Complex regional pain syndrome in children
- David D Sherry, MD
David D Sherry, MD
- Professor of Pediatrics
- University of Pennsylvania
- Section Editors
- Thomas JA Lehman, MD
Thomas JA Lehman, MD
- Section Editor — Pediatric Rheumatology
- Professor of Clinical Pediatrics
- Cornell University Medical College
- Marc C Patterson, MD, FRACP
Marc C Patterson, MD, FRACP
- Section Editor — Pediatric Neurology
- Professor of Neurology, Pediatrics, and Medical Genetics
- Chair, Division of Child and Adolescent Neurology
- Mayo Clinic College of Medicine
Complex regional pain syndrome (CRPS) is an amplified musculoskeletal pain syndrome that is characterized by extreme pain in a limb out of proportion to the history and physical findings, accompanied by one or more signs of autonomic dysfunction . Although CRPS was first reported in 1864 , it was not until 1971 that CRPS was described in a child . Since the initial pediatric report, CRPS is increasingly recognized in pediatric pain, rheumatology, and neurology clinics [4-6].
The clinical manifestations, diagnosis, and management of CRPS in children are reviewed here. CRPS in adults is discussed separately. (See "Complex regional pain syndrome in adults: Pathogenesis, clinical manifestations, and diagnosis" and "Complex regional pain syndrome in adults: Prevention and management".)
Alternative names for CRPS in the literature include reflex sympathetic dystrophy (RSD), algodystrophy, causalgia, Sudeck atrophy, transient osteoporosis, and acute atrophy of bone. In 1995, a consensus conference grouped these disorders under a single heading of CRPS .
CRPS is divided into two categories:
●Type I occurs in patients with CRPS without a definable nerve lesion.
- Merskey H, Bogduk N. Classification of chronic pain: descriptions of chronic pain syndromes and definitions of pain terms, 2nd edition, IASP Press, Seattle 1994.
- Mitchell S, Morehouse G, Keen W. Gunshot wounds and other injuries of nerves, Lippincott, New York 1864.
- Matles AI. Reflex sympathetic dystrophy in a child. A case report. Bull Hosp Joint Dis 1971; 32:193.
- Bernstein BH, Singsen BH, Kent JT, et al. Reflex neurovascular dystrophy in childhood. J Pediatr 1978; 93:211.
- Sherry DD, Wallace CA, Kelley C, et al. Short- and long-term outcomes of children with complex regional pain syndrome type I treated with exercise therapy. Clin J Pain 1999; 15:218.
- Wilder RT, Berde CB, Wolohan M, et al. Reflex sympathetic dystrophy in children. Clinical characteristics and follow-up of seventy patients. J Bone Joint Surg Am 1992; 74:910.
- Stanton-Hicks M, Jänig W, Hassenbusch S, et al. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain 1995; 63:127.
- Tan EC, Zijlstra B, Essink ML, et al. Complex regional pain syndrome type I in children. Acta Paediatr 2008; 97:875.
- Ashwal S, Tomasi L, Neumann M, Schneider S. Reflex sympathetic dystrophy syndrome in children. Pediatr Neurol 1988; 4:38.
- Barbier O, Allington N, Rombouts JJ. Reflex sympathetic dystrophy in children: review of a clinical series and description of the particularities in children. Acta Orthop Belg 1999; 65:91.
- Berde CB, Lebel A. Complex regional pain syndromes in children and adolescents. Anesthesiology 2005; 102:252.
- Kachko L, Efrat R, Ben Ami S, et al. Complex regional pain syndromes in children and adolescents. Pediatr Int 2008; 50:523.
- Kesler RW, Saulsbury FT, Miller LT, Rowlingson JC. Reflex sympathetic dystrophy in children: treatment with transcutaneous electric nerve stimulation. Pediatrics 1988; 82:728.
- Sherry DD, Weisman R. Psychologic aspects of childhood reflex neurovascular dystrophy. Pediatrics 1988; 81:572.
