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Imaging of Marfan Syndrome: Multisystemic Manifestations¹

¹ From the Departments of Radiology and the Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, 388-1 Pungnap-2 dong, Songpa-gu, Seoul 138-736, Korea. Presented as an education exhibit at the 2005 RSNA Annual Meeting. Received September 18, 2006; revision requested October 24 and received December 18; accepted December 18. All authors have no financial relationships to disclose.

View larger version (212K): [in this window] [in a new window] [Download PPT slide]   Figure 1a.  Histologic specimens from the aorta in a 24-year-old man show evidence of cystic medial necrosis. (a) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows deposition of mucopolysaccharide (*). (b) Photomicrograph (original magnification, x100; Masson trichrome stain) demonstrates disruption of the elastic lamina.

 

View larger version (221K): [in this window] [in a new window] [Download PPT slide]   Figure 1b.  Histologic specimens from the aorta in a 24-year-old man show evidence of cystic medial necrosis. (a) Photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows deposition of mucopolysaccharide (*). (b) Photomicrograph (original magnification, x100; Masson trichrome stain) demonstrates disruption of the elastic lamina.

 

View larger version (132K): [in this window] [in a new window] [Download PPT slide]   Figure 2.  Annuloaortic ectasia without valvular insufficiency in a 44-year-old woman. Multiplanar reformatted CT image shows the abnormal aortic root (double-ended arrow) with dilatation of the Valsalva sinus and an indistinct sinotubular junction.

 

View larger version (134K): [in this window] [in a new window] [Download PPT slide]   Figure 3a.  Annuloaortic ectasia without valvular insufficiency in a 20-year-old man. Multiplanar reformatted CT images from the systolic (a) and diastolic (b) phases show dilated aortic sinuses, tethering of leaflets (arrowheads in a), and the absence of a coaptation defect (* in b). Echocardiography helped confirm the absence of aortic valve insufficiency.

 

View larger version (133K): [in this window] [in a new window] [Download PPT slide]   Figure 3b.  Annuloaortic ectasia without valvular insufficiency in a 20-year-old man. Multiplanar reformatted CT images from the systolic (a) and diastolic (b) phases show dilated aortic sinuses, tethering of leaflets (arrowheads in a), and the absence of a coaptation defect (* in b). Echocardiography helped confirm the absence of aortic valve insufficiency.

 

View larger version (130K): [in this window] [in a new window] [Download PPT slide]   Figure 4a.  Annuloaortic ectasia with severe valvular insufficiency in a 32-year-old man. (a) Reformatted CT image shows marked dilatation of the Valsalva sinus and the sinotubular junction (arrowheads). (b, c) Reformatted CT images from the systolic (b) and diastolic (c) phases show dilated aortic sinuses, tethering of leaflets (arrowheads in b), and a central coaptation defect (* in c). (d) Postoperative reformatted CT image, obtained after aortic valve–sparing aortic annuloplasty, shows a normal appearance of the aortic root. Arrows indicate the prosthetic ring used to reconstruct the annulus. (e, f) Reformatted CT images from the systolic (e) and diastolic (f) phases show relief of tethering of leaflets (arrowheads in e) and disappearance of the central coaptation defect.

 

View larger version (164K): [in this window] [in a new window] [Download PPT slide]   Figure 4b.  Annuloaortic ectasia with severe valvular insufficiency in a 32-year-old man. (a) Reformatted CT image shows marked dilatation of the Valsalva sinus and the sinotubular junction (arrowheads). (b, c) Reformatted CT images from the systolic (b) and diastolic (c) phases show dilated aortic sinuses, tethering of leaflets (arrowheads in b), and a central coaptation defect (* in c). (d) Postoperative reformatted CT image, obtained after aortic valve–sparing aortic annuloplasty, shows a normal appearance of the aortic root. Arrows indicate the prosthetic ring used to reconstruct the annulus. (e, f) Reformatted CT images from the systolic (e) and diastolic (f) phases show relief of tethering of leaflets (arrowheads in e) and disappearance of the central coaptation defect.

 

View larger version (162K): [in this window] [in a new window] [Download PPT slide]   Figure 4c.  Annuloaortic ectasia with severe valvular insufficiency in a 32-year-old man. (a) Reformatted CT image shows marked dilatation of the Valsalva sinus and the sinotubular junction (arrowheads). (b, c) Reformatted CT images from the systolic (b) and diastolic (c) phases show dilated aortic sinuses, tethering of leaflets (arrowheads in b), and a central coaptation defect (* in c). (d) Postoperative reformatted CT image, obtained after aortic valve–sparing aortic annuloplasty, shows a normal appearance of the aortic root. Arrows indicate the prosthetic ring used to reconstruct the annulus. (e, f) Reformatted CT images from the systolic (e) and diastolic (f) phases show relief of tethering of leaflets (arrowheads in e) and disappearance of the central coaptation defect.

