Including Parents in the Treatment Of Pediatric Complex Regional Pain Syndrome

Shayleigh K. Dickson, MSN, APN, CPNPAC, CPN


Pediatr Nurs. 2017;43(1):16-21. 

In This Article

What Is CRPS?

CRPS is a clinical diagnosis without specific biomarkers or imaging for confirmation (Borchers & Gershwin, 2014). Historically, CRPS has been called causalgia, algodystrophy, Sudeck's atrophy, or reflex sympathetic dystrophy (Tan et al., 2008). In 1994, the International Association for the Study of Pain (IASP) defined these conditions as CRPS and further classified the pain as type I, without evidence of a nerve lesion, and type II, with evidence of a nerve lesion (Borchers & Gershwin, 2014). Amplified musculoskeletal pain syndrome (AMPS) is a broad term often used to describe complex chronic pain syndromes that include CRPS as well as other conditions, such as fibromyalgia, reflex neurovascular dystrophy (RND), and neuropathic pain. This article will focus on CRPS.

Patients with CRPS typically present after a minor injury or surgery with pain that is unresolved and disproportionate to the inciting event. Approximately 10% of patients have no history of trauma or surgery (Stanton-Hicks, 2010; Tan et al., 2008). Symptoms include sensory, vasomotor, sudomotor/edema, and motor/tropic dysfunction that vary widely both between patients and within a single patient based on unknown temporal factors (Stanton-Hicks, 2010). Table 1 lists specific symptoms within each of these categories. The Budapest Criteria was developed to define CRPS in adults, but no validated clinical criterion for children and adolescents exists (Borchers & Gershwin, 2014). Due to the wide variety of symptom presentations in pediatric patients, a separate clinical criterion to define pediatric CRPS is required. The five documented differences between children and adults with CRPS are 1) a higher prevalence among females, 2) increased incidence in the lower extremity, 3) cooler temperature of the affected limb, 4) less obvious sudomotor symptoms, and 5) more influential psychological factors (Low, Ward, & Wines, 2007; Tan et al., 2008). The diagnosis of CRPS in pediatric patients, therefore, relies on the experience and expertise of the clinician caring for the patient. A critical component of diagnosis is ruling out all other potential causes of the pain. For families who seek resolution of the pain, this process of excluding other causes can be time-consuming and frustrating.

The pathophysiology for CRPS remains under debate. Theories include an exaggerated regional inflammatory response, an autoimmune response, increased sympathetic activity, neurogenic inflammation with increased nociceptive stimulation, deep-tissue microvascular pathology, small-fiber neuropathy, dysfunction in central processing, and genetic predisposition (Borchers & Gershwin, 2014; Stanton-Hicks, 2010; Tan et al., 2008). Describing each theory is beyond the scope of this article, but it is likely that the pathophysiology is a combination of these proposed mechanisms. CRPS is often misclassified as psychosomatic pain or misdiagnosed as a psychiatric condition. Although pain due to CRPS is disproportionate to the inciting injury, the pain has a physiologic cause and should not be dismissed as exaggerated or "made up." Psycho logical and social factors are highly influential and can worsen pain that is already intense and debilitating.