KLIPPEL TRENAUNAY SYNDROME- WIDE VARIATION IN PRESENTATION: OUR EXPERIENCE OF 5 PATIENTS.
AIM: this is to report a case series of klippel trenaunay syndrome in our hospital and to make medical practitioners more aware of this rare condition and improve their diagnostic awareness.
BACKGROUND: Klippel trenaunay syndrome is characterized by a triad of port wine stain, varicose veins and soft tissue and bony hypertrophy. In 1900, two French physicians klippel and trenaunay first described this syndrome in 2 patients. Although 100 hundred years have past exact incidence, etiology and pathogenesis is yet to be elucided. Although clinical presentation ranges from minimal symptomatic disease to life threatening bleeding and embolism management includes careful diagnosis, prevention and treatment of complications.
CONCLUSION: Complexity of vascular malformation made it difficult not only to classify but also to diagnose its rare subtypes, usually klippel trenaunay syndrome present at later stages with complications due to lack of awareness. This case series is to indentify different presenting symptoms and management modalities.
Keywords: klippel trenaunay syndrome, varicose veins, port wine stain