Clinical Question: Does CRPS-I spread from the original site of appearance?

Clinical Bottom line: Yes, CRPS- I does spread. Although the exact frequency of spread of CRPS-I is not available in the published literature, a pattern called ‘Independent Spread’ is estimated by Maleki etal, to occur in 6.4% of CRPS-I patients. Other investigators agree that spread is not uncommon.

Search Profile

Search Terms: 'reflex sympathetic dystrophy AND spread'; 'complex regional pain syndrome AND spread'

Databases:

Pubmed

ISI Web of Knowledge

Citations used for evidence:

1. Maleki J, LeBel AA, Bennett GJ, Schwartzman RJ. Patterns of spread in complex regional pain syndrome, type I (reflex sympathetic dystrophy). Pain. 2000;88(3):259-66.

2. Bhatia KP, Bhatt MH, Marsden CD. The causalgia-dystonia syndrome. Brain. 1993;116:843-51.

The Studies

	Study Attribute		Maleki etal.		Bhatia etal.
	Design		Case series		Case series
	Setting		Pain clinic		National Hospital
	Diagnosis Used		IASP		Schott G.D.1
	Number of subjects		27		18
	Number of Males - n (%)		5 (18.5)		2 (11.1)
	Number of Females		22 (81.5)		16 (88.9)
	Age at presentation - mean (range)		38.7 (22-52)		28.5 (12-56)
	Trauma as initiating factor - n (%)		27 (100)		15 (88.33)
	Upper limb as initial site - n (%)		11 (40.7)		6 (33.3)
	Lower limb as initial site - n (%)		16 (59.3)		12 (66.7)
	Time between injury and onset of CRPS		5 days or less - 55% 6 days or more - 34%		Immediate - 2 years (only range available)
	Time between onset and diagnosis - mean (range)		7 months (1 week - 3 years)		Unavailable
	Time between onset and spread - mean (range)		CS1 - 78 (2 days - 13 months) IS2 - 2.6 years (1 month - 12 years) MS3 - 2.5 years (1 month - 7.6 years)		Few months - 3 years (only range available)
	Mean VAS (range)		6.7 (3-10)		Unavailable
	Dystonia - n(%)		Unavailable		18 (100%)

Footnotes:
¹ CS- Contiguous Spread
² IS – Independent Spread
³ MS – Mirror Spread


The Evidence

• Level of evidence according to CEBM rating3 - IV C
• Four varieties of spread are described:
• Contiguous spread is the most common overall type of spread.
• Present in 100% of cases and preceded other types of spread in Maleki etal’s series.
• Present in 71.4% of cases in Bhatia etal’s series.

	Types of Spread		Contiguous Spread		Independent Spread		Mirror Spread		Dissociated Spread
	Propostions of patients		100%		70%		15%		33%
	Average interval between initial RSD and spread		78 days (2 days - 13 months)		2.6 years (1 month - 12 years)		2.5 years (1 month - 7.6 years)		34 months (1 month - 8 years)
	No identifiable precipitating factor		27%		26%		0		Unavailable

• Therapeutic interventions such as surgery or neural block and compensatory overuse of opposite limb were the two common suspected precipitating factors for spread by Maleki etal.
• The frequency of Independent spread is estimated by Maleki etal, to be 6.4 % of CRPS patients, from Veldman and Goris’s study.²

• No study could be found that described the overall incidence of spread in CRPS patients.
• Both the studies that mentioned spread of CRPS are case series and hence the evidence obtained is not of the best quality.
• Maleki etal’s study was done to delineate the different patterns of spread and inferences were based on retrospective analysis, which may involve substantial recall bias.
• Bhatia etal’s study was a select group of CRPS patients with dystonia and hence the findings may not be completely applicable to CRPS patients without dystonia.
• The precipitating factor for initial onset and spread of CRPS-I in most cases was reported to be trauma in both studies.
• The etiology and theoretical basis of onset and/or spread of RSD/CRPS-I is not well established.

References

1. Schott GD (1986a) Mechanisms of causalgia and related clinical conditions. Brain. 109: 717-738.
2. Veldman PH, Goris RJ.Multiple reflex sympathetic dystrophy. Which patients are at risk for developing a recurrence of reflex sympathetic dystrophy in the same or another limb. Pain. 1996 Mar;64(3):463-6.
3. Bob Phillips, Chris Ball, Dave Sackett, Doug Badenoch, Sharon Straus, Brian Haynes, Martin Dawes “Oxford Centre for Evidence-based Medicine Levels of Evidence”. May 2001.

Appraised by: Prabhav Tella, August 2001