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Peripheral Vascular Malformations: Imaging, Treatment Approaches, and Therapeutic Issues¹

¹ From the Department of Radiology, Sapporo Medical University, S1 W16 Chuo-ku, Sapporo 060-8543, Japan. Presented as an education exhibit at the 2004 RSNA Annual Meeting. Received February 7, 2005; revision requested March 2 and received March 28; accepted April 15. All authors have no financial relationships to disclose.

View larger version (28K): [in a new window]   Figure 1a.  Treatment of vascular malformation. (a) Diagram shows a simple vascular malformation that consists of one feeding artery, the nidus, and one drainage vein. For this type of lesion, sclerotherapy with percutaneously administered liquid sclerosant is indicated. (b) Diagram shows a vascular malformation with several feeding arteries and drainage veins. For this type of lesion, sclerotherapy begins with a flow-control procedure (the drainage vein is occluded by means of a balloon catheter with or without use of coils or n-butyl cyanoacrylate [NBCA] to decrease the number of drainage veins) to achieve sclerosant stasis. Additional sclerotherapy to the nidus is then performed. (c) Diagram shows a vascular malformation with several drainage veins and feeding arteries, one of which was embolized. This is an ineffective procedure that makes the latent feeding arteries apparent. The nidus flow volume usually increases, and clinical symptoms worsen. As with embolization of the feeding artery, coil embolization of the drainage vessel alone is not sufficient treatment. Without ablation of the nidus, a good outcome cannot be expected.

 

View larger version (42K): [in a new window]   Figure 1b.  Treatment of vascular malformation. (a) Diagram shows a simple vascular malformation that consists of one feeding artery, the nidus, and one drainage vein. For this type of lesion, sclerotherapy with percutaneously administered liquid sclerosant is indicated. (b) Diagram shows a vascular malformation with several feeding arteries and drainage veins. For this type of lesion, sclerotherapy begins with a flow-control procedure (the drainage vein is occluded by means of a balloon catheter with or without use of coils or n-butyl cyanoacrylate [NBCA] to decrease the number of drainage veins) to achieve sclerosant stasis. Additional sclerotherapy to the nidus is then performed. (c) Diagram shows a vascular malformation with several drainage veins and feeding arteries, one of which was embolized. This is an ineffective procedure that makes the latent feeding arteries apparent. The nidus flow volume usually increases, and clinical symptoms worsen. As with embolization of the feeding artery, coil embolization of the drainage vessel alone is not sufficient treatment. Without ablation of the nidus, a good outcome cannot be expected.

 

View larger version (36K): [in a new window]   Figure 1c.  Treatment of vascular malformation. (a) Diagram shows a simple vascular malformation that consists of one feeding artery, the nidus, and one drainage vein. For this type of lesion, sclerotherapy with percutaneously administered liquid sclerosant is indicated. (b) Diagram shows a vascular malformation with several feeding arteries and drainage veins. For this type of lesion, sclerotherapy begins with a flow-control procedure (the drainage vein is occluded by means of a balloon catheter with or without use of coils or n-butyl cyanoacrylate [NBCA] to decrease the number of drainage veins) to achieve sclerosant stasis. Additional sclerotherapy to the nidus is then performed. (c) Diagram shows a vascular malformation with several drainage veins and feeding arteries, one of which was embolized. This is an ineffective procedure that makes the latent feeding arteries apparent. The nidus flow volume usually increases, and clinical symptoms worsen. As with embolization of the feeding artery, coil embolization of the drainage vessel alone is not sufficient treatment. Without ablation of the nidus, a good outcome cannot be expected.

 

View larger version (142K): [in a new window]   Figure 2a.  Slow-flow vascular malformation in a 33-year-old man with pain in the left palm and thumb. (a) Photograph demonstrates a blue lesion that protrudes from the thenar eminence. (b) Coronal fat-saturated T2-weighted MR image with maximum intensity projection shows a high-signal-intensity mass. (c, d) Images obtained during sclerotherapy show direct injection of ethanolamine oleate, which was performed after the maximum dose was determined and with intermittent fluoroscopic guidance. (e) Photograph obtained 2 days after sclerotherapy shows internal hemorrhage in the puncture sites, but protrusion of the lesion and clinical symptoms have decreased.

