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• Reflex sympathetic dystrophy in children - our experience
  Karen Davies
  Published on: 30 August 2000

• Published on: 30 August 2000

  Reflex sympathetic dystrophy in children - our experience

  • Karen Davies, Clinical researh fellow in paediatric rheumatology

  Dear Editor:

  We noted with interest the recent study reported by Murray et al[1] in which the characteristics of a cohort of 46 children with reflex sympathetic dystrophy (RSD) were reported. In a similar fashion in our paediatric rheumatology unit, chronic idiopathic musculoskeletal pain syndromes including RSD form a significant proportion of referrals. A retrospective review of 23 children referred to us in...

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  Dear Editor:

  We noted with interest the recent study reported by Murray et al[1] in which the characteristics of a cohort of 46 children with reflex sympathetic dystrophy (RSD) were reported. In a similar fashion in our paediatric rheumatology unit, chronic idiopathic musculoskeletal pain syndromes including RSD form a significant proportion of referrals. A retrospective review of 23 children referred to us in whom a diagnosis of RSD was made between April 1993 and November, revealed many similarities with the population described by Murray et al. This is illustrated in the table below:

  Clinical characterisitics of 23 children with RSD compared with 46 described by Murray et al
   

  	Murray et al	Our cohort
  Patients (n)	46	23
  Female (n)	35 (76%)	17 (77%)
  Upper limb (n)	14 (30%)	3 (13%)
  Lower limb (n)	30 (65%)	19 (87%)
  L side (n)	18 (39%)	11 (50%)
  R side (n)	26 (57%)	9 (40%)
  Both sides (n)	2 (4%)	2 (10%)
  Median age at onset RSD	Not reported	11.4 years (range 8.6-16.4)
  Median age at diagnosis (range)	12.0 years (8-15.2)	12.4 years (range 8.8-16.6)
  Mean duration onset - diagnosis (median; range)	23.6 weeks (12; 1-130)	40 weeks (14; 2-260)
  Antecedent traumatic event involving affected limb	25 (54%)	12 (52%)
  Median time of antecedent event to onset of RSD (range)	NR	3.5 weeks (1 - 2 weeks)
  Pre-existing chronic condition (not RSD) involving affected limb	6 (13%)	5 (22%)

  In a similar manner many of our patients had seen multiple medical and health practitioners prior to referral and diagnosis. Five patients had pre-existing medical conditions (2 inactive juvenile idiopathic arthritis, 2 recurrent transient synovitis of the hip, one mild cerebral palsy,and one patient with asthma). A high level of pre-exisiting conditions with psychological components was seen in our cohort with 4 patients reporting a history of chronic recurrent headaches, 1 of chronic abdominal pain, 5 of sleep disorders, 2 of anxiety and 2 of depression. Three patients had a history of conversion disorders: and one recurrent hyperventilation. In addition 11 (48%) had one or more close relatives with a chronic or debilitating illness, and in seven of these it was of musculoskleletal origin. Five patients had a total of 7 first degree relatives with a diagnosed psychiatric condition.

  Presenting symptoms were similar to the reported study with all patients reporting severe pain in the affected limb(s) in addition to restriction of joint range of motion (91%); sensory symptoms (55%); swelling (50%); colour change (55%), and abnormal sweating (14%). Seventeen patients (77%) had used crutches at some point during their illness and 7 (32%) had used a wheelchair.

  Investigation prior to diagnosis was similar to the reported study. Eighteen patients (82%) had had plain radiographs of which 4 showed osteopenia. Eight patients (36%) underwent a bone scan of which 3 demonstrated reduced uptake in the affected limb. No other investigations, which included MRI, EMG, thermography and nerve conduction studies were felt to be contributory to diagnosis or management.

  Six of 19 patients were admitted to another hospital prior to referral to our unit of which only 2 of these were reported to have received in-patient physiotherapy. Ten patients (53%) had outpatient physiotherapy in some form. In contrast 10 patients (53%), were treated with a period of limb immobilisation.