 

View larger version (137K): [in this window] [in a new window] [Download PPT slide]   Figure 4d.  Annuloaortic ectasia with severe valvular insufficiency in a 32-year-old man. (a) Reformatted CT image shows marked dilatation of the Valsalva sinus and the sinotubular junction (arrowheads). (b, c) Reformatted CT images from the systolic (b) and diastolic (c) phases show dilated aortic sinuses, tethering of leaflets (arrowheads in b), and a central coaptation defect (* in c). (d) Postoperative reformatted CT image, obtained after aortic valve–sparing aortic annuloplasty, shows a normal appearance of the aortic root. Arrows indicate the prosthetic ring used to reconstruct the annulus. (e, f) Reformatted CT images from the systolic (e) and diastolic (f) phases show relief of tethering of leaflets (arrowheads in e) and disappearance of the central coaptation defect.

 

View larger version (151K): [in this window] [in a new window] [Download PPT slide]   Figure 4e.  Annuloaortic ectasia with severe valvular insufficiency in a 32-year-old man. (a) Reformatted CT image shows marked dilatation of the Valsalva sinus and the sinotubular junction (arrowheads). (b, c) Reformatted CT images from the systolic (b) and diastolic (c) phases show dilated aortic sinuses, tethering of leaflets (arrowheads in b), and a central coaptation defect (* in c). (d) Postoperative reformatted CT image, obtained after aortic valve–sparing aortic annuloplasty, shows a normal appearance of the aortic root. Arrows indicate the prosthetic ring used to reconstruct the annulus. (e, f) Reformatted CT images from the systolic (e) and diastolic (f) phases show relief of tethering of leaflets (arrowheads in e) and disappearance of the central coaptation defect.

 

View larger version (165K): [in this window] [in a new window] [Download PPT slide]   Figure 4f.  Annuloaortic ectasia with severe valvular insufficiency in a 32-year-old man. (a) Reformatted CT image shows marked dilatation of the Valsalva sinus and the sinotubular junction (arrowheads). (b, c) Reformatted CT images from the systolic (b) and diastolic (c) phases show dilated aortic sinuses, tethering of leaflets (arrowheads in b), and a central coaptation defect (* in c). (d) Postoperative reformatted CT image, obtained after aortic valve–sparing aortic annuloplasty, shows a normal appearance of the aortic root. Arrows indicate the prosthetic ring used to reconstruct the annulus. (e, f) Reformatted CT images from the systolic (e) and diastolic (f) phases show relief of tethering of leaflets (arrowheads in e) and disappearance of the central coaptation defect.

 

View larger version (112K): [in this window] [in a new window] [Download PPT slide]   Figure 5.  Abdominal aortic aneurysm in a 27-year-old man with annuloaortic ectasia. Maximum intensity projection CT image shows fusiform dilatation of the infrarenal abdominal aorta (arrowheads).

 

View larger version (92K): [in this window] [in a new window] [Download PPT slide]   Figure 6a.  Triple-barreled aortic dissection in a 41-year-old man after surgical replacement of the ascending aorta for a DeBakey type I aortic dissection. (a, b) Axial CT scans (a at a level higher than b) show a dissection of the thoracic aorta. (c, d) Axial CT scans obtained 5 months later (c at a level higher than d) show complex dissection flaps (arrowheads).

 

View larger version (163K): [in this window] [in a new window] [Download PPT slide]   Figure 6b.  Triple-barreled aortic dissection in a 41-year-old man after surgical replacement of the ascending aorta for a DeBakey type I aortic dissection. (a, b) Axial CT scans (a at a level higher than b) show a dissection of the thoracic aorta. (c, d) Axial CT scans obtained 5 months later (c at a level higher than d) show complex dissection flaps (arrowheads).

 

View larger version (108K): [in this window] [in a new window] [Download PPT slide]   Figure 6c.  Triple-barreled aortic dissection in a 41-year-old man after surgical replacement of the ascending aorta for a DeBakey type I aortic dissection. (a, b) Axial CT scans (a at a level higher than b) show a dissection of the thoracic aorta. (c, d) Axial CT scans obtained 5 months later (c at a level higher than d) show complex dissection flaps (arrowheads).

