Erik Hansen and Jonathan Isaacs

The patient presented with disproportionate postoperative pain and hyperesthesia extending beyond the surgical field. Classical signs of complex regional pain syndrome (CRPS) including diffuse erythema, swelling, and soft-tissue changes were noted shortly after surgery and did not abate with routine interventions such as elevation, therapy, and even oral steroids. CRPS is a pain syndrome that affects the upper and lower extremities and is of unknown etiology and often presents after seemingly minor trauma, fracture, surgery, or rarely may develop spontaneously. There is an estimated incidence rate of 26.2 per 100,000 person-years. The syndrome most commonly affects Caucasian females with a 2 to 4:1 female-to-male ratio. There may be a genetic component with recent genomic data showing a possible predisposition in association with HLA-DQ1 and HLA-DR3. Severe, intense pain within a week of the injury/intervention, as seen in our patient due to the postoperative hematoma formation, may also be a risk factor or indicator for the development of CRPS.

CRPS is divided into three types: CRPS type I (formerly known as reflex sympathetic dystrophy), CRPS type II (also known as causalgia), and CRPS NOS (not otherwise specified). CRPS type II is characterized by injury to a peripheral nerve with associated abnormalities noted on nerve testing (including anatomic distribution sensory loss, focal percussion sign, electromyography, and nerve conduction studies). CRPS NOS includes patients who only partially meet the above-listed diagnostic criteria but whose condition is not otherwise better explained by another pathologic process. Our patient in question had trophic and autonomic changes such as hypersensitivity, cutaneous discoloration, cold sensations, hyperhidrosis, and edema with shiny-appearing skin. Based on her signs and symptoms, she was diagnosed with CRPS type I. The specific pathogenesis of this condition is unknown, but there is evidence of an elevated inflammatory state with central and peripheral sensitization leading to increased activity of afferent nociceptors and alterations in sympathetic activity.

CRPS is a complicated problem without a straightforward solution or treatment. The management of this condition requires a multidisciplinary and multimodal approach. Additionally, early diagnosis with early treatment initiation may reduce the severity and longevity of the syndrome. The main goal of treatment is restoration of the functional use of the extremity. Other endpoints include pain control, return to work, and improved quality of life. There are ample data indicating the importance of physical and occupational therapy as a vital early and first-line intervention. These modalities improve range of motion, function, edema control, and pain when utilized in the acute setting. A recent Cochrane review showed some supporting evidence for graded motor imagery and mirror therapy in providing improved pain and function in this patient population. These modalities should be used in all patients with findings consistent with CRPS.

Vitamin C is a supplement that has been given prophylactically in high doses after several studies have shown its effectiveness in lowering a patient’s risk of developing CRPS after injury, surgery, or other mechanisms that may increase the possibility of the patient to develop the syndrome. It should be noted that Ekrol et al published a double-blind randomized controlled trial of 336 patients in which vitamin C or a placebo was given prophylactically following distal radius fractures. They found no difference in outcomes or incidence of CRPS between the groups. This was supported by a meta-analysis by Evaniew et al. However, given the low cost and risk of this intervention, it is often still given and recommended for prophylaxis.