Sunday, February 25, 2018

Complex Regional Pain Syndrome: A Pediatric Case Report

CRPS_ped copy

by Anita L. Davis PT, DPT, DAAPM

Complex Regional Pain Syndrome (CRPS) affects adults and children. Its onset is typically associated with fracture, sprain/strain, or other trauma. Yet a small percentage of cases present with a spontaneous onset. Genetic factors have been thought to facilitate the development of CRPS in some patients. The impact of psychological factors has also been debated especially when CRPS occurs in the absence of physical trauma. This case reviews the findings, treatment, and outcome of an adolescent referred with spontaneous onset of CRPS. I will: 1) review the differential diagnostic criteria for CRPS as implemented by a physical therapist and 2) describe the treatment and outcomes. This case illustrates that physical therapists who specialize in complex conditions are equipped to effectively evaluate and treat them.

Episodes of pain are common in children, with some developing into chronic conditions. When there is no known etiology, clinical reasoning and diagnosis become challenging. Survey data indicate that 54% of children have experienced pain in the previous three months with 25% of those reporting chronic symptoms. In that study, painful symptoms were most commonly described as headache, abdominal pain, and limb pain (1).

Complex Regional Pain Syndrome (CRPS) is a painful condition affecting children and adults (2,3).  However, the prevalence of CRPS in children has not been extensively evaluated. Underreporting is suspected due to the time lapse from symptom onset to diagnosis (4,5). Injuries such as fracture, strain/sprain, surgery, or general trauma are commonly associated with CRPS onset; however, in up to 10% of children there is no known etiology (6,7). Patients with a spontaneous onset may possess an inherent susceptibility to the disease. They also tend to be younger and have more intense symptoms (8).

The hypothesis of a genetic link for CRPS proposes that maternal mitochondrial DNA sequence variants can predispose children toward the development of CRPS-I and other dysautonomias (9). This theory correlates with reports that CRPS is approximately three times more prevalent in women compared to men (7,10). Furthermore, de Rooij identified 31 families with two to five family members affected by CRPS, supporting a potential familial relationship. Those families showed an increased frequency of spontaneous onset of CRPS and onset at a younger age (11).

With no gold standard test, establishing a diagnosis of CRPS is primarily based on clinical findings. Several diagnostic criteria have been suggested in the literature including those from the International Association for the Study of Pain (IASP) (12). One set of commonly referenced criteria, known as the Budapest Criteria, was developed at a consensus conference and published in 2010 (13).

These criteria were based on the analysis of four factors to detect statistically significant groupings of signs and symptoms. The factors include sensory, vasomotor, sudomotor, and motor/trophic characteristics, which may be reported by the patient as symptoms or observed by the clinician as signs (10,13-15). According to the Budapest Criteria, when two of four signs and three of four symptoms are present, CRPS can be diagnosed with a sensitivity of 0.99 and a specificity of 0.68, which is more precise than the criteria of the IASP (13). No different criteria have been established for the diagnosis of pediatric CRPS.

Stressful life events more commonly occur in patients with CRPS, which leaves some suspecting a link between psychological factors and CRPS (16,17). Stress, anxiety, and fear have been known to be expressed as physical symptoms (18-20). Children are not immune to stress. One study identified multiple stressors in children with CRPS including marital conflict, school problems, and sexual abuse. Other psychosocial factors affecting a child’s pain include parental enmeshment, neuroticism, fear of failure, and decreased social acceptance. Children with pain were more likely to have a parent with chronic pain serving as a role model (18).

Case Description
In this case study, a 12-year-old girl (CR) was referred for physical therapy with a diagnosis of CRPS. CR developed spontaneous onset of CRPS in the left ankle. She was seen by a physiatrist within one week of the onset of symptoms and referred to physical therapy after three weeks.

Her medical history was negative. Her family history revealed that her mother had been diagnosed with CRPS approximately nine months ago. She underwent physical therapy, sympathetic blockade, and medication management. At the time of CRPS onset in the child, she was in remission.