 

View larger version (173K): [in this window] [in a new window] [Download PPT slide]   Figure 6d.  Triple-barreled aortic dissection in a 41-year-old man after surgical replacement of the ascending aorta for a DeBakey type I aortic dissection. (a, b) Axial CT scans (a at a level higher than b) show a dissection of the thoracic aorta. (c, d) Axial CT scans obtained 5 months later (c at a level higher than d) show complex dissection flaps (arrowheads).

 

View larger version (117K): [in this window] [in a new window] [Download PPT slide]   Figure 7a.  Bentall procedure in a 42-year-old man with a Stanford type A aortic dissection. (a) Preoperative reformatted CT image shows a thin intimal flap in the ascending aorta (arrow). (b) Postoperative reformatted CT image shows the composite graft and mechanical aortic valve used to replace the aortic root, as well as the reimplanted left coronary artery (arrow), which is attached to the composite graft.

 

View larger version (142K): [in this window] [in a new window] [Download PPT slide]   Figure 7b.  Bentall procedure in a 42-year-old man with a Stanford type A aortic dissection. (a) Preoperative reformatted CT image shows a thin intimal flap in the ascending aorta (arrow). (b) Postoperative reformatted CT image shows the composite graft and mechanical aortic valve used to replace the aortic root, as well as the reimplanted left coronary artery (arrow), which is attached to the composite graft.

 

View larger version (126K): [in this window] [in a new window] [Download PPT slide]   Figure 8.  Dilatation of the main pulmonary artery in a 16-year-old boy. Maximum intensity projection CT image at the level of the Valsalva sinus shows dilatation of the main pulmonary artery trunk to a diameter of 41 mm, as well as annuloaortic ectasia with a sinus diameter of 57 mm.

 

View larger version (69K): [in this window] [in a new window] [Download PPT slide]   Figure 9a.  Scoliosis in a 17-year-old boy. (a) Preoperative standing radiograph of the whole spine shows thoracolumbar scoliosis with a leftward curvature of 54° (Lippman-Cobb method). (b) Postoperative standing radiograph shows a reduced thoracolumbar curvature of 30°.

 

View larger version (63K): [in this window] [in a new window] [Download PPT slide]   Figure 9b.  Scoliosis in a 17-year-old boy. (a) Preoperative standing radiograph of the whole spine shows thoracolumbar scoliosis with a leftward curvature of 54° (Lippman-Cobb method). (b) Postoperative standing radiograph shows a reduced thoracolumbar curvature of 30°.

 

View larger version (91K): [in this window] [in a new window] [Download PPT slide]   Figure 10a.  Scoliosis in an 18-year-old man. (a) Preoperative reformatted CT image obtained to plan the positioning of screws. (b, c) Preoperative axial T2-weighted (b) and coronal T2-weighted fast spin-echo (c) MR images obtained to evaluate the spinal cord and nerve root and the relationships of the spinal cord, pedicles, and vertebral bodies. (d, e) Postoperative coronal (d) and sagittal (e) reformatted CT images, obtained to evaluate the placement of instrumentation and to identify complications, show proper positioning of the screws.

 

View larger version (162K): [in this window] [in a new window] [Download PPT slide]   Figure 10b.  Scoliosis in an 18-year-old man. (a) Preoperative reformatted CT image obtained to plan the positioning of screws. (b, c) Preoperative axial T2-weighted (b) and coronal T2-weighted fast spin-echo (c) MR images obtained to evaluate the spinal cord and nerve root and the relationships of the spinal cord, pedicles, and vertebral bodies. (d, e) Postoperative coronal (d) and sagittal (e) reformatted CT images, obtained to evaluate the placement of instrumentation and to identify complications, show proper positioning of the screws.

 

View larger version (89K): [in this window] [in a new window] [Download PPT slide]   Figure 10c.  Scoliosis in an 18-year-old man. (a) Preoperative reformatted CT image obtained to plan the positioning of screws. (b, c) Preoperative axial T2-weighted (b) and coronal T2-weighted fast spin-echo (c) MR images obtained to evaluate the spinal cord and nerve root and the relationships of the spinal cord, pedicles, and vertebral bodies. (d, e) Postoperative coronal (d) and sagittal (e) reformatted CT images, obtained to evaluate the placement of instrumentation and to identify complications, show proper positioning of the screws.