 

View larger version (162K): [in a new window]   Figure 2b.  Slow-flow vascular malformation in a 33-year-old man with pain in the left palm and thumb. (a) Photograph demonstrates a blue lesion that protrudes from the thenar eminence. (b) Coronal fat-saturated T2-weighted MR image with maximum intensity projection shows a high-signal-intensity mass. (c, d) Images obtained during sclerotherapy show direct injection of ethanolamine oleate, which was performed after the maximum dose was determined and with intermittent fluoroscopic guidance. (e) Photograph obtained 2 days after sclerotherapy shows internal hemorrhage in the puncture sites, but protrusion of the lesion and clinical symptoms have decreased.

 

View larger version (113K): [in a new window]   Figure 2c.  Slow-flow vascular malformation in a 33-year-old man with pain in the left palm and thumb. (a) Photograph demonstrates a blue lesion that protrudes from the thenar eminence. (b) Coronal fat-saturated T2-weighted MR image with maximum intensity projection shows a high-signal-intensity mass. (c, d) Images obtained during sclerotherapy show direct injection of ethanolamine oleate, which was performed after the maximum dose was determined and with intermittent fluoroscopic guidance. (e) Photograph obtained 2 days after sclerotherapy shows internal hemorrhage in the puncture sites, but protrusion of the lesion and clinical symptoms have decreased.

 

View larger version (82K): [in a new window]   Figure 2d.  Slow-flow vascular malformation in a 33-year-old man with pain in the left palm and thumb. (a) Photograph demonstrates a blue lesion that protrudes from the thenar eminence. (b) Coronal fat-saturated T2-weighted MR image with maximum intensity projection shows a high-signal-intensity mass. (c, d) Images obtained during sclerotherapy show direct injection of ethanolamine oleate, which was performed after the maximum dose was determined and with intermittent fluoroscopic guidance. (e) Photograph obtained 2 days after sclerotherapy shows internal hemorrhage in the puncture sites, but protrusion of the lesion and clinical symptoms have decreased.

 

View larger version (132K): [in a new window]   Figure 2e.  Slow-flow vascular malformation in a 33-year-old man with pain in the left palm and thumb. (a) Photograph demonstrates a blue lesion that protrudes from the thenar eminence. (b) Coronal fat-saturated T2-weighted MR image with maximum intensity projection shows a high-signal-intensity mass. (c, d) Images obtained during sclerotherapy show direct injection of ethanolamine oleate, which was performed after the maximum dose was determined and with intermittent fluoroscopic guidance. (e) Photograph obtained 2 days after sclerotherapy shows internal hemorrhage in the puncture sites, but protrusion of the lesion and clinical symptoms have decreased.

 

View larger version (128K): [in a new window]   Figure 3a.  Slow-flow vascular malformation in a 45-year-old woman with left thigh tenderness. (a) Coronal fat-saturated T2-weighted MR image shows a high-signal-intensity mass. (b, c) Intraoperative photograph (b) and angiogram (c) were obtained during sclerotherapy, which was performed with intermittent fluoroscopic guidance. Polidocanol was injected directly, but the sclerosing agent drained into the vein, and flow stasis was difficult to achieve within the nidus. Thus, multiple needle punctures were needed to distribute the sclerosing agent. After the procedure, the patient experienced reversible low blood pressure because of the anesthetic effect of polidocanol.

 

View larger version (107K): [in a new window]   Figure 3b.  Slow-flow vascular malformation in a 45-year-old woman with left thigh tenderness. (a) Coronal fat-saturated T2-weighted MR image shows a high-signal-intensity mass. (b, c) Intraoperative photograph (b) and angiogram (c) were obtained during sclerotherapy, which was performed with intermittent fluoroscopic guidance. Polidocanol was injected directly, but the sclerosing agent drained into the vein, and flow stasis was difficult to achieve within the nidus. Thus, multiple needle punctures were needed to distribute the sclerosing agent. After the procedure, the patient experienced reversible low blood pressure because of the anesthetic effect of polidocanol.

 

View larger version (92K): [in a new window]   Figure 3c.  Slow-flow vascular malformation in a 45-year-old woman with left thigh tenderness. (a) Coronal fat-saturated T2-weighted MR image shows a high-signal-intensity mass. (b, c) Intraoperative photograph (b) and angiogram (c) were obtained during sclerotherapy, which was performed with intermittent fluoroscopic guidance. Polidocanol was injected directly, but the sclerosing agent drained into the vein, and flow stasis was difficult to achieve within the nidus. Thus, multiple needle punctures were needed to distribute the sclerosing agent. After the procedure, the patient experienced reversible low blood pressure because of the anesthetic effect of polidocanol.

