VENOUS MALFORMATIONS
VENOUS MALFORMATIONS ICD-10: q82.9
• Venous malformations are deep vascular malformations characterized by soft, compressible deep-tissue swelling. Lesions are present at birth.
• They manifest as blue patches or soft blue nodules (Fig. 9-28). When extending to the epidermis, the surface may be verrucous. Borders are poorly defined, and there is considerable variation in size. They are easily compressed and fill promptly when pressure is released.
• Patients with extensive lesions should be evaluated for a coagulopathy.
• Sclerotherapy and compression are first-line treatments. Laser may also be considered.
VARIANTS
KLIPPEL–TRÉNAUNAY SYNDROME Triad of capillary malformation + venous malformation + bony/soft tissue hypertrophy of the leg (Fig. 9-29). Complications include clots, limb length discrepancy, pain and infection. BLUE RUBBER BLEB NEVUS Venous malformation that may be painful, tender or have overlying hyperhidrosis. Multiple compressible, soft, blue nodules in the dermis and
subcutaneous tissue (Fig. 9-30) that are most often on arms, trunk, and soles. Can have internal involvement, especially GI tract. MAFFUCCI SYNDROME Genetic syndrome with endochondromas, venous malformations, and possibly spindle cell hemangiomas. Soft blue nodules on distal extremities. Endochondromas present as nodular outgrowths. Patients with increased malignancy risk, especially chondrosarcoma.
FIGURE 9-28 • Capillary–venous malformation in an infant There is a soft, compressible, and bluish-red tissue swelling distorting the upper lip and lower eyelid. It is a slow-flow lesion but requires therapeutic intervention.
FIGURE 9-29 • Capillary–venous malformation In a 31-year-old Thai woman. This nevus flammeus-like lesion was associated with phlebectasia, lymphedema, and an enlarged right lower extremity (Klippel–Trénaunay syndrome).
FIGURE 9-30 • Blue rubber bleb nevus A spontaneously painful and tender capillary-venous malformation. There are a number of compressible bluish-violaceous papules and nodules on the upper arm.