Fig. 1

KHEs with chronic lymphedema involving the left lower extremity in a female. This patient’s vascular lesions were noted at 9 months of age. Limb swelling occurred 4 months later. She had partial resection of the thigh and leg KHEs at an outside institution at 2 years of age. a The photograph showed an ill-defined hyperpigmented plaque involving the left leg at referral (4 years old). Coronal (b) and horizontal (c) T2-weighted MRI revealed hyperintense lesions involving the dermis, subcutaneous tissue and deep fascia of the left leg. d Nuclear lymphoscintigram obtained 1 and 3 h after pedal injection showed uptake in the right pelvic and inguinal lymph nodes but not on the left side. Note the abnormal accumulation of tracer in the left thigh. e The patient received 12 months of sirolimus monotherapy. No alleviation of lymphedema was noted. Then, sirolimus was discontinued, and treatment with compression bandaging was initiated. g Twelve months after compression therapy