 

View larger version (135K): [in a new window]   Figure 4a.  High-flow vascular malformation in a 22-year-old man with tenderness of the right lower limb. (a) Axial CT scan obtained with contrast material enhancement shows dilated vessels within the gastrocnemius muscle. (b, c) Angiograms show multiple feeding arteries and early venous return, findings that indicate a high-flow venous malformation. (d) Image obtained during percutaneous direct injection sclerotherapy. Balloon occlusion was performed at the popliteal vein, and 40 mL of ethanolamine oleate with 10 mL of absolute ethanol was injected into the nidus. Distribution of the sclerosant within the nidus (and the feeding artery retrogradely) is key to the success of the procedure. (e) Axial contrast-enhanced CT scan obtained at 2 years follow-up shows a thrombosed and diminished nidus.

 

View larger version (103K): [in a new window]   Figure 4b.  High-flow vascular malformation in a 22-year-old man with tenderness of the right lower limb. (a) Axial CT scan obtained with contrast material enhancement shows dilated vessels within the gastrocnemius muscle. (b, c) Angiograms show multiple feeding arteries and early venous return, findings that indicate a high-flow venous malformation. (d) Image obtained during percutaneous direct injection sclerotherapy. Balloon occlusion was performed at the popliteal vein, and 40 mL of ethanolamine oleate with 10 mL of absolute ethanol was injected into the nidus. Distribution of the sclerosant within the nidus (and the feeding artery retrogradely) is key to the success of the procedure. (e) Axial contrast-enhanced CT scan obtained at 2 years follow-up shows a thrombosed and diminished nidus.

 

View larger version (78K): [in a new window]   Figure 4c.  High-flow vascular malformation in a 22-year-old man with tenderness of the right lower limb. (a) Axial CT scan obtained with contrast material enhancement shows dilated vessels within the gastrocnemius muscle. (b, c) Angiograms show multiple feeding arteries and early venous return, findings that indicate a high-flow venous malformation. (d) Image obtained during percutaneous direct injection sclerotherapy. Balloon occlusion was performed at the popliteal vein, and 40 mL of ethanolamine oleate with 10 mL of absolute ethanol was injected into the nidus. Distribution of the sclerosant within the nidus (and the feeding artery retrogradely) is key to the success of the procedure. (e) Axial contrast-enhanced CT scan obtained at 2 years follow-up shows a thrombosed and diminished nidus.

 

View larger version (108K): [in a new window]   Figure 4d.  High-flow vascular malformation in a 22-year-old man with tenderness of the right lower limb. (a) Axial CT scan obtained with contrast material enhancement shows dilated vessels within the gastrocnemius muscle. (b, c) Angiograms show multiple feeding arteries and early venous return, findings that indicate a high-flow venous malformation. (d) Image obtained during percutaneous direct injection sclerotherapy. Balloon occlusion was performed at the popliteal vein, and 40 mL of ethanolamine oleate with 10 mL of absolute ethanol was injected into the nidus. Distribution of the sclerosant within the nidus (and the feeding artery retrogradely) is key to the success of the procedure. (e) Axial contrast-enhanced CT scan obtained at 2 years follow-up shows a thrombosed and diminished nidus.

 

View larger version (109K): [in a new window]   Figure 4e.  High-flow vascular malformation in a 22-year-old man with tenderness of the right lower limb. (a) Axial CT scan obtained with contrast material enhancement shows dilated vessels within the gastrocnemius muscle. (b, c) Angiograms show multiple feeding arteries and early venous return, findings that indicate a high-flow venous malformation. (d) Image obtained during percutaneous direct injection sclerotherapy. Balloon occlusion was performed at the popliteal vein, and 40 mL of ethanolamine oleate with 10 mL of absolute ethanol was injected into the nidus. Distribution of the sclerosant within the nidus (and the feeding artery retrogradely) is key to the success of the procedure. (e) Axial contrast-enhanced CT scan obtained at 2 years follow-up shows a thrombosed and diminished nidus.

