KLIPPEL-TRENAUNAY SYNDROME

ABSTRACT KLIPPEL-TRENAUNAY SYNDROME IS A RARE CONGENITAL VASCULARDISORDER . IT CONSISTS OF ENLARGEMENT OF AN EXTREMITY AS THE RESULT OFCIRCUMFERENTIAL SOFT-TISSUE HYPERTROPHY, BONE ELONGATION, CUTANEOUSVASCULAR LESIONS, MOSTLY CAPILLARY MALFORMATIONS AND UNDERLYING DIFFUSEVENOUS AND LYMPHATIC MALFORMATIONS.CUTANEOUS MALFORMATION USUALLYINVOLVES THE LOWER LIMB AND MOSTLY UNILATERAL.THE VENOUS ABNORMALITIES OFTHE DEEP VENOUS SYSTEM THAT OCCUR IN KTS INCLUDE ANEURYSMAL DILATATION,APLASIA AND HYPOPLASIA .THE POPLITEAL VEIN IS MOST COMMONLY INVOLVED.HYPOPLASIA, APLASIA, COMPRESSION, AND FENESTRATION ARE THE MOST COMMONLYENCOUNTERED POPLITEAL VEIN ANOMALIES. THE FEMORAL VEIN IS SECOND MOST COMMONLY AFFECTED. IF DEEP VENOUS APLASIA OR HYPOPLASIA IS PRESENT,COLLATERAL VENOUS STRUCTURES DEVELOP. COMMON COLLATERAL FLOW PATTERNS OF ILIAC AND FEMORAL APLASIA AND HYPOPLASIA INCLUDE THE ASCENDING LUMBARVEINS, ANTERIOR ABDOMINAL VEINS, AND THE INTERNAL PARAVERTEBRAL VENOUSPLEXUS. THE SCIATIC VEIN AND LATERAL VEINS OF THE LOWER EXTREMITY ALSO SERVEAS COMPENSATORY VENOUS PATHWAYS.A COMPLETE PERSISTENT SCIATIC VEIN, WHICHORIGINATES FROM THE POPLITEAL VEIN AND ENDS IN THE INTERNAL VENOUS SYSTEM ANUPPER PERSISTENT SCIATIC VEIN, WHICH ORIGINATES IN THE UPPER THIGH AND ENDS INTHE PELVIS AND A LOWER PERSISTENT SCIATIC VEIN, WHICH IS PRESENT IN THE DISTALOR MID PORTION OF THE THIGH. ANOMALOUS LATERAL VEINS THAT ORIGINATE NEAR THEANKLE AND EXTEND . KLIPPEL-TRENAUNAY SYNDROME DANIEL STANLEY PETER NARAYANAN Department of Radio Diagnosis, MADRAS MEDICAL COLLEGE AND GOVERNMENT GENERAL HOSPITAL An Initiative of The Tamil Nadu Dr. M.G.R. Medical University University Journal of Medicine and Medical Sciences TO THE INFRAINGUINAL OR PELVIC DEEP VENOUS SYSTEM .DOPPLEREXAMINATION IS A USEFUL INITIAL INVESTIGATION IN PATIENTS WITH KTSYNDROME.CONVENTIONAL VENOGRAPHY AND CT VENOGRAPHY ARE USEFUL FORIMAGING VEINS FILLED WITH CONTRAST MATERIAL.MOST PATIENTS WITH KTS ARETREATED CONSERVATIVELY, BUT SURGERY (SURGICAL STRIPPING PHLEBECTOMYSUBFASCIAL ENDOSCOPIC LIGATION OF PERFORATING VEINS) OR IMAGINGGUIDEDINTERVENTIO(SCLEROTHERAPY WITH ALCOHOL OR FOAM, SELECTIVE ENDOVENOUSTHERMAL ABLATION) IS PERFORMED IN SELECTED CASES