- Silber TJ, Majd M. Reflex sympathetic dystrophy syndrome in children and adolescents. Report of 18 cases and review of the literature. Am J Dis Child 1988; 142:1325.
- Stanton RP, Malcolm JR, Wesdock KA, Singsen BH. Reflex sympathetic dystrophy in children: an orthopedic perspective. Orthopedics 1993; 16:773.
- Wilder RT. Management of pediatric patients with complex regional pain syndrome. Clin J Pain 2006; 22:443.
- Richards S, Chalkiadis G, Lakshman R, et al. Complex regional pain syndrome following immunisation. Arch Dis Child 2012; 97:913.
- Maihöfner C, Handwerker HO, Neundörfer B, Birklein F. Cortical reorganization during recovery from complex regional pain syndrome. Neurology 2004; 63:693.
- Lebel A, Becerra L, Wallin D, et al. fMRI reveals distinct CNS processing during symptomatic and recovered complex regional pain syndrome in children. Brain 2008; 131:1854.
- Rommel O, Gehling M, Dertwinkel R, et al. Hemisensory impairment in patients with complex regional pain syndrome. Pain 1999; 80:95.
- Ruggeri SB, Athreya BH, Doughty R, et al. Reflex sympathetic dystrophy in children. Clin Orthop Relat Res 1982; :225.
- Silber TJ. Anorexia nervosa and reflex sympathetic dystrophy syndrome. Psychosomatics 1989; 30:108.
- Silber TJ. Eating disorders and reflex sympathetic dystrophy syndrome: is there a common pathway? Med Hypotheses 1997; 48:197.
- de Rooij AM, de Mos M, Sturkenboom MC, et al. Familial occurrence of complex regional pain syndrome. Eur J Pain 2009; 13:171.
- 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; 93:390.
- Sandroni P, Benrud-Larson LM, McClelland RL, Low PA. Complex regional pain syndrome type I: incidence and prevalence in Olmsted county, a population-based study. Pain 2003; 103:199.
- Güler-Uysal F, Başaran S, Geertzen JH, Göncü K. A 2 1/2-year-old girl with reflex sympathetic dystrophy syndrome (CRPS type I): case report. Clin Rehabil 2003; 17:224.
- Jastaniah WA, Dobson S, Lugsdin JG, Petty RE. Complex regional pain syndrome after hepatitis B vaccine. J Pediatr 2003; 143:802.
- Genc H, Karagoz A, Saracoglu M, et al. Complex regional pain syndrome type-I after rubella vaccine. Eur J Pain 2005; 9:517.
- Kozin F, Haughton V, Ryan L. The reflex sympathetic dystrophy syndrome in a child. J Pediatr 1977; 90:417.
- Goldsmith DP, Vivino FB, Eichenfield AH, et al. Nuclear imaging and clinical features of childhood reflex neurovascular dystrophy: comparison with adults. Arthritis Rheum 1989; 32:480.
- Laxer RM, Allen RC, Malleson PN, et al. Technetium 99m-methylene diphosphonate bone scans in children with reflex neurovascular dystrophy. J Pediatr 1985; 106:437.
- Tondeur M, Sand A, Ham H. Interobserver reproducibility in the interpretation of bone scans from patients suspected of having reflex sympathetic dystrophy. Clin Nucl Med 2005; 30:4.
- Graif M, Schweitzer ME, Marks B, et al. Synovial effusion in reflex sympathetic dystrophy: an additional sign for diagnosis and staging. Skeletal Radiol 1998; 27:262.
- Schürmann M, Zaspel J, Löhr P, et al. Imaging in early posttraumatic complex regional pain syndrome: a comparison of diagnostic methods. Clin J Pain 2007; 23:449.
- Meier PM, Alexander ME, Sethna NF, et al. Complex regional pain syndromes in children and adolescents: regional and systemic signs and symptoms and hemodynamic response to tilt table testing. Clin J Pain 2006; 22:399.
