A Newborn with Cardiac Failure Secondary to a Large Vein of Galen Malformation

Sergio G. Golombek, MD; Shamiza Ally, MD; Paul K. Woolf, MD


South Med J. 2004;97(5) 

In This Article

Abstract and Case Report

We report the case of a term newborn with vein of Galen aneurysm. No antenatal diagnosis was done. The infant was admitted secondary to cardiac failure, with cardiomegaly and pulmonary hypertension. Physical examination was remarkable for acrocyanosis, microcephaly, cranial bruit, bilateral neck swelling, coarse breath sounds, and a hyperdynamic precordium with continuous murmur. Head ultrasound demonstrated a large, midline venous structure. Computed tomographic scan confirmed vein of Galen malformation.

A male infant was delivered vaginally to a 28-year-old primigravida at 38 weeks' gestation, after an uneventful pregnancy, at a community hospital. In the delivery room, the infant had respiratory distress, which did not improve with positive-pressure ventilation and required intubation. The infant's Apgar scores were 5, 6, and 6 at 1, 5, and 10 minutes, respectively, and his birthweight was 3,274 g.

Physical examination revealed a microcephalic infant with bilateral neck swelling. Coarse breath sounds and subcostal retractions were noted. Cardiovascular examination at the referring hospital was remarkable for a grade II/VI systolic murmur and a quiet precordium. Chest radiography revealed cardiomegaly. An echocardiogram obtained at 4 hours of life revealed a structurally normal heart, with evidence of persistent pulmonary hypertension of the newborn, tricuspid regurgitation, a large patent ductus arteriosus with a right-to-left shunt, and aortic isthmus narrowing, without coarctation. The infant was transferred to the regional neonatal center for further management.

At admission to the neonatal intensive care unit, physical examination revealed an intubated infant with acrocyanosis, with a temperature of 99.0°F; a heart rate of 154 beats/min; a respiratory rate of 60 breaths/min; and blood pressure of 74/46 mm Hg in the left arm, 61/40 mm Hg in the left leg, 66/37 mm Hg in the right arm, and 72/42 mm Hg in the right leg. Arterial blood gases (postductal) showed a pH of 7.33, a PCO2 of 39.9 mm Hg, a PaO2 of 43.6 mm Hg, an HCO3 of 20.8 mmol/L, a base deficit of 5.1 mmol/L, and an oxygen saturation of 91.7%. Examination of the head revealed molding and a cranial bruit heard through the anterior fontanel. A carotid bruit was auscultated over pulsatile neck vessels bilaterally. Breath sounds remained coarse over both lung fields, with intercostal retractions. Cardiovascular examination showed a hyperdynamic precordium with a grade II to III/VI harsh continuous murmur at the base. The liver was palpable 2 cm below the costal margin. Neurologic examination revealed intermittent tremors of upper and lower extremities.

Repeat echocardiography confirmed the aforementioned findings, with dilated atria and ventricles. A head ultrasound and CT scan were obtained to evaluate the cranial bruit. Despite mechanical ventilation with inhaled nitric oxide, the infant remained in significant cardiopulmonary distress due to congestive heart failure. The infant's poor prognosis was discussed with the parents and supportive care was withdrawn. The infant died 2 days after birth. An autopsy was performed.

A head ultrasound scan demonstrated a large venous structure in the midline connecting to other spaces in the posterior portion of the cranium. No normal cerebral tissue was seen, and hydranencephaly was suspected (Fig. 1). CT scan of the brain revealed a large, hourglass-shaped density, which enhanced intensely after contrast administration (Fig. 2). This was consistent with a large vein of Galen malformation, due to fistulous communication between the supraclinoid carotid arteries and an enlarged draining venous structure, which took up most of the supratentorial compartment (Fig. 3). Calcifications were seen throughout the subependyma. TORCH titers were negative.

Real-time ultrasonographic examination performed through the anterior fontanelle showing a large, ovoid vascular structure in the midline connecting the hypoechoic regions posteriorly. The midline structure measures approximately 3.0 × 3.4 × 4.0 cm and contains turbulent venous flow.

CT scan of the brain revealing a large, somewhat hourglass-shaped density occupying most of the midline supratentorial compartment, which is intensely enhanced after contrast administration.

CT three-dimensional image reconstruction showing findings consistent with vein of Galen malformation with little or no surrounding brain parenchyma.

The infant died as a result of congestive heart failure due to the vascular malformation. Autopsy confirmed a cerebral arteriovenous malformation associated with a vein of Galen aneurysm, measuring 2 cm in diameter, located beneath the splenium of the corpus callosum. The aneurysmal vessel wall was uniform in thickness. The brain weight (fixed) was 71 g (normal average brain weight at term, 362 g). Microcephaly, intracerebral (IC) hemorrhage, and hydrocephalus were also present, as a result of chronic hypoxic-ischemic cerebral necrosis.


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