Poster 111: Treatment of chronic pain in a 17-year-old with Klippel-Trenaunay-Weber syndrome: a case report.

Abstract

Setting: Outpatient pain management program. Patient: A 17-year-old girl with history of Klippel-Trenaunay-Weber syndrome (KTWS). Case Description: The patient was diagnosed at birth with KTWS, which affected her right leg and resulted in a propensity for bleeding due to cutaneous capillary malformations and severe edema due to deeper venous malformations. At age 3, she underwent amputation of 2 toes and a skin graft in an attempt to arrest bleeding. At presentation, she described constant burning pain of intensity 8 on a 0 to 10 pain scale and allodynia of the lateral ankle and medial thigh, which interfered with sleep, concentration, and gait. A 5% lidocaine transdermal patch to the ankle was added to her existing regimen of 300mg of gabapentin 3 times daily, 200mg of celecoxib every day, and weekly aquatic therapy. Because her pain remained unchanged at the 6-week follow-up, 2 lidocaine patches were placed on her ankle, gabapentin was increased to 300mg 4 times daily, and therapy was increased to twice a week. At 12 weeks, the pain intensity was still rated at 8, and the only change made to the regimen was the addition of a 50μg/h fentanyl transdermal patch. Fentanyl was not tolerated and was stopped by the patient at week 13. Assessment/Results: At 18-week follow-up, her pain had decreased to an intensity of 4 and her functional status and sleep were significantly improved. Discussion: A literature search has revealed no other examples of pain or treatment of pain caused by KTWS. From our experience with this case, gabapentin and lidocaine patch can decrease pain and, consequently, in conjunction with aquatic therapy, can increase the patient’s functional status. Conclusion: The sequence of medication choices and dosages in addition to the exercise modality provided effective treatment in this case of KTWS.