CRPS in children: the literature

CRPS affects both adults and children. There is no reason as best the science tells us, that the pathophysiology should be different, involving both central and peripheral mechanisms. This includes inflammation, central nervous system adaptation, immune system activity and the wide range of possible psychosocial influences.

In any persisting condition it is important to be patient-centred and to involve significant others, family and friends. The influence of others’ behaviours should not be underestimated. With children in pain, the primary caregivers are intricately involved in offering loving support, making treatment choices and motivating the young patient to follow the treatment programme.

We learn how to respond to pain early in life, frequently mirroring the behaviours of those around us and looking to them for feedback. It is not uncommon for a child to fall and then look to the parent for their facial and physical response before deciding what to do next.

For these reasons, any treatment programme must involve the parents and other significant people (e.g./ grandparent, teachers).

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Schmerz. 2012 Aug;26(4):389-95.

Please don’t hurt me!: a plea against invasive procedures in children and adolescents with complex regional pain syndrome (CRPS).

[Article in German]

Zernikow B, Dobe M, Hirschfeld G, Blankenburg M, Reuther M, Maier C.

Abstract

BACKGROUND:

Complex regional pain syndrome (CRPS; formerly known as Morbus Sudeck/reflex dystrophy) is diagnosed in children and adolescents, but the clinical presentation is often atypical. Unfortunately, potentially harmful, invasive treatments are used in pediatric patients.

PATIENTS AND METHODS:

A retrospective chart study of pediatric chronic pain patients with CRPS was performed.

RESULTS:

Over the course of 6 years, 37 (35 girls) children and adolescents took part in a multidisciplinary chronic pain inpatient program. At admission, patients took on average 4.4 (range 1-10) different medications and 29 different pharmaceuticals were used overall. Prior to admission, invasive pain treatments were performed without success in 16 of the children (43%). At least 13 children received two or more invasive treatments. Although sympathetic blocks were most prevalent, operations and regional anesthesia were also used.

CONCLUSION:

Despite a lack of evidence for invasive procedures, these continue to be used in children and adolescents with CRPS, who later respond positively to conventional treatment. The English full-text version of this article is available at SpringerLink (under “Supplemental”)

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Acta Paediatr. 2008 Jul;97(7):875-9. Epub 2008 Apr 9.

Complex regional pain syndrome type I in children.

Tan EC, Zijlstra B, Essink ML, Goris RJ, Severijnen RS.

Abstract

BACKGROUND:

Complex Regional Pain Syndrome type I (CRPS I) is a potentially incapacitating syndrome which can occur after a minor injury or operation to a limb. It is a disorder characterized by pain, sensory and motor disturbances. CRPS I is well known in adults, but a relatively new diagnostic entity in children. The clinical presentation of CRPS I in children is, to some extent, different from adults and therefore sometimes not recognized early. The aim of this study was to search for differences in patient characteristics between children and adults with CRPS I.

METHODS:

We have performed a retrospective chart review of 78 children (age </=16 year) with CRPS I and compared the data with those of 951 adults with CRPS I.

RESULTS:

The child population consisted predominantly of girls and older children (median age 13 years). The child population differed from adults in that the skin temperature of the involved extremity at onset was more often cooler, the lower extremity was involved more frequently and neurological and sympathetic symptoms were less pronounced.

CONCLUSIONS:

In several aspects, CRPS I in children has a different presentation than in adults

Click here

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Pain Med. 2010 Aug;11(8):1216-23.

Plasticity of complex regional pain syndrome (CRPS) in children.

Stanton-Hicks M.

