No abstract

Majority of adult SCD patients have delayed DTPA clearance unlike in inflammatory lung diseases, but similar to diabetes mellitus. DTPA clearance may be a useful modality for monitoring pulmonary involvement in SCD.

A two-month old Kuwaiti boy was admitted to our hospital for the first time with difficulties of feeding and tachypnea of two weeks' duration. He was born at term to healthy consanguineous parents. He is one of twins, the other being healthy; two brothers and two sisters are also healthy. Examination at that time revealed a dyspneic baby who was not cyanosed and had a heart rate of 150/min with a respiratory rate of 60/min and with intercostal recessions. No murmurs could be heard. There was significant hepatosplenomegaly, the liver being 4 cm and spleen 3 cm below the costal margin. Chest x-ray revealed cardiomegaly with a cardiothoracic ratio of 70%.Echocardiogram revealed left atrial dilatation. He was given antifailure therapy. He was seen again at the age of six months, at which time the symptoms were less, hepatosplenomegaly became less, and cardiomegaly persisted. An ejection systolic murmur was now heard over the left sternal border. His head was noticed to be increasing in size with a head circumference of 46 cm and a bruit was auscultated over the skull. Cranial ultrasound revealed hydrocephalus and a computed tomographic (CT) brain scan showed dilatation of the subarachnoid spaces over the frontal and anterior parts of temporal lobes. The lateral and third ventricles showed slight dilatation. On the nonenhanced scan, a mass of increased density was present in the left thalamic region extending across the midline, displacing the third ventricle to the right side. After enhancement enlarged and tortuous vertebral and basilar vessels could be seen, the vein of Galen was ectatic and the straight sinus dilated (Figure 1). There were large vessels in both thalamic regions. Angiography confirmed the diagnosis of a high flow AVM situated in the velum interpositum and supplied by large posteromedial chordoidal and thalamoperforating arteries from both sides and draining to an ectatic vein of Galen (Figure 2). The straight and transverse sinuses were dilated but there was no evidence of durai sinus stenosis or further abnormality.\Fig ure 1. CT scan of brain with contrast showing aneurysm of vein of Galen, dilated straight sinus, and ventriculomegaly. Aneurysm of the Vein of Galen Successfully Treated by Interventional Neuroradiology Annals of Saudi Medicine, Vol 12 No. 2; 1992No shunting procedure was performed for the hydrocephalus. At the age of eight months, the right posteromedial choroidal and thalamoperforating branches supplying the AVM were selectively injected using a microcatheter introduced through a guiding catheter in the right femoral artery and the angiomatous vessels supplied from them were ablated using isobutyl-cyanoacrylate (IBCA) (Figures 3 and 4). This was well tolerated and at the age of 12 months a similar procedure was successfully performed on the left posteromedial choroidal supply. After the emobilization, though four vessel angiography showed filling of a few dilated vessels from a left thalamoperforating artery, there was only minimal residual arteriovenous shunting and the intracr...

No abstract

No abstract