Vasculitis

Vasculitis involving arterial structures is rare in children. However, inflammatory changes of arterial vessels can be detected in the context of Wegen-er's granulomatosis, Churg-Strauss syndrome (Fig. 12) and systemic connective tissue diseases such as lupus erythematodes (LE) or dermatomyositis [27]. Wegener's granulomatosis is a necrotizing vasculi-tis with granuloma formation that involves the respiratory tract and the kidneys [28].

The most common radiographic finding in children involves diffuse interstitial and alveolar opacities. CT detects centrilobular, perivascular faint, or groundglass opacities. MR imaging can demonstrate vascular irregularities on CE-MRA and focal wall enhancement or enhancement of the surrounding tissue in cases of inflammatory infiltrations (Fig. 13). In addition to specific laboratory findings, biopsy is usually required for definitive diagnosis.

Rvot Reconstruction

Fig. 10a-d. A 12 year old female patient with obstruction of the right ventricular outflow tract (RVOT). The MIP reconstruction of the arterial phase pulmonary MRA (Gd-BOPTA, 0.1 mmol/kg) (a) reveals a stenosis of the RVOT (arrow) and a subsequent dilatation of the pulmonary trunk (arrowhead). The corresponding surface reconstruction of a second acquisition in anterior-posterior view (b) demonstrates ectasia of the internal mammarian artery as one of numerous collateral vessels (arrow). Collateral blood flow takes place through intercostal vessels (arrowheads) with connection to a major aorto-pulmonary collateral artery (MAPCA), which is depicted on the VRT reconstruction in the posterior-anterior view (c, arrows). The connection between the aorta and the pulmonary artery is confirmed on axial multiplanar reconstructions (d, arrows)

Rvot Reconstruction

Fig. 10a-d. A 12 year old female patient with obstruction of the right ventricular outflow tract (RVOT). The MIP reconstruction of the arterial phase pulmonary MRA (Gd-BOPTA, 0.1 mmol/kg) (a) reveals a stenosis of the RVOT (arrow) and a subsequent dilatation of the pulmonary trunk (arrowhead). The corresponding surface reconstruction of a second acquisition in anterior-posterior view (b) demonstrates ectasia of the internal mammarian artery as one of numerous collateral vessels (arrow). Collateral blood flow takes place through intercostal vessels (arrowheads) with connection to a major aorto-pulmonary collateral artery (MAPCA), which is depicted on the VRT reconstruction in the posterior-anterior view (c, arrows). The connection between the aorta and the pulmonary artery is confirmed on axial multiplanar reconstructions (d, arrows)

Rvot ReconstructionRvot Reconstruction

Fig. 11a-c. MIP reconstruction (a) and surface rendering (b, c) of a 3D CE MRA dataset of a patient with pulmonary stenosis (arrowsin a, b) and subsequent post-stenostic dilatation of the pulmonary trunk (arrowheadsin a, b). Formation of a MAPCA (arrow) between the aortic arch and the left superior pulmonary artery is demonstrated on the surface rendered image in posterior-anterior view

Fig. 11a-c. MIP reconstruction (a) and surface rendering (b, c) of a 3D CE MRA dataset of a patient with pulmonary stenosis (arrowsin a, b) and subsequent post-stenostic dilatation of the pulmonary trunk (arrowheadsin a, b). Formation of a MAPCA (arrow) between the aortic arch and the left superior pulmonary artery is demonstrated on the surface rendered image in posterior-anterior view

Rvot Reconstruction

Fig. 12. A 14 year old patient with Churg-Strauss syndrome. On screening echocardiography a pathologic dilatation of the right ventricular outflow tract was found. MIP reconstruction of a pulmonary MRA demonstrates an aneurysm (arrows) of the RVOT

Rvot Reconstruction

Fig. 13a, b. MIP reconstruction of an abdominal MRA (Gd-BOPTA, 0.1 mmol/kg) in a 2 month old male baby. a The abdominal aorta shows a subtotal occlusion due to aortitis (arrowin a) in the area of the celiac trunk. Distal to the occlusion the diameter of the aorta is significantly reduced. Note the subsequent formation of various collaterals (arrowheadsin a, b) which establish blood supply distal to the stenosis through connections between the celiac trunk and the superior and inferior mesenteric artery

Fig. 13a, b. MIP reconstruction of an abdominal MRA (Gd-BOPTA, 0.1 mmol/kg) in a 2 month old male baby. a The abdominal aorta shows a subtotal occlusion due to aortitis (arrowin a) in the area of the celiac trunk. Distal to the occlusion the diameter of the aorta is significantly reduced. Note the subsequent formation of various collaterals (arrowheadsin a, b) which establish blood supply distal to the stenosis through connections between the celiac trunk and the superior and inferior mesenteric artery

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