CRPS and the anaesthetist – A problem all round!
Dr Meredith Craigie, Adelaide, Australia
Paediatric Anaesthetist and Specialist Pain Medicine Physician
Flinders Medical Centre, South Australia
Board member and Chair, Paediatric Pain Working Party
Faculty of Pain Medicine, ANZCA
Once considered rare in children although it had been reported in adults since 1860, Complex
Regional Pain Syndrome (CRPS) has been described in the paediatric population since the 1970s
1
and is increasingly being recognised, especially in adolescents . CRPS presents a variety of
problems for all involved. The child’s problem is one of continuing severe pain out of proportion to
the inciting event, allodynia and hyperalgesia with accompanying vasomotor and sudomotor changes
of varying severity. Being “experts in managing pain”, Anaesthetists are frequently called on to help
manage these patients. This condition presents a number of problems for the Anaesthetist. The
aetiology is unknown and a full understanding the pathophysiology remains elusive. Persistent
inflammatory activity and changing inflammatory profiles between acute and chronic CRPS have been
2
observed . Alterations in peripheral and central sensitisation with changes in brain processing are
3,4
other lines of investigation . Reorganisation of the S1 cortex of the contralateral side to the
affected limb has been demonstrated and appears to be associated with the complaints of
4
neuropathic pain . Most research has been performed in adults. However, quantitative sensory
testing has demonstrated thermal and mechanical sensory changes in children with cold allodynia the
5
most common .
Diagnosis poses another problem. There have been a variety of diagnostic schemes all based on
6
clinical criteria. The Budapest Criteria are the most recent . They have very high sensitivity with
greatly improved specificity compared with the previous criteria set out by the International
Association for the Study of Pain. There are four components to the Budapest Criteria. They
consist of (1) continuing pain, which is disproportionate to any inciting event; (2) report of at least one
symptom in three of four categories (sensory, vasomotor, sudomotor/oedema and motor/trophic); (3)
display of at least one sign at time of evaluation in two or more of four categories (sensory,
vasomotor, sudomotor/oedema and motor/trophic); and (4) no other diagnosis that explains the signs
and symptoms. Although these criteria are commonly used, they have not been validated in children.
There are some differences in the presentation of children compared with adults. CRPS is more
7
common in females and more commonly presents in the lower limb . Early adolescence (12 to 14
years) is the peak age of onset although individual cases have been described in much younger
8
children . Onset can follow a wide variety of events, often minor trauma but also major surgery and
7,9,10
. Occasionally, a trigger cannot be identified. A psychological basis for CRPS
even immunisation
8,11,12
.
has been discounted although psychological factors may contribute to the severity
As in most chronic pain states in childhood or adolescence, CRPS is a problem involving the whole
13
family . Somatization, anxiety, and depression occur in up to 60% of patients. Relationship
problems with peers, school problems and social withdrawal are common. Caregivers have time
away from work, losing income at a time of increased medical expenditure for the child resulting in
14
considerable financial impact .
Treatment of CRPS is also problematic. Physical rehabilitation has long been considered an
15
essential element . The problem has been how facilitate it. Over the years, a variety of oral and
intravenous medications, sympathetic blockade, regional anaesthesia have been used in an
16, 17,18,19
. Anaesthetists are frequently involved at this
endeavour to reduce the severity of the pain
stage, implementing and managing interventional therapies. More novel treatments have been
20, 21, 22
.
reported including intrathecal analgesia, spinal cord stimulation and intravenous pamidronate
However, most children can recover well with non-invasive rehabilitative approaches. Cognitive
behaviour therapy or similar therapies, along with physical therapy in a combined approach forms the
basis of most intensive inpatient and outpatient programs, and most recently, a Day-hospital
23,24
. Changes in both the child’s and parental willingness to self-manage pain are important
program
in achieving functional and psychological improvement and may be facilitated by an interdisciplinary
25
team approach . Generally, much better outcomes are expected in children with CRPS although
26
26
the evidence for this is only fair . Relapse rates may be moderately high . However, early
diagnosis and referral with appropriate intervention is the key to decreasing pain, restoring function
and minimising suffering of children with CRPS.
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