Pediatr Cardiol 12:107-109, 1991 Pediatric Cardiology 9 Springer-Verlag New York Inc. 1991 Cerebral Arteriovenous Malformation Diagnosis by Two-Dimensional Color-Coded Doppler Ultrasonography of the Head J. Hegesh, J. Kuint, M. Frand, A. Setton, R. Tadmor, D. Nass, E. Kaplinsky, and M. Morro The Heart Institute, The Chaim Sheba Medical Center, Tel Hashomer, Israel SUMMARY. Two-dimensional color-coded Doppler examination revealed a cranial arterio- venous (AV) malformation in a critically ill newborn with intractable congestive heart failure. This case emphasizes the value of color-coded Doppler in the diagnosis of this rare malforma- tion. KEY WORDS: Arteriovenous malformation- Color-coded Doppler- Vein of Galen- Aneurysm -- Catheterization Case Report A male baby, weighing 2965 g, was born to a G2 PI 28-year-old mother. Fetal distress (type I deceleration) was observed prior to delivery. The baby was born hypotonic, with bradycardia of 80 beats/min and cyanosis. Apgar score was I at I min. Cardiopul- monary resuscitation was performed, ending in complete recov- ery by 5 min of age. Head circumference was 34 cm, and the temperature 36.6~ At 12 hours of age, he presented with difficulties in feed- ing and breathing. Physical examination revealed mild central cyanosis, tachypnea of 70/rain, pulse rate of 179 beats/rain, and blood pressure of 65/55 mmHg. Arterial oxygen saturation was 85%. The infant was severely ill. His skin color was pale gray, the peripheral pulses were hardly palpable, and his liver was en- larged 3 cm below the costal margin. The heart was hyper- dynamic and a soft systolic ejection murmur could be heard over the entire precordium and the base of the neck. A soft bruit was heard on the anterior fontanelle and right parietal regions of the skull. The electrocardiogram (ECG) showed extreme right ven- tricular hypertrophy. Chest x-ray showed an enlarged heart sil- houette, with hypervascularity of both lungs. Cardiac ultrasound examination, with the Hewlett-Packard color-coded ultrasound imaging system (77020 AC) and a 5-MHz transducer, at the age of 12 h showed an anatomically normal heart, with normal relation of the great vessels. The right atrium and right ventricle were markedly enlarged, with deviation of the atrial septum toward the left atrium. Right-to-left shunting through a small patent foramen ovale was defined blue by color- coded Doppler. The superior vena cava and the innominate vein were dilated compared to the inferior vena cava. The left atrial, Address offprint requests to." Dr. J. Hegesh, The Heart Institute, The Chaim Sheba Medical Center, Tel Hashomer 52621, Israel. left ventricular, and aortic dimensions were normal. The ductus arteriosus was patent, and a bidirectional shunt throughout sys- tole and diastole was detected in the ductus by pulsed and color- coded Doppler. Color-coded Doppler recorded a torrential high velocity (40 cm/s by pulsed Doppler) and turbulent flow through the superior vena cava toward the right atrium. Two-dimensional sonography of the skull with the same 5- MHz transducer, looking through the anterior fontanelle in the horizontal and left parasagittal view, revealed a large cystic echo-free zone located posteriorly and to the left of the third ventricle, consistent with an aneurysm of the vein of Galen. This vein could be visualized from the left posterooccipital region as well. Inside the cystic formation, color-coded Doppler was in- stantly visualized in deep blue, a low velocity continuous flow directed away from the transducer down to the jugular veins with a velocity of 20 cm/s, proving it to be a vascular formation. Furthermore, color-coded Doppler showed several vessels in yellow-red converging toward the echo-free cystic formation in which the flow ran toward the transducer. Pulsed Doppler with the cursor located in these vessels demonstrated a pulsating con- tinuous flow with a velocity of 55 cm/s, proving them to be the feeding arteries of the arteriovenous (AV) malformation (Fig. l). Computed tomographic (CT) scan with contrast revealed tortuous blood vessels mainly in the left hemisphere and a mid- line, markedly enlarged vein of Galen and transverse sinuses, consistent with an AV malformation of Galen. Symmetrical en- largement of the ventricles was also seen (Fig. 2). The infant died after 24 h. A vein of Galen malformation was proved by postmortem angiogram and at autopsy (Fig. 3). Discussion The clinical manifestation of an intracranial AV malformation in neonates can imitate congenital heart malformation by the presence of severe con-