 

View larger version (60K): [in this window] [in a new window] [Download PPT slide]   Figure 10d.  Scoliosis in an 18-year-old man. (a) Preoperative reformatted CT image obtained to plan the positioning of screws. (b, c) Preoperative axial T2-weighted (b) and coronal T2-weighted fast spin-echo (c) MR images obtained to evaluate the spinal cord and nerve root and the relationships of the spinal cord, pedicles, and vertebral bodies. (d, e) Postoperative coronal (d) and sagittal (e) reformatted CT images, obtained to evaluate the placement of instrumentation and to identify complications, show proper positioning of the screws.

 

View larger version (59K): [in this window] [in a new window] [Download PPT slide]   Figure 10e.  Scoliosis in an 18-year-old man. (a) Preoperative reformatted CT image obtained to plan the positioning of screws. (b, c) Preoperative axial T2-weighted (b) and coronal T2-weighted fast spin-echo (c) MR images obtained to evaluate the spinal cord and nerve root and the relationships of the spinal cord, pedicles, and vertebral bodies. (d, e) Postoperative coronal (d) and sagittal (e) reformatted CT images, obtained to evaluate the placement of instrumentation and to identify complications, show proper positioning of the screws.

 

View larger version (86K): [in this window] [in a new window] [Download PPT slide]   Figure 11a.  Pectus excavatum and annuloaortic ectasia in a 28-year-old man. (a) Axial CT scan obtained at the level of the aortic root shows severe retraction of the sternum (CT index, 8.5) with resultant compression of the left atrium (*). Marked dilatation of the aortic root also is visible. (b) Postoperative chest radiograph shows a metallic bar positioned beneath the sternum, across the anterior thoracic wall (Nuss procedure), and a mechanical aortic valve replacement. (c) Postoperative axial CT scan obtained at the level of the aortic root shows an increased anteroposterior diameter of the thorax and relief of left atrial compression (*).

 

View larger version (155K): [in this window] [in a new window] [Download PPT slide]   Figure 11b.  Pectus excavatum and annuloaortic ectasia in a 28-year-old man. (a) Axial CT scan obtained at the level of the aortic root shows severe retraction of the sternum (CT index, 8.5) with resultant compression of the left atrium (*). Marked dilatation of the aortic root also is visible. (b) Postoperative chest radiograph shows a metallic bar positioned beneath the sternum, across the anterior thoracic wall (Nuss procedure), and a mechanical aortic valve replacement. (c) Postoperative axial CT scan obtained at the level of the aortic root shows an increased anteroposterior diameter of the thorax and relief of left atrial compression (*).

 

View larger version (82K): [in this window] [in a new window] [Download PPT slide]   Figure 11c.  Pectus excavatum and annuloaortic ectasia in a 28-year-old man. (a) Axial CT scan obtained at the level of the aortic root shows severe retraction of the sternum (CT index, 8.5) with resultant compression of the left atrium (*). Marked dilatation of the aortic root also is visible. (b) Postoperative chest radiograph shows a metallic bar positioned beneath the sternum, across the anterior thoracic wall (Nuss procedure), and a mechanical aortic valve replacement. (c) Postoperative axial CT scan obtained at the level of the aortic root shows an increased anteroposterior diameter of the thorax and relief of left atrial compression (*).

 

View larger version (130K): [in this window] [in a new window] [Download PPT slide]   Figure 12a.  Pectus carinatum in a 17-year-old boy. Lateral chest radiograph (a) and axial CT scan at the level of the ventricles (b) show the characteristic anterior protrusion of the lower portion of the sternum and the costal cartilages, with flattening of both sides of the chest.

 

View larger version (63K): [in this window] [in a new window] [Download PPT slide]   Figure 12b.  Pectus carinatum in a 17-year-old boy. Lateral chest radiograph (a) and axial CT scan at the level of the ventricles (b) show the characteristic anterior protrusion of the lower portion of the sternum and the costal cartilages, with flattening of both sides of the chest.

 

View larger version (97K): [in this window] [in a new window] [Download PPT slide]   Figure 13.  Arachnodactyly in a 16-year-old girl. Radiograph shows a metacarpal index of approximately 9.5.

 

View larger version (152K): [in this window] [in a new window] [Download PPT slide]   Figure 14.  Acetabular protrusion in an 18-year-old man. Pelvic radiograph shows medial displacement of the acetabulum (dotted line). The center-edge angle of Wiberg is 54°. The teardrop sign is obscured by the femoral head (arrow).

