Affiliations: Department of Pathology and Laboratory Medicine,
University of Texas Health Science Center, Houston, TX, USA | Department of Pathology, Baylor College of Medicine,
Houston, TX, USA | Texas Children Hospital, Houston, TX, USA
Note: [] Correspondence: Dr. Min Wang, M.D., PhD, Department of Pathology
and Laboratory Medicine, University of Texas Health Science Center at Houston,
6431 Fannin Street, MBS 2.272, Houston, TX, USA. Tel.: +1 713 500 7874; Fax: +1
713 500 0733; E-mail: [email protected]
Abstract: Vein of Galen aneurysmal malformations (VGAMs) are rare congenital
vascular, malformation. The malformation usually develops between the 6th to
11th wk of gestation. We report three autopsy cases, all prenatally diagnosed
by ultrasound or fetal magnetic resonance imaging with VGAM with associated
high-output cardiac failure. Prenatal fetal echocardiogram on two patients
showed cardiomegaly, ventricular dilatation, pulmonary hypertension and
reversed aortic flow. The cause of death in all the three patients was
high-output cardiac failure due to VGAM. The autopsy findings confirmed feeding
arteries from posterior cerebral arteries to VGAM in all patients. Other
significant neuropathologic findings at autopsy were severe hydrocephalus,
polymicrogyria, germinal matrix hemorrhage, periventricular leukomalacia, and
microcalcification. The findings support that VGAM may be due to abnormal
arterial venous communication and the most common cause of death in these
patients is high-output cardiac failure. The presence of other associated brain
abnormalities indicates a poor prognosis.
Keywords: Vein of Galen, aneurysm, malformation, cardiac failure, diagnosis, autopsy
