Complex Regional Pain Syndrome in a Young Athlete with Von Willebrand Disease

Abstract

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.

Complex Regional Pain Syndrome in Athletes: Scoping Review

“…Imaging, including X-ray, ultrasound, magnetic resonance, computer tomography, and bone scans, was often used for differential diagnosis. Only four studies reported details about warm- [20,21,26] or cold-type [16] CRPS in the considered clinical case. Time to diagnosis varies from a few days to several months, reaching up to 4 years in Hind et al [15].…”

“…Other conditions, such as psychiatric comorbidities, are often found in CRPS patients, but their relationship has not been clarified yet [32]. Moreover, pathogenic hypothesis has been done to link other comorbidities to the CRPS occurrence, such as microvascular damage in von Willebrand disease [16].…”