  Examination findings on presentation to our unit included tenderness in 100%, hyperalgesia 76%, reduced temperature 71%; discolouration 67%; muscle wasting 48%; swelling 38%; abnormal sweating 24%, hair loss14%, and skin atrophy in 9%. Fifteen patients required admission to hospital, and all received physiotherapy, in addition to simple analgesics and non steroidal anti-inflammatory drugs as required. All patients had a pscyhological assessment by the paediatric team, received specific education and were taught self-management strategies. All were given a tailored home-exercise programme on discharge. 5 patients in whom adverse psychological factors were felt to be severe were referred for formal clinical psychology assessment and intervention. Intervention included patient and family counselling, behavioural and pain management interventions. Of the patients not admitted, information about RSD was given in the outpatient department, referral made to local physiotherapy services and specific medical follow-up arranged.

  Average follow-up time from diagnosis was 26 months in our group (range 5 months to 7 years). Current status was determined from the time of last clinic visit or by telephone interview (if this was more than 6 months previously). Full outcome information is currently available for 19 patients, 11 of whom were completely well (58%), 5 were clinically improved but still symptomatic (26%), and 3 patients had not improved since diagnosis (15%). Of those who had recovered fully the median time to definite improvement was 4 weeks (range 2-10 weeks) and the mean time to full recovery 25 weeks (range 4 weeks to 15 months). As with the reported study by Murray et al, a high rate of reccurence or relapse in our patient group was seen with 11/19 (58%) having further episodes, of which 4 patients relapsed more than once, and in 2 patients the recurrence developed in different limb.

  A full recovery from the original episode was achieved in 80% of patients. We found that although there was no reduction in the time reported to definite improvement in symptoms, patients admitted had a significantly reduced time interval between diagnosis and full recovery being 3.25 months, (range 1-7 months) compared with 6 months, (range 2-12) months in those not admitted. In our cohort a higher proportion of patients in whom the diagnosis was made within 4 months of onset of RSD made a full recovery (88% compared to 67%).

  We agree with Murray et al[1] that RSD in children is an important cause of morbidity and suggest that earlier recognition and treatment is likely to result in improved outcome. It is clear that psychological issues are common and perhaps under recognised, and we believe it is mandatory that these be actively sought and addressed in all patients, as has been suggested by previous authors.[2,3] Although formal psychological intervention or psychiatric referral may only be required in some patients, we believe psychological assessment and teaching of coping skills and pain management strategies is essential in the management of all patients with chronic pain syndromes including RSD. If intensive outpatient physiotherapy support is not available, admission for a period of intensive physiotherapy should always be considered. We also strongly agree that there is a very limited role for sympathetic nerve blocks, or narcotic analgesia in the management of RSD in children, and that limb immobilisation in this condition is inappropriate and contraindicated.

  Karen Davies
  Specialist Registrar in Paediatric Rheumatology

  R Khubchandani
  Consultant Paediatrician

  Kevin J Murray
  Consultant Paediatric Rheumatologist

  Institute of child Health
  Great Ormond Street Hospital
  London WC1N 3JH, UK
  Kevin.Murray@gos-tr.nthames.nhs.uk

  References

  (1) Murray CS, Cohen A, Perkins T, Davidson JE, Sills JA. Morbidity in reflex sympathetic dystrophy. Arch Dis child 2000;82:231-3.

  (2) Sherry DD, McGuire T, Mellins E, Salmonson K, Wallace CA, Nepom B. Psychomatic musculoskeletal pain in childhood: clinical and psychological analysis of 100 children. Pediatrics 1991;88:1093-9.

  (3) Sherry DD, Wallace CA, Kelley C, Kidder M, Sapp L. Short- and long-term outcomes of children with complex regional pain syndrome type I treated with exercise therapy. Clin J Pain 1999;15:218-23.

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  Conflict of Interest:
  None declared.

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