Poster 244: Lymphedema Therapy in Klippel-Trénaunay Syndrome: A Case Report

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Setting: Tertiary medical center. Patient: A 17-month-old girl born with an edematous left leg. Case Description: The patient was found to have an enlarged left leg during a third trimester ultrasound. This finding persisted to the time of birth. At 12 months, she developed a clear drainage from her left leg and cellulitis. The patient was referred at 17 months of age for lymphedema consultation for persistent limb edema. Assessment/Results: On exam, the patient was a healthy 17-month-old child who was friendly and playful. She was an intelligent child and had met her milestones on target. Her left leg was markedly larger than her right throughout, measuring a mean 8cm difference in circumference. Superficial venous vascular malformations were noted, but no port wine stains. There were no further signs of cellulitis. No neurologic abnormalities were present. Scanogram showed a 1.7cm leg-length discrepancy, left longer than right. Magnetic resonance imaging demonstrated extensive vascular malformations present in the subcutaneous tissue and less extensively into deep muscle compartments of the left leg without obvious extensive involvement of the hip, knee, or ankle. She was diagnosed with Klippel-Trénaunay syndrome (KTS). For lymphedema control, she was fitted with a thigh-high compression stocking with a waist attachment. Heel lift was recommended for leg-length discrepancy. Discussion: KTS is a congenital anomaly characterized by unilateral limb overgrowth, venous varicosities, and capillary malformations. 95% of cases involve the lower extremity. Hypertrophy of the extremities can be extreme, requiring amputation. Lymphatic involvement can affect wound healing. Treatment is targeted at symptom management as well as maximizing and maintaining function. Physiatric referral can provide patients with effective conservative measures. Conclusions: KTS poses relevant clinical challenges to the physiatrist, including lymphedema management, leg-length assessment with modifications, gait analysis, and presurgical counseling.