Complex regional pain syndrome in a young athlete with von Willebrand disease

A 17-year-old female with type 1 Von Willebrand Disease (vWD) developed left medial calf pain while running track. Over the next 6 months, orthopedic surgery, sports medicine, vascular surgery, and neurology treated her under various working diagnoses; however, the pain, allodynia, coldness, and pale skin color worsened. She was admitted to a tertiary pediatric hospital for intractable pain where PM&R diagnosed her with complex regional pain syndrome (CRPS) type 1, began gabapentin, and initiated an aggressive inpatient rehabilitation program. During her 3 weeks of inpatient rehabilitation, passive range of motion of knee extension improved from 40° from extension to full extension, and ankle dorsiflexion improved from 15° from neutral to a consistent range of motion beyond neutral. Additional outcome measures were distance of ambulation and assistive device usage; from admission to inpatient rehabilitation to 2 months postdischarge, her weight-bearing tolerance progressed from nonweight-bearing to partial weight-bearing, and ambulation improved from 20 feet with a three-point crutch gait to unlimited distances with a four-point crutch gait. This is the first known case of a bleeding disorder as the likely underlying microvascular pathology associated with CRPS, a theory exposed in 2010.