerebral veins and goes on to drain into the straight sinus. An arterioveneous malformation occurs when either directly or via an interposed angiomatous malformation the vein of Galen receives an arterial input from one or more major intracranial arteries resulting in arteriovenous shunting of blood. In reality, the term vein of Galen malformation should not be used any more. e malformation is due to the fact that the precise defect is now known to be a persistent embryonic prosencephalic vein of Markowski developing during 6 to 11 weeks of gestation, which actually drains into the vein of Galen. e vein of Galen malformation, therefore, is a misnomer. Advances in diagnostic and therapeutic modalities have considerably altered the perception of vein of Galen malformation manifesting in the neonatal period, from a condition with uniformly grave morbidity and mortality to a favorable one,1-2 except in the most severe cases. As intractable cardiac failure is a common denominator in a majority of neonatal cases,3 and the coexistence of any significant congenital heart disease that can imperil the already jeopardized cardiac status portrays a dismal outcome.3-4 Only twenty-three (23) cases of vein of Galen malformation coexisting with a congenital cardiac defect have been reported, including five patients by McElhinney5 in his review of 1998. Although coarctation of aorta has been noted in 20% (9/43) of cases of vein of Galen malformation, to our knowledge this is the first case to be reported in a neonate with arteriovenous malformation of the vein of Galen coexistent with the interrupted aortic arch defect.