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Vascular Malformation and Hemangiomatosis Syndromes: Spectrum of Imaging Manifestations

¹ Department of Radiology, University of Michigan Health System, 1500 E Medical Center Dr., Ann Arbor, MI, 48109.
² Mallinckrodt Institute of Radiology, Washington University, St. Louis, MO.

View larger version (129K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —32-year-old woman with blue rubber bleb nevus syndrome. Axial STIR MR image (TR/TE, 5,500/80; inversion time, 165 milliseconds; slice thickness, 4 mm) shows circumferential rectal wall and perirectal heterogeneous predominantly high signal intensity.

View larger version (117K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —32-year-old woman with blue rubber bleb nevus syndrome. Axial unenhanced (B) and contrast-enhanced (C) T1-weighted gradient-recalled echo (225/3.4; flip angle, 70°; slice thickness, 4 mm) delayed venous phase MR images show abnormal circumferential rectal wall and perirectal soft-tissue enhancement pathologically proved to represent large venous malformation.

View larger version (152K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C —32-year-old woman with blue rubber bleb nevus syndrome. Axial unenhanced (B) and contrast-enhanced (C) T1-weighted gradient-recalled echo (225/3.4; flip angle, 70°; slice thickness, 4 mm) delayed venous phase MR images show abnormal circumferential rectal wall and perirectal soft-tissue enhancement pathologically proved to represent large venous malformation.

View larger version (63K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A —12-year-old girl with blue rubber bleb nevus syndrome. Coronal STIR (TR/TE, 4,000/26; inversion time, 165 milliseconds; slice thickness, 5 mm) (A) and contrast-enhanced fat-saturated T1-weighted spoiled gradient-recalled echo (255/3.3; flip angle, 90°; slice thickness, 5 mm) (B) delayed venous phase MR images show large venous malformation involving subcutaneous tissue and underlying musculature of left lower extremity.

View larger version (61K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B —12-year-old girl with blue rubber bleb nevus syndrome. Coronal STIR (TR/TE, 4,000/26; inversion time, 165 milliseconds; slice thickness, 5 mm) (A) and contrast-enhanced fat-saturated T1-weighted spoiled gradient-recalled echo (255/3.3; flip angle, 90°; slice thickness, 5 mm) (B) delayed venous phase MR images show large venous malformation involving subcutaneous tissue and underlying musculature of left lower extremity.

View larger version (128K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3 —39-year-old man with blue rubber bleb nevus syndrome. Axial contrast-enhanced portal venous phase fat-saturated T1-weighted spoiled gradient-recalled echo MR image (TR/TE, 4.2/2.0; flip angle, 12°; slice thickness, 4 mm) shows multiple peripherally enhanced hemangioma-like lesions within liver (long arrows), spleen (short arrows), and spine (arrowheads).

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —23-year-old man with Proteus syndrome. Axial contrast-enhanced portal venous phase CT scan through upper abdomen reveals multiple low-attenuation splenic lesions thought to represent vascular malformation.

View larger version (126K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —23-year-old man with Proteus syndrome. Axial CT scan through lower abdomen shows marked soft-tissue asymmetry, including asymmetric prominence of right gluteus and iliopsoas muscles (long arrows) (compared with opposite side) and increased fatty tissue within left lower quadrant of abdomen and left lateral body wall (short arrows).

View larger version (129K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5A —10-year-old boy with Proteus syndrome. Axial T1-weighted FLAIR MR image (TR/TE, 2,036/21; slice thickness, 5 mm) through lower abdomen shows large well-circumscribed mass of high signal intensity in left anterior abdominal wall consistent with lipoma (arrows).

View larger version (139K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5B —10-year-old boy with Proteus syndrome. Axial contrast-enhanced T1-weighted FLAIR venous phase image (2,055/21; slice thickness, 4 mm) through level of midthoracic spine reveals enhanced large right paraspinous presumed vascular malformation (arrows).

View larger version (150K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6A —5-year-old boy with Proteus syndrome and megalencephaly. (Courtesy of Parmar H, University of Michigan Health System, Ann Arbor, MI) Axial T2-weighted fast spin-echo MR image (TR/TE, 3,200/102; slice thickness, 6 mm) through level of upper cervical spine and maxilla shows extensive right facial soft-tissue hypertrophy (asterisks).

View larger version (127K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6B —5-year-old boy with Proteus syndrome and megalencephaly. (Courtesy of Parmar H, University of Michigan Health System, Ann Arbor, MI) Axial T2-weighted fast spin-echo MR image (3,200/102; slice thickness, 6 mm) through level of cerebral hemispheres reveals enlargement of right cerebrum with associated abnormal cortical sulcation and gyration (arrowheads).

View larger version (99K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7 —19-year-old woman with Klippel-Trénaunay syndrome. Axial contrast-enhanced CT scan through midthigh level reveals soft-tissue hypertrophy (arrows) of right thigh, including asymmetrically increased circumference compared with opposite side.

View larger version (142K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8 —22-year-old man with Klippel-Trénaunay syndrome. Axial contrast-enhanced portal venous phase CT scan through upper abdomen shows multiple low-attenuation splenic lesions (arrows), presumably hemangiomas or vascular malformations.