CR was accompanied by her parents on the initial visit. They were appropriately encouraging and supportive. The parents were married with no apparent relational distress. CR was described as a high-achiever, previously engaged in competitive gymnastics and cheerleading and successfully taking high-school level courses. She enjoyed school with plans to engage in academic and gymnastic contests.

She indicated no distress regarding school, sports, or family relationships. She presented with appropriate situational distress with moderate pain behaviors including facial grimacing, holding, and protective posturing.

Pain prevented normal shoe wear and contact with sheets, and it interfered with bathing. She avoided wearing shoes and experiencing other tactile sensations. She reported the affected foot was colder than the other and was often pale or mottled. Intermittent swelling was reported. She was able to move the foot but experienced increased pain and occasional ”jerking.”

Examination Findings
CR was unable to bear weight on the affected (left) limb and used axillary crutches for mobility. She freely moved the hip and knee for re-positioning. On a number rating scale, her pain intensity ranged from 7-9/10. Of three commonly used rating scales, the Numeric Rating Scale can be administered verbally with greatest ease and clinical utility to measure changes in pain intensity (21). An infrared thermometer showed temperature differences ranging from 1.6°C to 3.2°C at bilateral landmarks. Infrared thermometers have been found to be more accurate for clinical use than traditional ones (22). Limb circumference was measured with a standard measuring tape and revealed 0.6 cm of swelling on the affected side. Her skin color was slightly mottled; there were no skin and nail changes. Active movement was present but limited to approximately 10-12° total ankle plantar- and dorsiflexion.

The patient reported intense pain, swelling, temperature and color changes, as well as intermittent tremor or jerking in the affected foot. Findings included significant pain to non-painful stimuli and changes in color, temperature, and swelling. Temperature differences of 1°C have been cited as significant by the IASP, yet others use differences of 2°C or more as a reference standard for clinical significance (10,12,23). No such criteria for swelling have been proposed. There were no trophic changes in her skin, nails, or hair. Motor changes most associated with CRPS are tremors or other forms of dystonia, none of which was observed at the time of the examination (10,14).

Overall, CR reported four of the four of the symptoms and presented with three of the four signs, which exceeded the recommended count of signs and symptoms which yielded a sensitivity of 86% and specificity of 75% for the diagnosis of CRPS (15). (See Table 1.)

Screen Shot 2016-01-11 at 3.09.58 PM

CR received nine sessions of physical therapy utilizing evidence-based strategies including graded exposure to normal stimuli such as weight bearing, walking, standing, and shoe wearing (24-26). Interventions were implemented using distraction, competition, and humor to encourage the patient’s participation. Positive encouragement was given readily with no reinforcement of pain behaviors.

Various positions were used including sitting on a therapy ball, unilateral tall kneeling, and elevated sitting. In each position, foot contact on the floor was attained with various weight shifts during games or distraction activities. Weight loading was further increased during sitting via manually resisted push/pull with the therapist. She progressed to standing on the Balance Master® with selected targets to guide her weight shifts. Other activities included asymmetrical standing while having the left leg lower to facilitate greater weight bearing. Walking with the Body Weight Support Treadmill system provided graded weight bearing with functional activity. Standing diagonal weight exchanges disguised as a relay were also implemented.

Desensitization was addressed with exposure to textures beginning with silk, then progressing to cotton and standard clothing. Tactile discrimination was also applied with the therapist tracing single digits and letters on the skin in the affected area. Tactile discrimination has been shown to be effective for pain reduction and increased tactile acuity (27).

Prior to the last visit, she received a sympathetic block. She returned to therapy presenting with full weight bearing and no assistive device. She engaged in walking, squatting, balance, and stair stepping reporting 0/10 pain. Color and temperature were symmetrical. There was no evidence of swelling. Her physician did not recommend further treatment.

This patient presented with spontaneous onset and a maternal history of CRPS. The pattern suggests that a genetic tendency is possible. There was no indication of psychological distress that would negatively impact her pain perception. CR’s affect and distress were situationally appropriate and improved as her symptoms subsided. She was motivated to resume her usual activities in academics and gymnastics.

CRPS in children typically responds favorably to conservative treatment (28). When additional treatment is needed, following the treatment algorithm described for adult care is advised. This case followed that pattern with physical therapy and conservative medical management (29,30).

