Pediatric emergency medicine trisk 3983 3983
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COMPLEX REGIONAL PAIN SYNDROME TYPE 1 (REFLEX
SYMPATHETIC DYSTROPHY)
Current Evidence
Complex regional pain syndrome type 1 (CRPS1), formerly known as reflex
sympathetic dystrophy (RSD) and also called reflex neurovascular dystrophy
(RND) and amplified musculoskeletal pain syndrome (AMPS), is a poorly
understood disorder characterized by pain, abnormal sensation, and in some
patients circulatory irregularities. Over time, disuse and atrophic changes of the
extremity may develop. Initially thought to be primarily an adult disease, CRPS1
is increasingly being recognized in children. There are often delays in diagnosing
children with CRPS1. Emergency clinicians can play a valuable role by
considering CRPS1 in children with pain and making appropriate referrals for a
prompt, definitive diagnosis. In most patients symptoms of CRPS1 will
eventually resolve, but early treatment may prevent prolonged disability.
Children with CRPS1 as young as 3 years have been described. The average
age of children with CRPS1 is approximately 12 years, girls outnumbering boys
by as much as 6:1. Most cases in children involve the lower extremity. CRPS1
usually follows minor trauma, but some cases develop without an identified
precipitant.
The pathophysiology of CRPS1 is not well understood. Early theories
suggested abnormal synapses develop between sensory afferent nerves and
sympathetic afferents after an injury. “Sympathetic” dystrophy is probably a
misnomer, and nomenclature has changed because local epinephrine and
norepinephrine levels are lower, not higher than normal, and vasodilation, not
sympathetic vasoconstriction, may predominate. Current theories include regional
sensitization of the central and perhaps peripheral nervous system from an initial
injury. Experimental evidence suggests involvement of glutamate and NMDA (N
-methyl-D -aspartic acid) receptors in this process. Patients with other
dysautonomic conditions, including gastrointestinal dysmotility, migraine, cyclic
vomiting, and chronic fatigue, may also have CRPS1.