- Lightman HI, Pochaczevsky R, Aprin H, Ilowite NT. Thermography in childhood reflex sympathetic dystrophy. J Pediatr 1987; 111:551.
- Dietz FR, Mathews KD, Montgomery WJ. Reflex sympathetic dystrophy in children. Clin Orthop Relat Res 1990; :225.
- Wesdock KA, Stanton RP, Singsen BH. Reflex sympathetic dystrophy in children. A physical therapy approach. Arthritis Care Res 1991; 4:32.
- Lee BH, Scharff L, Sethna NF, et al. Physical therapy and cognitive-behavioral treatment for complex regional pain syndromes. J Pediatr 2002; 141:135.
- Brooke V, Janselewitz S. Outcomes of children with complex regional pain syndrome after intensive inpatient rehabilitation. PM R 2012; 4:349.
- Logan DE, Carpino EA, Chiang G, et al. A day-hospital approach to treatment of pediatric complex regional pain syndrome: initial functional outcomes. Clin J Pain 2012; 28:766.
- Buchta RM. Reflex sympathetic dystrophy in a 14-year-old female. J Adolesc Health Care 1983; 4:121.
- Wheeler DS, Vaux KK, Tam DA. Use of gabapentin in the treatment of childhood reflex sympathetic dystrophy. Pediatr Neurol 2000; 22:220.
- Lalwani K, Shoham A, Koh JL, McGraw T. Use of oxcarbazepine to treat a pediatric patient with resistant complex regional pain syndrome. J Pain 2005; 6:704.
- Low AK, Ward K, Wines AP. Pediatric complex regional pain syndrome. J Pediatr Orthop 2007; 27:567.
- McNicol ED, Midbari A, Eisenberg E. Opioids for neuropathic pain. Cochrane Database Syst Rev 2013; 8:CD006146.
- di Vadi PP, Brill S, Jack T, et al. Intravenous regional blocks with guanethidine and prilocaine combined with physiotherapy: two children with complex regional pain syndrome, type 1. Eur J Anaesthesiol 2002; 19:384.
- Geertzen JH, Rietman JS, Smit AJ, Zimmerman KW. A young female patient with reflex sympathetic dystrophy of the upper limb in whom amputation became inevitable. Prosthet Orthot Int 1997; 21:159.
- Krasna MJ, Jiao X, Sonett J, et al. Thoracoscopic sympathectomy. Surg Laparosc Endosc Percutan Tech 2000; 10:314.
- Petje G, Radler C, Aigner N, et al. Treatment of reflex sympathetic dystrophy in children using a prostacyclin analog: preliminary results. Clin Orthop Relat Res 2005; :178.
- Tong HC, Nelson VS. Recurrent and migratory reflex sympathetic dystrophy in children. Pediatr Rehabil 2000; 4:87.
- Wettrell G, Hallböök T, Hultquist C. Reflex sympathetic dystrophy in two young females. Acta Paediatr Scand 1979; 68:923.
- Sherry DD. DVD: Amplified musculoskeletal pain in children. Diagnosis and treatment. A guide for physical and occupational therapists. www.childhoodrnd.org 2002. 46 minutes.
- Zollinger PE, Tuinebreijer WE, Breederveld RS, Kreis RW. Can vitamin C prevent complex regional pain syndrome in patients with wrist fractures? A randomized, controlled, multicenter dose-response study. J Bone Joint Surg Am 2007; 89:1424.
- Childhood versus adult CRPS
- Local factors
- Role of the central nervous system
- - Psychologic issues
- Genetic predisposition
- CLINICAL MANIFESTATIONS
- Autonomic dysfunction
- Sleep disturbances
- Psychologic findings
- Other findings
- Laboratory studies
- - Blood tests
- - Imaging studies
- Other studies
- DIFFERENTIAL DIAGNOSIS
- Physical and occupational therapy
- Psychologic and behavioral therapy
- Pharmacologic therapy
- Pain-reducing procedures
- Our approach
- Primary care
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS