Wound Treatment Options for Klippel-Trenaunay Syndrome

Problem:

Klippel-Trenaunay syndrome is a rare complex congenital disorder that exhibits capillary malformation, varicosities, bony or soft tissue hypertrophy and a port wine stain.  It can affect several systems, but in this case affected one lower limb of a 23 year old male.  The patient lacked medical insurance and did his own wound care in which pain was a major issue.

Objective:

Patient developed leg ulcers at age 15 which progressively worsened.  Numerous medical interventions were tried with poor outcomes. The literature is limited in describing effective wound management for KT Syndrome.  Patient was seen by Infectious Disease, Vascular, and a Plastic Surgeon who recommended leg amputation.

Methodology:

Our Wound Team sporadically followed the patient over several years.  Patient had used wet to dry dressings as the most cost effective treatment.  Initial dressing was silver impregnated gauze with a calamine Unna boot twice a week to control edema and itching.  The wound proved too exudative for this dressing.  Soft silicone dressing became available and was applied to the open areas allowing for weekly dressing changes and decreased pain. 

Results:

Some of the smaller wounds re-epithelialized.  The patient worked on his feet and continued to take care of his own wounds.  He chose non-adherent gauze and abdominal pads with elastic wraps for cost containment.   

His pain continued to worsen and more ulcers developed with heavier exudate.  Patient presented to the E.R. with 75% of his left lower leg affected by the full thickness ulcers between the knee and ankle. 

Conclusion:

Prior to any amputation, patient was transferred to the Burn/Tissue team for a second opinion.  After serial debridements and with NPWT for several weeks, skin grafts were applied to the leg.  The donor site was covered with the soft silicone dressing, and patient was discharged to be followed in Burn Clinic.