 

View larger version (94K): [in this window] [in a new window] [Download PPT slide]   Figure 15a.  Dural ectasia and anterior meningocele with hemorrhagic complication in an 18-year-old man. (a) Axial CT scan at the level of the upper sacrum shows diffuse dilatation of the spinal canal and scalloping of vertebral bodies. (b, c) Axial T1-weighted (b) and axial T2-weighted fat-suppressed (c) fast spin-echo MR images at the level of the lower sacrum show a large complicated anterior meningocele with a subacute hemorrhage (*) in the presacral space and a fluid-hemorrhage level indicative of dural ectasia (arrow). A smaller meningocele (arrowhead) is visible along the left sacral nerve. (d) Sagittal contrast-enhanced T1-weighted fat-suppressed fast spin-echo image shows a cerebrospinal fluid–hemorrhage level (arrow) near the area of dural ectasia at the level of the sacrum (arrowheads) and a subacute hematoma in the large meningocele (*).

 

View larger version (122K): [in this window] [in a new window] [Download PPT slide]   Figure 15b.  Dural ectasia and anterior meningocele with hemorrhagic complication in an 18-year-old man. (a) Axial CT scan at the level of the upper sacrum shows diffuse dilatation of the spinal canal and scalloping of vertebral bodies. (b, c) Axial T1-weighted (b) and axial T2-weighted fat-suppressed (c) fast spin-echo MR images at the level of the lower sacrum show a large complicated anterior meningocele with a subacute hemorrhage (*) in the presacral space and a fluid-hemorrhage level indicative of dural ectasia (arrow). A smaller meningocele (arrowhead) is visible along the left sacral nerve. (d) Sagittal contrast-enhanced T1-weighted fat-suppressed fast spin-echo image shows a cerebrospinal fluid–hemorrhage level (arrow) near the area of dural ectasia at the level of the sacrum (arrowheads) and a subacute hematoma in the large meningocele (*).

 

View larger version (109K): [in this window] [in a new window] [Download PPT slide]   Figure 15c.  Dural ectasia and anterior meningocele with hemorrhagic complication in an 18-year-old man. (a) Axial CT scan at the level of the upper sacrum shows diffuse dilatation of the spinal canal and scalloping of vertebral bodies. (b, c) Axial T1-weighted (b) and axial T2-weighted fat-suppressed (c) fast spin-echo MR images at the level of the lower sacrum show a large complicated anterior meningocele with a subacute hemorrhage (*) in the presacral space and a fluid-hemorrhage level indicative of dural ectasia (arrow). A smaller meningocele (arrowhead) is visible along the left sacral nerve. (d) Sagittal contrast-enhanced T1-weighted fat-suppressed fast spin-echo image shows a cerebrospinal fluid–hemorrhage level (arrow) near the area of dural ectasia at the level of the sacrum (arrowheads) and a subacute hematoma in the large meningocele (*).

 

View larger version (137K): [in this window] [in a new window] [Download PPT slide]   Figure 15d.  Dural ectasia and anterior meningocele with hemorrhagic complication in an 18-year-old man. (a) Axial CT scan at the level of the upper sacrum shows diffuse dilatation of the spinal canal and scalloping of vertebral bodies. (b, c) Axial T1-weighted (b) and axial T2-weighted fat-suppressed (c) fast spin-echo MR images at the level of the lower sacrum show a large complicated anterior meningocele with a subacute hemorrhage (*) in the presacral space and a fluid-hemorrhage level indicative of dural ectasia (arrow). A smaller meningocele (arrowhead) is visible along the left sacral nerve. (d) Sagittal contrast-enhanced T1-weighted fat-suppressed fast spin-echo image shows a cerebrospinal fluid–hemorrhage level (arrow) near the area of dural ectasia at the level of the sacrum (arrowheads) and a subacute hematoma in the large meningocele (*).

 

View larger version (183K): [in this window] [in a new window] [Download PPT slide]   Figure 16a.  Spontaneous pneumothorax in a 15-year-old girl. (a) CT projection radiograph shows a linear wisp of high attenuation (arrow) that represents the edge of the retracted visceral pleura in the right hemithorax. (b) Axial CT scan shows several blebs in the left apex, as well as a right pneumothorax.

 

View larger version (114K): [in this window] [in a new window] [Download PPT slide]   Figure 16b.  Spontaneous pneumothorax in a 15-year-old girl. (a) CT projection radiograph shows a linear wisp of high attenuation (arrow) that represents the edge of the retracted visceral pleura in the right hemithorax. (b) Axial CT scan shows several blebs in the left apex, as well as a right pneumothorax.

 

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