 

View larger version (93K): [in a new window]   Figure 5a.  High-flow vascular malformation in the pelvis of a 14-year-old girl with general fatigue. (a) Axial contrast-enhanced CT scan shows dilated veins surrounding the abdominal aorta and extension of the veins to the pre-vertebral canal. (b, c) Preoperative angiograms obtained after fibered microcoils and n-butyl cyanoacrylate embolization were used to decrease the inflow before surgical exclusion. (d) Angiogram obtained after unsuccessful surgical ligation shows new feeding arteries formed from the superior and inferior mesenteric arteries. Although 20-mm occlusive balloon catheters were placed in the drainage vein, blood flow stasis was not achieved, and sclerotic agents such as ethanolamine oleate or polidocanol could not be used. (e) Angiogram obtained after additional n-butyl cyanoacrylate and coil embolizations were performed for palliation.

 

View larger version (153K): [in a new window]   Figure 5b.  High-flow vascular malformation in the pelvis of a 14-year-old girl with general fatigue. (a) Axial contrast-enhanced CT scan shows dilated veins surrounding the abdominal aorta and extension of the veins to the pre-vertebral canal. (b, c) Preoperative angiograms obtained after fibered microcoils and n-butyl cyanoacrylate embolization were used to decrease the inflow before surgical exclusion. (d) Angiogram obtained after unsuccessful surgical ligation shows new feeding arteries formed from the superior and inferior mesenteric arteries. Although 20-mm occlusive balloon catheters were placed in the drainage vein, blood flow stasis was not achieved, and sclerotic agents such as ethanolamine oleate or polidocanol could not be used. (e) Angiogram obtained after additional n-butyl cyanoacrylate and coil embolizations were performed for palliation.

 

View larger version (91K): [in a new window]   Figure 5c.  High-flow vascular malformation in the pelvis of a 14-year-old girl with general fatigue. (a) Axial contrast-enhanced CT scan shows dilated veins surrounding the abdominal aorta and extension of the veins to the pre-vertebral canal. (b, c) Preoperative angiograms obtained after fibered microcoils and n-butyl cyanoacrylate embolization were used to decrease the inflow before surgical exclusion. (d) Angiogram obtained after unsuccessful surgical ligation shows new feeding arteries formed from the superior and inferior mesenteric arteries. Although 20-mm occlusive balloon catheters were placed in the drainage vein, blood flow stasis was not achieved, and sclerotic agents such as ethanolamine oleate or polidocanol could not be used. (e) Angiogram obtained after additional n-butyl cyanoacrylate and coil embolizations were performed for palliation.

 

View larger version (101K): [in a new window]   Figure 5d.  High-flow vascular malformation in the pelvis of a 14-year-old girl with general fatigue. (a) Axial contrast-enhanced CT scan shows dilated veins surrounding the abdominal aorta and extension of the veins to the pre-vertebral canal. (b, c) Preoperative angiograms obtained after fibered microcoils and n-butyl cyanoacrylate embolization were used to decrease the inflow before surgical exclusion. (d) Angiogram obtained after unsuccessful surgical ligation shows new feeding arteries formed from the superior and inferior mesenteric arteries. Although 20-mm occlusive balloon catheters were placed in the drainage vein, blood flow stasis was not achieved, and sclerotic agents such as ethanolamine oleate or polidocanol could not be used. (e) Angiogram obtained after additional n-butyl cyanoacrylate and coil embolizations were performed for palliation.

 

View larger version (144K): [in a new window]   Figure 5e.  High-flow vascular malformation in the pelvis of a 14-year-old girl with general fatigue. (a) Axial contrast-enhanced CT scan shows dilated veins surrounding the abdominal aorta and extension of the veins to the pre-vertebral canal. (b, c) Preoperative angiograms obtained after fibered microcoils and n-butyl cyanoacrylate embolization were used to decrease the inflow before surgical exclusion. (d) Angiogram obtained after unsuccessful surgical ligation shows new feeding arteries formed from the superior and inferior mesenteric arteries. Although 20-mm occlusive balloon catheters were placed in the drainage vein, blood flow stasis was not achieved, and sclerotic agents such as ethanolamine oleate or polidocanol could not be used. (e) Angiogram obtained after additional n-butyl cyanoacrylate and coil embolizations were performed for palliation.

