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Vascular Malformations and Hemangiomas

A Practical Approach in a Multidisciplinary Clinic

Lane F. Donnelly1,2,3, Denise M. Adams1,4 and George S. Bisset, III1,2

1 Clinic for the Treatment of Vascular Malformations, Duke University Medical Center, Durham, NC 27710.
2 Department of Radiology, Division of Pediatric Radiology, Duke University Medical Center, Durham, NC 27710.
3 Present address: Department of Radiology, Children's Hospital Medical Center and the University of Cincinnati, 3333 Burnet Ave., Cincinnati, OH 45229-3039
4 Department of Pediatrics, Division of Hematology-Oncology, Duke University Medical Center, Durham, NC 27710.



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Fig. 1A. —4-month-old female infant with extensive distribution of infantile hemangioma revealed on MR imaging. Photograph shows hemangioma of right perirectal region, which was extent of disease suggested on physical inspection. Because of foot drop on physical examination, MR imaging of lumbar spine was performed.

 


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Fig. 1B. —4-month-old female infant with extensive distribution of infantile hemangioma revealed on MR imaging. Coronal (B) and axial (C) T2-weighted fat-saturated fast spin-echo MR images (3000/98 [TR/TE]) show abnormally increased signal intensity (long arrow, B) in subcutaneous region of right buttock. Extensive hemangioma throughout retroperitoneum of pelvis and abdomen is seen as abnormally high signal intensity (short arrows, B and C). Mass was found to engulf sacrum, rectum, uterus, and vagina. Note prominent veins that appear as signal voids.

 


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Fig. 1C. —4-month-old female infant with extensive distribution of infantile hemangioma revealed on MR imaging. Coronal (B) and axial (C) T2-weighted fat-saturated fast spin-echo MR images (3000/98 [TR/TE]) show abnormally increased signal intensity (long arrow, B) in subcutaneous region of right buttock. Extensive hemangioma throughout retroperitoneum of pelvis and abdomen is seen as abnormally high signal intensity (short arrows, B and C). Mass was found to engulf sacrum, rectum, uterus, and vagina. Note prominent veins that appear as signal voids.

 


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Fig. 2A. —Venous malformation involving posterior abdominal wall in 3-year-old boy with pain and progressively enlarging lesion. Photograph shows skin involvement with red discoloration and enlargement of underlying soft tissues.

 


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Fig. 2B. —Venous malformation involving posterior abdominal wall in 3-year-old boy with pain and progressively enlarging lesion. Axial T2-weighted fat-saturated fast spin-echo MR image (3500/72 [TR/TE]) shows high-signal-intensity mass predominantly involving skin and subcutaneous tissue. Note involvement of underlying abdominal wall musculature (large arrow) and prominent draining veins (small arrows).

 


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Fig. 3. —Kaposiform hemangioendothelioma involving lip and left face in 8-month-old female infant who had been treated with steroids for Kasabach-Merritt syndrome. Photograph shows superficial involvement causing skin to appear red. Note deep component distorting region inferiro to left ear. Region inferior to left lip developed fissures.

 


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Fig. 4A. —Infantile hemangioma in 21-day-old male neonate. Photograph shows lobulated mass extending from region of knee. Lack of superficial involvement renders lesion bluish rather than strawberry red.

 


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Fig. 4B. —Infantile hemangioma in 21-day-old male neonate. Axial T1-weighted MR image (500/14 [TR/TE]) shows mass (arrows) with signal intensity similar to that of skeletal muscle. Note low signal intensity and prominent veins.

 


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Fig. 4C. —Infantile hemangioma in 21-day-old male neonate. Axial T2-weighted fat-saturated fast spin-echo MR image (4000/98) shows heterogeneous high-signal-intensity mass (arrows) confined to subcutaneous tissue. Because lesion did not have classic temporal pattern of growth on physical examination, biopsy was performed to confirm diagnosis of hemangioma.

 


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Fig. 5A. —Venous malformation of left anterior pelvis in 10-year-old girl. Axial T1-weighted MR image (500/8 [TR/TE]) shows mass (arrows) confined to subcutaneous tissues. Mass is isointense in signal intensity to adjacent muscle. Note prominent draining veins.

 


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Fig. 5B. —Venous malformation of left anterior pelvis in 10-year-old girl. Axial T2-weighted fat-saturated fast spin-echo MR image (4000/98) shows mass (arrows) consisting of multiple high-signal-intensity serpentine structures. Mass is confined to subcutaneous tissue. Note prominent draining veins.

 


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Fig. 6A. —Lymphatic malformation involving arm and chest wall of 4-month-old female infant. Photograph shows enlargement and multilobulated contour of left upper extremity.

 


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Fig. 6B. —Lymphatic malformation involving arm and chest wall of 4-month-old female infant. Coronal T2-weighted fat-saturated fast spin-echo MR image (4316/98 [TR/TE]) shows multilocular cystic-appearing mass (m) involving subcutaneous tissues of left upper extremity. Note chest wall involvement (arrow).

