Giant dural supratentorial arteriovenous malformation

Abstract

This 3,700 g male infant was the product of an uncomplicated pregnancy, born to a 23-year-old, gravida 2, pama 1 mother. Apgar scone was 9 at 1 mm and 10 at 5 mm. At age 36 hn, the infant was noted to have respiratory difficulty. Examination mevealed moderate intercostalretractionswith a respiratorymateof 80/mm. Pulse rate was 150/mm, and peripheral pulses were normal in volume. Head circumference was 38.5 cm, the antenon fontanelle was full and measured 4 x 4 cm, and the postenor fontanelle was 4 x 3 cm. Coronal and sagittal sutures were spread, and the pamietal and frontal bones were separated by 1 cm. Scalp veins were prominent over the frontal and panietal regions, and a continuous bruit was heard over the temporal and occipital areas bilaterally. There was no transillumination of the skull. The pmecondium was hypendynamic, and a grade Ill/VI systolic ejection murmur was heard over the left upper sternal border. The second heart sound was normal. The liver edge was 3 cm below the night costal margin, and the spleen tip was palpable. Skull nadiognaphs (fig. 1) showed widely separated sutures and intracranial calcifications. Chest films revealed generalized candiomegaly and increased pulmonary vasculatune. The electrocandiogmam showed a normal axis and evidence of biventniculam hypertnophy. Hematocnit was 46%; white blood cell count, 12,700; and platelet count, 28,000. After digitalis and diuretic therapy for congestive heart failume, a bilateral carotid angiogram was performed (fig. 2) which showed a huge dunal venous sac fed by markedly dilated extennal carotid arteries. The vertebral arteries could not be catheterized because of extreme tortuosity. Because of a deteriorating cardiovascular status, both extennal carotid arteries were ligated in an attempt to control the patient’s congestive heart failure. After initial improvement, the infant developed a decrease in cardiac output, uncontrollable acidosis, and, despite all measures to support him, expired 48 hr after surgery. At postmortem examination, a 7 cm saclike cavity was found within the interhemisphenic duna. There was no evidence of hydrocephalus. The straight sinus and torcula were low in position, and the posterior fossa was small due to the large dunal arteniovenous malformation. The fomamen spinosum was markedly enlarged bilaterally due to the large flow through the middIe meningeal vessels. Discussion