 

View larger version (82K): [in a new window]   Figure 6a.  Klippel-Trénaunay-Weber disease in a 16-year-old boy with swelling of the leg. Klippel-Trénaunay-Weber disease is characterized by venous angioma or combined capillary-venous-lymphatic malformation with bone and soft-tissue hypertrophy. (a) Photograph shows a dilated, blue vein that protrudes from the right leg. (b) Sagittal fat-saturated T2-weighted MR image shows the dilated vessel cavity with high signal intensity. Small areas of low signal intensity at the calf indicate phleboliths (or calcification). Sclerotherapy was not attempted in this case because of advanced muscular atrophy, which greatly increased the risk of adverse effects, such as skin ulcers or neurologic deficit.

 

View larger version (88K): [in a new window]   Figure 6b.  Klippel-Trénaunay-Weber disease in a 16-year-old boy with swelling of the leg. Klippel-Trénaunay-Weber disease is characterized by venous angioma or combined capillary-venous-lymphatic malformation with bone and soft-tissue hypertrophy. (a) Photograph shows a dilated, blue vein that protrudes from the right leg. (b) Sagittal fat-saturated T2-weighted MR image shows the dilated vessel cavity with high signal intensity. Small areas of low signal intensity at the calf indicate phleboliths (or calcification). Sclerotherapy was not attempted in this case because of advanced muscular atrophy, which greatly increased the risk of adverse effects, such as skin ulcers or neurologic deficit.

 

View larger version (66K): [in a new window]   Figure 7a.  Parks-Weber syndrome in a 21-year-old woman with cardiac failure. Parks-Weber syndrome is characterized by vascular malformation with an arteriovenous fistula. (a) Coronal T2-weighted MR image shows multiple flow voids throughout the entire left leg. (b) Angiogram shows chaotic vascular loops and multiple arteriovenous fistulas. (c) Image obtained during phlebography after percutaneous absolute ethanol sclerotherapy, ethanolamine oleate sclerotherapy with balloon occlusion at the drainage vein, coil embolization, and stent-grafts were used to improve clinical symptoms. Skin ulcers were observed near the absolute ethanol injection area. (d) Photograph shows broad skin ulcers caused by increased venous pressure in the gastrocnemius area after the procedures.

 

View larger version (114K): [in a new window]   Figure 7b.  Parks-Weber syndrome in a 21-year-old woman with cardiac failure. Parks-Weber syndrome is characterized by vascular malformation with an arteriovenous fistula. (a) Coronal T2-weighted MR image shows multiple flow voids throughout the entire left leg. (b) Angiogram shows chaotic vascular loops and multiple arteriovenous fistulas. (c) Image obtained during phlebography after percutaneous absolute ethanol sclerotherapy, ethanolamine oleate sclerotherapy with balloon occlusion at the drainage vein, coil embolization, and stent-grafts were used to improve clinical symptoms. Skin ulcers were observed near the absolute ethanol injection area. (d) Photograph shows broad skin ulcers caused by increased venous pressure in the gastrocnemius area after the procedures.

 

View larger version (109K): [in a new window]   Figure 7c.  Parks-Weber syndrome in a 21-year-old woman with cardiac failure. Parks-Weber syndrome is characterized by vascular malformation with an arteriovenous fistula. (a) Coronal T2-weighted MR image shows multiple flow voids throughout the entire left leg. (b) Angiogram shows chaotic vascular loops and multiple arteriovenous fistulas. (c) Image obtained during phlebography after percutaneous absolute ethanol sclerotherapy, ethanolamine oleate sclerotherapy with balloon occlusion at the drainage vein, coil embolization, and stent-grafts were used to improve clinical symptoms. Skin ulcers were observed near the absolute ethanol injection area. (d) Photograph shows broad skin ulcers caused by increased venous pressure in the gastrocnemius area after the procedures.

 

View larger version (123K): [in a new window]   Figure 7d.  Parks-Weber syndrome in a 21-year-old woman with cardiac failure. Parks-Weber syndrome is characterized by vascular malformation with an arteriovenous fistula. (a) Coronal T2-weighted MR image shows multiple flow voids throughout the entire left leg. (b) Angiogram shows chaotic vascular loops and multiple arteriovenous fis-tulas. (c) Image obtained during phlebography after percutaneous absolute ethanol sclerotherapy, ethanolamine oleate sclerotherapy with balloon occlusion at the drainage vein, coil embolization, and stent-grafts were used to improve clinical symptoms. Skin ulcers were observed near the absolute ethanol injection area. (d) Photograph shows broad skin ulcers caused by increased venous pressure in the gastrocnemius area after the procedures.

 

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