View larger version (117K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9A —12-year-old girl with Klippel-Trénaunay syndrome. Axial (A) and coronal (B) contrast-enhanced CT scans of thorax show large avidly enhanced mass (arrows) within posterior mediastinum, including bilateral paraspinous regions, representing pathologically proven hemangioma.

View larger version (169K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9B —12-year-old girl with Klippel-Trénaunay syndrome. Axial (A) and coronal (B) contrast-enhanced CT scans of thorax show large avidly enhanced mass (arrows) within posterior mediastinum, including bilateral paraspinous regions, representing pathologically proven hemangioma.

View larger version (26K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10A —29-year-old woman with Klippel-Trénaunay syndrome. Coronal STIR MR image (TR/TE, 4,308/30; inversion time, 165 milliseconds; slice thickness, 5 mm) of left forearm shows extensive venous malformation with both superficial and deep venous components.

View larger version (89K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10B —29-year-old woman with Klippel-Trénaunay syndrome. Conventional venogram shows findings similar to those in A, including venous varices and aneurysms.

View larger version (167K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 11 —22-year-old woman with Kasabach-Merritt syndrome. Axial STIR MR image (TR/TE, 3,200/55; inversion time, 165 milliseconds; slice thickness, 4 mm) shows presumed hemangiomatosis of spleen and overlying subcutaneous tissue (arrows). Both splenic and body wall lesions are hyperintense on STIR images, as is typical of hemangiomas.

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12A —25-year-old man with Kasabach-Merritt syndrome. Coronal T2-weighted single-shot fast spin-echo MR image (TE, 180 milliseconds; slice thickness, 8 mm) shows innumerable hyperintense lesions within spleen.

View larger version (131K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12B —25-year-old man with Kasabach-Merritt syndrome. Contrast-enhanced axial portal venous phase (B) and delayed phase (C) fat-saturated T1-weighted spoiled gradient-recalled echo MR images (TR/TE, 4.2/2.0; flip angle, 12°; slice thickness, 4 mm) through upper part of abdomen show multiple enhanced presumed hemangiomas (hemangiomatosis) of spleen.

View larger version (133K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12C —25-year-old man with Kasabach-Merritt syndrome. Contrast-enhanced axial portal venous phase (B) and delayed phase (C) fat-saturated T1-weighted spoiled gradient-recalled echo MR images (TR/TE, 4.2/2.0; flip angle, 12°; slice thickness, 4 mm) through upper part of abdomen show multiple enhanced presumed hemangiomas (hemangiomatosis) of spleen.

View larger version (90K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13A —64-year-old man with giant hepatic cavernous hemangioma and consumptive coagulopathy (elevated international normalized ratio and D-dimer levels) suggesting Kasabach-Merritt-like syndrome. Abdominal radiograph shows large upper abdominal mass (arrows) displacing bowel loops into pelvis.

View larger version (109K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13B —64-year-old man with giant hepatic cavernous hemangioma and consumptive coagulopathy (elevated international normalized ratio and D-dimer levels) suggesting Kasabach-Merritt-like syndrome. Contrast-enhanced coronal fat-saturated T1-weighted spoiled gradient-recalled echo portal venous phase MR image (TR/TE, 4.0/2.0; flip angle, 12°, slice thickness, 3 mm) (B) and 99mTc-labeled RBC nuclear scintigraphic image (C) confirm presence of extremely large hepatic cavernous hemangioma. Arrowhead (C) denotes central filling defect consistent with central scar or thrombus.

View larger version (156K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13C —64-year-old man with giant hepatic cavernous hemangioma and consumptive coagulopathy (elevated international normalized ratio and D-dimer levels) suggesting Kasabach-Merritt-like syndrome. Contrast-enhanced coronal fat-saturated T1-weighted spoiled gradient-recalled echo portal venous phase MR image (TR/TE, 4.0/2.0; flip angle, 12°, slice thickness, 3 mm) (B) and 99mTc-labeled RBC nuclear scintigraphic image (C) confirm presence of extremely large hepatic cavernous hemangioma. Arrowhead (C) denotes central filling defect consistent with central scar or thrombus.

View larger version (124K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13D —64-year-old man with giant hepatic cavernous hemangioma and consumptive coagulopathy (elevated international normalized ratio and D-dimer levels) suggesting Kasabach-Merritt-like syndrome. Conventional angiogram after selective celiac artery injection and before transcatheter embolization of lesion confirms presence of giant cavernous hemangioma.

View larger version (108K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 14A —2-month-old boy with Kasabach-Merritt syndrome. Axial contrast-enhanced portal venous phase MR images through levels of midright kidney (A) and iliac fossae (B) reveal large enhanced retroperitoneal mass (arrows) extending from left renal hilum to left midthigh.

View larger version (107K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 14B —2-month-old boy with Kasabach-Merritt syndrome. Axial contrast-enhanced portal venous phase MR images through levels of midright kidney (A) and iliac fossae (B) reveal large enhanced retroperitoneal mass (arrows) extending from left renal hilum to left midthigh.

View larger version (101K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 14C —2-month-old boy with Kasabach-Merritt syndrome. STIR MR image (TR/TE, 4,000/24; inversion time, 165 milliseconds; section thickness, 5 mm) shows hyperintense mass (arrows) that represents pathologically proven hemangioendothelioma.

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