Skin Hemangiomas with Limb Hypertrophy

Klippel-Trenaunay syndrome. It is a sporadic rare cutaneous vascular disorder characterized by a triad of a port-wine stain, varicose veins, and hemangiomata along with bony or soft tissue hypertrophy of an extremity associated with life-threatening complications.^1,2 Complications include limb-length discrepancy leading to impaired gait and pain, thromboembolism, bleeding, venous insufficiency, and soft-tissue infection.³ A primary mesodermal abnormality in fetal development leads to persistence of microscopic arteriovenous communications.¹ The differential diagnosis are Parkes Weber syndrome, Proteus syndrome, Macrodystrophia lipomatosa, Beckwith-Wiedemann syndrome, neurofibromatosis, soft tissue sarcomas, and lymphangioma.⁴ Ultrasound-guided foam sclerotherapy is the state of the art new treatment to potentially close many large vascular malformations.⁵ Compression therapies and surgical debulking can be considered.⁶

References :

Zea MI, Hanif M, Habib M, Ansari A. Klippel-Trenaunay Syndrome: a case report with brief review of literature. J Dermatol Case Rep. 2009;3:56-9. Jacob AG, Driscoll DJ, Shaughnessy WJ, Stanson AW, Clay RP, Gloviczki P. Klippel-Trénaunay syndrome: spectrum and management. Mayo Clin Proc. 1998; 73:28-36. Sharma D, Lamba S, Pandita A, Shastri S. Klippel-trénaunay syndrome - a very rare and interesting syndrome. Clin Med Insights Circ Respir Pulm Med. 2015;9:1-4 Lacerda LdaS, Alves UD, Zanier JF, Machado DC, Camilo GB, Lopes AJ. Differential diagnoses of overgrowth syndromes: the most important clinical and radiological disease manifestations. Radiol Res Pract 2014; 2014: 947451 Smith PC. Chronic venous disease treated by ultrasound guided foam sclerotherapy. Eur J Vasc Endovasc Surg. 2006; 32:577-583 Noel AA, Gloviczki P, Cherry KJ Jr, Rooke TW, Stanson AW, Driscoll DJ. Surgical treatment of venous malformations in Klippel-Trénaunay syndrome. J Vasc Surg. 2000; 32:840-847