Large vessel vasculopathy: An underrecognized complication in Wiskott–Aldrich syndrome

Figures

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  Figure 1.. Panel of CT angiography images showing aneurysmal dilataion of aorta and its branches with development of collaterals and mediatstinal lymphadenopathy in patient 1. (a–d) Panel of CT angiogram images of patient 1: reconstructed coronal images (a and b) show focal stenosis at the origin of the right brachiocephalic trunk (solid white arrow below the arrowhead) with poststenotic dilation (arrowhead). Right common carotid (solid white arrow above the arrowhead in a) and subclavian (arrows in b) are normal. Reconstructed coronal image (c) shows nonopacified (occluded) proximal left subclavian artery until the origin of the vertebral artery (arrow). Note the left common carotid artery is also not opacified. Conglomerate necrotic lymph nodes are seen in right paratracheal location (thick arrow in c). Axial image (d) shows circumferential mural thickening of the ascending and descending thoracic aorta with mural calcifications (thin arrows). Note necrotic subcarinal lymphadenopathy (thick arrow) and right pleural effusion (asterisk). (e and f) CT angiogram volume rendered (e) and maximum-intensity projection (f) images show aneurysmal dilatation of the aorta extending from the aortic…

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  Figure 2.. Panel of CT angiography images demonstrating vascular involvement of thoracic and abdominal vessels in Patient 2. (a–e) CT angiography of the thorax and abdomen of patient 2: coronal (a), sagittal (b), and axial (c–e) sections demonstrating the fusiform and irregular outline of the abdominal aorta (long red arrow in b) extending from the diaphragmatic hiatus to its bifurcation at the L5 vertebral level with a maximum transverse caliber of 2.7 cm. A thick rim of hypodense soft tissue is seen surrounding the aneurysmal segment suggestive of partial thrombosis (green arrow mark). Variable lengths of proximal parts of the celiac trunk (upper short red arrow in b), superior mesenteric artery (lower short red arrow in b), and left renal artery show fusiform dilatation. Aortic root, ascending aorta, aortic arch (c) and its branches (arrowhead), and descending thoracic aorta are normal in course, caliber, and contrast opacification. The two renal arteries are seen on the right side, and the inferior mesenteric artery is normal in course, caliber, and outline.

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  Figure 3.. Panel of CT angiogram images showing vascular involvement and stenotic lesions and PET scan revealing non-necrotic lympadenopathy in patient 3. (a, c, and d) CT angiography of abdominal arteries of patient 3 shows concentric wall thickening from the level of the aortic hiatus, the entire suprarenal and renal segments (arrows labelled as 1 to 4 in a to d), and the short infrarenal segment of the abdominal aorta for a length of 9.0–9.5 cm and maximum wall thickness of 3.2 mm. (b–d) Mild eccentric narrowing at the celiac artery origin of about 20–30% stenosis, severe short-segmental stenosis/near-total occlusion at the origin of the anterior right renal artery (c), and long segmental occlusion for a length of 14 mm of the posterior right renal artery (d). The rest of the infrarenal abdominal aorta, aortic bifurcation, and both iliac arteries appear normal in course, caliber, outline, and branching pattern. (e and f) Right kidney (measures 7.5 cm) shows mild diffuse shrinkage in volume with mild hypoperfusion of the right kidney with delayed excretion, whereas the left kidney (measures 11.1 cm) shows compensatory enlargement, and normal perfusion and normal excretion. (g) Positron…

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  Figure 4.. Panel of CT angiograms showing extent of vascular involvement in patient 4. (a–f) CT angiography of thoracic and abdominal vessels shows long segmental occlusion/tight stenosis of the entire length of the left subclavian artery with associated mild circumferential mural thickening. Left vertebral and left axillary arteries show attenuated caliber. Mild circumferential mural thickening involving the right and left common carotid arteries from the origin to bifurcation (c). Aortic root, ascending aorta, arch of aorta, descending thoracic aorta, abdominal aorta and its branches, bilateral common iliac arteries, bilateral external iliac arteries, and bilateral internal iliac arteries are normal in caliber and opacification (d–f).