Klippel Trenaunay Syndrome Case presentation Dr. Abdelmotaal Waleed, MD Vascular Surgery, Qena University Hospitals,Egypt

- presentation: . pain . heaviness . disfigurement of left lower limb • Klippel-Trenaunay syndrome (KTS) is a rare, congenital vascular anomaly, defined as a triad including a port-wine stain, underlying bone and soft tissue hypertrophy and varicose veins and/or venous malformations. (Holak H et al,2006) • Case presentation: - A 23 years old young man - presentation: . pain . heaviness . disfigurement of left lower limb - family history was insignificant as far as genetic disorders were concerned.

Clinical Picture: -atypical veins in left lower limb, -numerous flat haemangi-omas (port wine stains) , located in the left lower extremity and left gluteal region and lower back that had been started since birth. - mild increase in size of the left lower limb.

multiple dilated atypical veins with haemangiomas in the left lower extremity

• In our case, the diseased limb was shorter than the contralateral one with difference in length about 1 cm as shown in the x-ray film. • At thigh level the difference in circumference was 11 mm& at lower leg level about 10 mm. shortening of the left lower limb in comparison with the right one

Investigations: left lower extremity venous duplex ultrasound showed: - Superficial femoral vein (+/-4mm) and popliteal vein(+/-5.5mm) hypoplasia. - incompetent saphenofemoral junction. - extensive superficical varicose veins seen at the medial aspect of lower thigh and lower leg. - multiple dilated refluxing perforators: +/-10 cm above the knee, 15 cm below the knee medially and 10cm above ankle postromedially.

MRA showed: prominent subcutaneous tissue mainly, with normal bony anatomy. - prominent venous collaterals including the large marginal superficial vein mainly at the medial aspect of the leg around the knee.  

Management: KTS is a complex disorder that need multidisciplinary approach Following radiological imaging and full delineation of the pathology, orthopedic staff recommended wearing high heel shoes for the mild discrepancy in length (<1cm). Treatment included compression therapy (compression garments), injection sclerotherapy for the atypical varicosities guided by duplex ultrasonography by vascular surgeons. Port wine stains were managed with multiple LASER settings(pulsed dye) by plastic surgeons.

Take home message • Vascular malformations are not uncommon and one should keep it in mind to prevent misdiagnosis • Establishing the diagnosis of KTS before the onset of severe vascular complications, may help avoid/diminish the severity and significantly delay the development of venous failure of the affected limb. • Compression is the hallmark of conservative treatment. Laser, sclero-theraphy (with alcohol or foam), endovenous thermal ablation, surgical stripping and phlebectomy can also be used. • Management of Klippel Trenaunay syndrome isn’t only the responsibility of vascular surgeon but it is a team work management in a multidi-sciplinary approach (Plastic,Orthopedic and Radiological) to ensure the best results of therapy.

Thank you