Source

Pain Management Department, Center for Neurological Restoration, Consulting Staff, Children’s Hospital CCF Shaker Campus, Pediatric Pain Rehabilitation Program, Cleveland Clinic, Cleveland, Ohio 44195, USA. stantom@ccf.org

Abstract

Complex regional pain syndrome I (CRPS I) is defined by the International Association for the Study of Pain (IASP) criteria to include pain that is disproportionate to the inciting event, sensory disturbances such as allodynia/ hyperalgesia, autonomic dysfunction, and motor dysfunction that usually occurs after trauma that is frequently trivial and generally expressed in an extremity. These symptoms are well described in the adult population, but there are relatively few data or reports of its prevalence in the pediatric population. Recent studies have demonstrated that unlike the adult population, about 90% of the cases reported are females in a range of 8 to 16 years, the youngest being 3 years old. There tends to be delay in recognizing the diagnosis, which may be as long as 4 months. In contrast to adults, the response to treatment, particularly exercise therapy with behavioral management will achieve almost 97% remission. While the pathophysiology is poorly understood, many features, particularly the neurologic abnormalities, suggest both peripheral and central nervous system involvement. Peripheral small fiber neuropathy as an etiology and inflammation involving small nerve fibers (neurogenic inflammatory pain) has been suggested. A tissue inflammatory etiology has been investigated over the past 25 years. However, these inflammatory aspects differ from those seen in other conditions involving tissue inflammation. The suggestion that CRPS in children is a different clinical entity than that seen in the adult, is probably incorrect, as recent evidence would suggest that the pathophysiology is most likely identical involving endocrine, behavioral, developmental, and environmental factors that distinguish clinical presentation in children from the adult. Behavioral management is a mandatory accompaniment of any program of exercise therapy and the sometimes extreme sensory disturbances and parental enmeshment do distinguish the clinical presentation from that in the adult. Interventional procedures may be required in the face of extreme allodynia preventing exercise therapy, and in occasional cases interruption of the sympathetic nerves may reverse this symptom in a few children. Occasionally, continuous analgesia techniques such as that which can be delivered by tunneled epidural catheter or an externalized neurostimulator (spinal cord stimulation) for short periods of time are effective.

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J Am Podiatr Med Assoc. 2012 Mar-Apr;102(2):99-104.

Complex regional pain syndrome of the pediatric lower extremity: a retrospective review.

Harris EJ, Schimka KE, Carlson RM.

Source

Section of Podiatry, Department of Orthopedic Surgery, Loyola University Medical Center, Maywood, IL, USA.

Abstract

BACKGROUND:

Complex regional pain syndrome (CRPS) type 1 is a disorder of the extremities characterized by pain, edema, limited range of motion, integument changes, and vasomotor instability often after an inciting event. In the pediatric population, CRPS may be misdiagnosed, or missed entirely, as CRPS literature for this patient population is lacking.

METHODS:

Twenty-seven pediatric patient medical records with the diagnosis of CRPS type 1 from the institutional and private practices of the principal investigator (E.J.H.) were reviewed for demographics, inciting event, lower-extremity clinical examination, ancillary testing, previous treatments, time to diagnosis, treatment after diagnosis, and time to resolution of symptoms.

RESULTS:

Females composed 85.2% of the patient population (n = 23) (mean age of females, 11.11 years). An inciting event preceded pain in 74.1% of patients (n = 20). On physical examination, more than 50% of patients were identified as having changes in skin color and temperature, edema to the affected lower extremity, painful or decreased range of motion in affected joints, and intact lower-extremity motor function. The average time to resolution of symptoms was 6.8 weeks for the entire population.

CONCLUSIONS:

Diagnosis of CRPS type 1 should be considered in a preadolescent female complaining of pain out of proportion after an inciting event with a physical examination demonstrating change in skin color, decrease in skin temperature, edema, and painful or diminished range of motion in affected joints. Prompt diagnosis can decrease the time to resolution of symptoms.

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Mayo Clin Proc. 2010 Mar;85(3 Suppl):S33-41.

Neuropathic pain in children: Special considerations.

Walco GA, Dworkin RH, Krane EJ, LeBel AA, Treede RD.

Source

Department of Anesthesiology & Pain Medicine, Seattle Children’s Hospital, WA 98105, USA. gawalco@u.washington.edu

Abstract

Neuropathic pain is relatively uncommon in children. Although some syndromes closely resemble those found in adults, the incidence and course of the condition can vary substantially in children, depending on developmental status and contextual factors. There are some neuropathic pain syndromes that are rare and relatively unique to the pediatric population. This article discusses the array of neuropathic pain conditions in children and available treatment strategies. Data are limited by small numbers and few randomized controlled trials. Research and clinical implications are discussed.

Full article here

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