FOUR CASES OF KLIPPEL TRENAUNAY-WEBER SYNDROM SEEN AT JRA ANTANANARIVO UNIVERSITY HOSPITAL

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Tags : -vascularmalformation -klippeltrenaunaysyndrome

Category : Cases reports

Authors : Rajaobelison Tsirimalala

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Introduction: KlippelTrenaunay syndrome (SKP) is a malformative congenital association characterized by the presence of a triad: cutaneous hemangioma, arteriovenous fistula or varicose veins; unilateral hypertrophy of soft and bone tissues. Our aim is to report the management of our four cases.
Methods: Retrospective descriptive study performed in the service of cardiovascular surgery unit of the university teaching hospital / JRA Antananarivo, from June 2004 to October 2017. All cases of KlippelTrenaunay syndrome seen within cardiovascular surgery were included.
Results: Four cases of KlippelTrenaunay Syndrome were collected during 12 years, theaverage age is 14.6 years with extremes of [6; 32], afemale preponderance with a sex ratio of 1.5. The association of limb pain and limb varicosis represents the patient's discovery rate (100%), the average age of onset of symptoms is 6 years. Imaging shows bone hypertrophy on x-ray in 80%. The evolution is unfavorable with the appearance of pain of the limbs with limitation of the movements (100%), extension of the angiomas, the inequality of the limbs (75%), phlebitis (50%), and aesthetic sequelae (100%). No deaths were found.
Discussion and conclusion: Our 04 cases present at least two diagnostic criteria. His incidence is low, etiology remains unknown. His care is multidisciplinary; bone hypertrophy causes a 9 cm average limb unevenness that requires orthopedic and / or surgical management, a veneering is necessary if painful varicosity.