Pediatric complex regional pain syndrome: a review

Pediatric Rheumatology, Apr 2016

Complex regional pain syndrome (CRPS) is a chronic, intensified localized pain condition that can affect children and adolescents as well as adults, but is more common among adolescent girls. Symptoms include limb pain; allodynia; hyperalgesia; swelling and/or changes in skin color of the affected limb; dry, mottled skin; hyperhidrosis and trophic changes of the nails and hair. The exact mechanism of CRPS is unknown, although several different mechanisms have been suggested. The diagnosis is clinical, with the aid of the adult criteria for CRPS. Standard care consists of a multidisciplinary approach with the implementation of intensive physical therapy in conjunction with psychological counseling. Pharmacological treatments may aid in reducing pain in order to allow the patient to participate fully in intensive physiotherapy. The prognosis in pediatric CRPS is favorable.

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Pediatric complex regional pain syndrome: a review

Weissmann and Uziel Pediatric Rheumatology Pediatric complex regional pain syndrome: a review Rotem Weissmann 0 1 Yosef Uziel 0 1 0 Sackler School of Medicine, Tel Aviv University , Tel Aviv , Israel 1 Pediatric Rheumatology Unit, Department of Pediatrics, Meir Medical Center , 49 Tshernichovsky St., Kfar Saba 44281 , Israel Complex regional pain syndrome (CRPS) is a chronic, intensified localized pain condition that can affect children and adolescents as well as adults, but is more common among adolescent girls. Symptoms include limb pain; allodynia; hyperalgesia; swelling and/or changes in skin color of the affected limb; dry, mottled skin; hyperhidrosis and trophic changes of the nails and hair. The exact mechanism of CRPS is unknown, although several different mechanisms have been suggested. The diagnosis is clinical, with the aid of the adult criteria for CRPS. Standard care consists of a multidisciplinary approach with the implementation of intensive physical therapy in conjunction with psychological counseling. Pharmacological treatments may aid in reducing pain in order to allow the patient to participate fully in intensive physiotherapy. The prognosis in pediatric CRPS is favorable. Complex regional pain syndrome; Pediatric; CRPS; Pain amplification syndrome; Chronic pain Background Musculoskeletal pain is the most common reason for referral to pediatric rheumatologists. Causes of chronic musculoskeletal pain include a wide variety of inflammatory or non-inflammatory conditions, such as arthritis, hypermobility, fibromyalgia, growing pains, complex regional pain syndrome (CRPS) and more. Amplified musculoskeletal pain syndrome (AMP) is a generic and descriptive term used to describe chronic pain syndromes of unconfirmed etiology, such as fibromyalgia and CRPS. In AMP, pain signals are amplified, thus mildly painful or nonpainful stimuli are registered by the body as very painful, and this leads to a functional disability – as the patient tries to avoid the induction of pain. In this review we will focus on the condition of pediatric chronic peripheral pain, more commonly referred to as pediatric complex regional pain syndrome (CRPS). Various nomenclature are used to describe this condition, including complex regional pain syndrome (CRPS), reflex sympathetic dystrophy, reflex neurovascular dystrophy, causalgia, and localized idiopathic pain. Matles was the first to describe a case of reflex sympathetic dystrophy in a child [ 1 ]. CRPS is a condition of chronic peripheral pain, usually of a distal extremity. This syndrome is characterized by spontaneous or stimuli-induced pain, which is amplified to a very high visual analog scale (VAS) score, disproportionate to the actual incident trauma/stimulus, in the presence of a wide variety of autonomic and motor disturbances. CPRS is divided into two types. CRPS type I, previously known as reflex sympathetic dystrophy, usually develops after a preliminary event; usually minor a trauma or fracture, without any significant nerve injury. In contrast, CRPS type II, which is very rare in childhood, previously named causalgia, is induced by partial injury of a nerve or one of its major branches [ 2 ]. This review summarizes the current information regarding the epidemiology, etiology, diagnosis, treatment and prognosis of pediatric CRPS. Epidemiology Among the pediatric population (children under 18 years of age), CRPS type I is more common among girls. The mean age at diagnosis is around 12 years of age. The lower extremity is more commonly involved than the upper, with some reports stating a predilection towards involvement of the foot [ 3–6 ]. Information regarding the epidemiology of pediatric CRPS type II is wanting. There are case reports of CRPS type II among children as young as 3 years of age [ 7–9 ]. Currently, there are no data available regarding the incidence of pediatric CRPS. Etiology and pathogenesis The specific causes of CRPS are unknown. There is a general paucity of studies among the pediatric population regarding the etiology and pathogenesis of CRPS, although a variety of studies among adult patients present interesting hypotheses. However, the pathogenesis of pediatric CRPS is not necessarily identical to that of adult CRPS. Trauma In many cases, CRPS follows a relatively minor trauma, usually a sprain, twist, dislocation or soft tissue injury. In some cases, no previous injury was recalled [ 3–6, 10–13 ]. Fractures are the precipitating event in about 5–14 % of cases and surgical procedures in 10–15 % [14]. Psychological factors Stress has an important role in inducing or perpetuating CRPS. Cruz et al. found that children with CRPS showed generally intact cognitive function. However, they demonstrated an elevated risk of somatic symptoms and emotional distress, especially anxiety [ 15 ]. These findings correlate with those of Logan et al., who found that children with CRPS reported greater functional disabilit (...truncated)


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Rotem Weissmann, Yosef Uziel. Pediatric complex regional pain syndrome: a review, Pediatric Rheumatology, 2016, pp. 29, 14, DOI: 10.1186/s12969-016-0090-8