CR’s mother attended most sessions and provided appropriate encouragement, acting as a positive role model during therapy. CR’s past experience in gymnastics may have also given her the self discipline and exposure to difficult tasks that she was able to generalize to her therapy experience.

Her rapid diagnosis and treatment were also positive factors in her recovery. When delays are encountered it is reasonable that patients become more anxious, fearful, and deconditioned, which pose as barriers to a positive outcome.

By knowing the clinical criteria for CRPS, the physical therapist was able to confirm the diagnosis and establish an evidence-based treatment approach. The treatment interventions focused on graded exposure to normal stimulation. Appropriate distractions and encouragement were applied as CR progressed from non-weight bearing to full weight bearing and high-level balance activities.

Specialized evaluations are indicated when complex conditions are presented. This case demonstrates the application of validated tests and measures for CPRS to assist in proper diagnosis. Establishing a correct diagnosis is critical prior to any treatment to ensure that the right condition is being treated, that the treatment is not contraindicated, and to spare the patient from prolonged pain and distress.

Subsequently, a referral to a physical therapist specializing in chronic pain conditions is advised to facilitate optimal recovery. Evidence-based treatment strategies can be progressively implemented to successfully reach the desired effect. In addition, therapists specialized in chronic pain are more likely to consider psychosocial issues that may be influencing symptoms and performance. The ability to recognize this and make appropriate recommendations is the sign of an expert therapist.

1.    Perquin CW, Hazebroek-Kampschreur AA, Hunfeld JAM, et al. Pain in children and adolescents: a common experience. Pain. 2000;87(1):51-58.
2.    Fermaglich DR. Reflex sympathetic dystrophy in children. Pediatrics. 1977;60(6):881-883.
3.    Ruggeri SB, Athreya BH, Doughty R, Gregg JR, Das MM. Reflex sympathetic dystrophy in children. Clin Orthop Relat Res. 1982;(163):225-230.
4.    Lemahieu RA, Van Laere C, Verbruggen LA. Reflex sympathetic dystrophy: an underreported syndrome in children? Eur J Pediatr. 1988;147(1):47-50.
5.    Kachko L, Efrat R, Ben Ami S, Mukamel M, Katz J. Complex regional pain syndromes in children and adolescents. Pediatr Int. 2008;50(4):523-527.
6.    Veldman PH, Reynen HM, Arntz IE, Goris RJ. Signs and symptoms of reflex sympathetic dystrophy: Prospective study of 829 patients. Lancet. 1993;342(8878):1012-1016.
7.    de Mos M, Huygen FJ, Dieleman JP, Koopman JS, Stricker BH, Sturkenboom MC. Medical history and the onset of complex regional pain syndrome (CRPS). Pain. 2008;139(2):458-466.
8.    de Rooij AM, Perez RS, Huygen FJ, et al. Spontaneous onset of complex regional pain syndrome. Eur J Pain. 2010;May;14(5):510-513.
9.    Higashimoto T, Baldwin EE, Gold JI, Boles RG. Reflex sympathetic dystrophy: complex regional pain syndrome type I in children with mitochondrial disease and maternal inheritance. Arch Dis Child. 2008;93(5):390-397.
10.    Harden RN, Bruehl S, Galer BS, et al. Complex regional pain syndrome: are the IASP diagnostic criteria valid and sufficiently comprehensive? Pain. 1999;83(2):211-219.
11.    de Rooij AM, de Mos M, Sturkenboom MC, Marinus J, van den Maagdenberg AM, van Hilten JJ.
Familial occurrence of complex regional pain syndrome. Eur J Pain. 2009 Feb;13(2):171-177.
12.    Merskey H, Bogduk N, editors. Classification of Chronic Pain. 2nd ed. Seatlle, WA: IASP Press; 1994.
13.    Harden RN, Bruehl S, Perez RS, et al. Validation of proposed diagnostic criteria (the “Budapest Criteria”) for complex regional pain syndrome. Pain. 2010;150(2): 268-274.
14.    Bruehl S, Harden RN, Galer BS, et al. External validation of IASP diagnostic criteria for complex regional pain syndrome and proposed research diagnostic criteria. International Association for the Study of Pain. Pain. 1999:81(1-2):147-154.
15.    Harden RN, Bruehl S, Stanton-Hicks M, Wilson PR. Proposed new diagnostic criteria for complex regional pain syndrome. Pain Med. 2007;8(4):326-331.
16.    Geertzen JH, de Bruijn-Kofman AT, de Bruijn HP, van de Wiel HB, Dijkstra PU. Stressful life events and psychological dysfunction in Complex Regional Pain Syndrome type I. Clin J Pain. 1998;14(2):143-147.
17.    Beerthuizen A, van ‘t Spijker A, Huygen FJ, Klein J, de Wit R. Is there an association between psychological factors and the complex regional pain syndrome type 1 (CRPS1) in adults? A systematic review. Pain. 2009;145(1-2):52-59.
18.    Deshpande SS, Vidya G, Bendre NS, Ghate MR. Children with medically unexplained pain symptoms: categorization and effective management. Indian J Psychol Med. 2011;33(2):163-166.
19.    Kozlowska K, Williams LM. Self-protective organization in children with conversion and somatoform disorders. J Psychosom Res. 2009;67(3):223-233.
20.    Shapiro MA, Nguyen ML. Psychological stress and abdominal pain in adolescents. Ment Health Fam Med. 2010;7(2):65-69.
21.    Williamson A, Hoggart B. Pain: a review of three commonly used pain rating scales. J Clin Nurs. 2005;14(7):798-804.
22.    Burnham RS, McKinley RS, Vincent DD. Three types of skin-surface thermometers: a comparison of reliability, validity, and responsiveness. Am J Phys Med Rehabil. 2006;85(7):553-558.
23.    Wasner G, Schattschneider J, Baron R. Skin temperature side differences—a diagnostic tool for CRPS? Pain. 2002;98(1-2):19-26.
24.    Daly AE, Bialocerkowski AE. Does evidence support physiotherapy management of adult Complex Regional Pain Syndrome Type One? A systematic review. Eur J Pain. 2009;13(4):339-353.
25.    de Jong JR, Vlaeyen JWS, Onghena P, Cuypers C, den Hollander M, Ruijkrok J. Reduction of pain-related fear in complex regional pain syndrome type I: the application of graded exposure in vivo. Pain. 2005;116(3):264-275.
26.    Ek JW, van Gijn JC, Samwel H, van Egmond J, Klomp FP, van Dongen RT. Pain exposure physical therapy may be a safe and effective treatment for longstanding complex regional pain syndrome type 1: a case series. Clin Rehabil. 2009;23(12):1059-1066.
27.    Moseley GL, Zalucki NM, Wiech K. Tactile discrimination, but not tactile stimulation alone, reduces chronic limb pain. Pain. 2008;137(3):600-608.
28.    Stanton-Hicks M, Baron R, Boas R, et al. Complex Regional Pain Syndromes: guidelines for therapy. Clin J Pain. 1998;14(2):155-166.
29.    Stanton-Hicks MD, Burton AW, Bruehl SP, et al. An updated interdisciplinary clinical pathway for CRPS: report of an expert panel. Pain Pract. 2002;2(1):1-16.
30.    Wilder RT. Management of pediatric patients with complex regional pain syndrome. Clin J Pain. 2006;22(5):443-448.

Dr. Davis has been practicing Physical Therapy since 1985. She has specialized in chronic pain since 1990. She received her BS in Physical Therapy from the Medical College of Georgia and her doctorate in Physical Therapy from Marymount University. She completed the Brooks Orthopedic Residency in 2007. She is a member of the American Physical Therapy Association, Florida Physical Therapy Association and is a Diplomate in the American Academy of Pain Management. She currently works in an interdisciplinary pain rehabilitation program in Jacksonville, Florida.

This article was originally published in The Pain Practitioner, Fall 2013.

Leave a Reply

Your email address will not be published. Required fields are marked *

More CRPS Stories

About The Academy

The American Academy of Pain Management improves the lives of people with Pain by advancing a person-centered, integrative model of pain care through evidence-guided education, credentialing, and advocacy.