 


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Fig. 7A. —Change in appearance after percutaneous ethanol sclerosis of venous malformation in 7-year-old girl with pain. Photograph before procedure shows bluish discoloration of skin with underlying fullness.

 


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Fig. 7B. —Change in appearance after percutaneous ethanol sclerosis of venous malformation in 7-year-old girl with pain. Photograph 4 days after sclerosis with only 7 ml of ethanol shows marked increase in swelling, hematoma, and area of skin ulceration. Findings all resolved over next several weeks; patient's pain resolved and fullness decreased.

 


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Fig. 8A. —Percutaneous sclerosis of venous malformation of foot of an 18-year-old female dancer with pain. Sagittal T2-weighted fat-saturated fast spin-echo MR image (4000/98 [TR/TE]) obtained before procedure shows serpentine areas of high signal intensity (arrows).

 


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Fig. 8B. —Percutaneous sclerosis of venous malformation of foot of an 18-year-old female dancer with pain. Image from percutaneous venogram obtained during sclerosis shows tangle of venous structures and draining veins. Note angiocatheter (arrow).

 


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Fig. 8C. —Percutaneous sclerosis of venous malformation of foot of an 18-year-old female dancer with pain. Sagittal T2-weighted fat-saturated fast spin-echo MR image (4000/98) obtained 7 months later than A and B shows resolution of serpentine high-signal-intensity structures and replacement with low-signal-intensity structures (arrows), most likely fibrotic scars.

 


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Fig. 9A. —High-flow vascular malformation of foot in 12-year-old boy. Sagittal T1-weighted MR image (750/12 [TR/TE]) shows multiple tubular flow voids (arrows). Note absence of discrete mass.

 


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Fig. 9B. —High-flow vascular malformation of foot in 12-year-old boy. Short-axis T2-weighted fat-saturated fast spin-echo MR image (3200/76) shows multiple tubular flow voids (arrow) with surrounding edema.

 


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Fig. 9C. —High-flow vascular malformation of foot in 12-year-old boy. Arteriogram shows abnormal increase in arterial flow to mid region of foot.

 


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Fig. 10A. —Embolization of arteriovenous malformation of liver in female neonate who presented with severe congestive heart failure requiring tracheal intubation and arterial pressers. Color Doppler sonogram of liver before embolization shows large feeding artery (long arrow) communicating with large draining vein (short arrow).

 


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Fig. 10B. —Embolization of arteriovenous malformation of liver in female neonate who presented with severe congestive heart failure requiring tracheal intubation and arterial pressers. Arteriogram before embolization performed with catheter in hepatic artery shows tangle of arterial structures in liver and large draining vein (arrows).

 


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Fig. 10C. —Embolization of arteriovenous malformation of liver in female neonate who presented with severe congestive heart failure requiring tracheal intubation and arterial pressers. Arteriogram after embolization shows elimination of flow through arteriovenous malformation. Patient's congestive heart failure resolved immediately and she is currently doing well 1 year later.

 


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Fig. 10D. —Embolization of arteriovenous malformation of liver in female neonate who presented with severe congestive heart failure requiring tracheal intubation and arterial pressers. Color Doppler sonogram after procedure shows thrombosis of large draining vein (arrow).

 


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Fig. 11A. —Blue rubber bleb nevus syndrome in 11-year-old boy. Photograph of tongue shows lobulated mass (arrows) in posterior tongue.

 


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Fig. 11B. —Blue rubber bleb nevus syndrome in 11-year-old boy. Axial T2-weighted fat-saturated fast spin-echo MR image (4550/84 [TR/TE]) shows venous malformation as lobulated, high-signal-intensity mass (arrows). Patient also suffered bleeding from multiple gastrointestinal sources because of other venous malformations of gastrointestinal tract.

 


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Fig. 11C. —Blue rubber bleb nevus syndrome in 11-year-old boy. Photograph of plantar surface of feet shows multiple venous malformations.

 


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Fig. 12A. —PHACE (posterior fossa abnormalities, facial hemangiomas, arterial abnormalities, cardiovascular defects, and eye abnormalities) syndrome in 1-month-old female infant. Photograph of face shows hemangioma of right orbit and ear. Eye is closed because of mass effect from hemangioma.

 


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Fig. 12B. —PHACE (posterior fossa abnormalities, facial hemangiomas, arterial abnormalities, cardiovascular defects, and eye abnormalities) syndrome in 1-month-old female infant. Axial T2-weighted fast spin-echo MR image (2800/100 [TR/TE]) with fat saturation through orbits shows lobulated high-signal-intensity hemangioma (large arrow) surrounding right globe. Note abnormal high signal intensity in subcutaneous region surrounding right ear (small arrows).

 


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Fig. 12C. —PHACE (posterior fossa abnormalities, facial hemangiomas, arterial abnormalities, cardiovascular defects, and eye abnormalities) syndrome in 1-month-old female infant. Photograph shows supraumbilical midline raphe.

 

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