0022-634719611552~.

00I0
Vol. 155. 634-637, February 1996
'1'nE JOVIlHAL OF UROLOGY Printed in U.S.A.
Copyright © 1996 by AMERICAN UROLOGICAL AssocIATION, INC.

UROLOGICAL SYMPTOMATOLOGY IN PATIENTS WITH REFLEX

SYMPATHETIC DYSTROPHY

MICHAEL B. CHANCELLOR, PATRIC J. SHENOT, DAVID A. RIVAS, STEVEN MANDEL AND

ROBERT J. SCHWARTZMAN

From the Departrrumts of Uro~ and Neurology, Thomas Jefferson University, Philadelphia, Pennsylvania

ABSTRACT
Purpose: We determined the effect of reflex sympathetic dystrophy on lower urinary tract
function.
Materials and Methods: A total of 20 consecutive patients (16 women and 4 men) with
neurologically verified reflex sympathetic dystrophy was referred for voiding symp~~s, includ­
ing urgency, frequency, incontinence and urinary retention. No patient had had VOidIng symp­
toms before the initial trauma that induced reflex sympathetic dystrophy. Evaluation included
medical history, physical examination, video urodynamic testing and cystoscopy.
Results: Mean patient age was 43.4 ::!: 10.2 years (range 28 to 58) and mean duration of
urological symptoms was 4.9 ::!: 3.6 years (range 1 to 14). Urodynamic study demonstrated a mean
cystometric bladder capacity of 417 ::!: 182 ml. (range 120 to 700). The urodynamic diagnoses
included detrusor hyperreflexia in 8 patients, detrusor areflexia in 8, sensory urgency in 3 and
detrusor hyperreflexia with detrusor-external sphincter dyssynergia in 1. In 4 women genuine
stress urinary incontinence was also documented urodynamically.
Conclusions: Reflex sympathetic dystrophy may have a profound effect on detrusor and
sphincter function.
KEY WORDS: reflex sympathetic dystrophy; cystitis; urodynamics; bladder, neurogenic; urinary incontinence

Reflex sympathetic dystrophy is a disabling syndrome sacral sprain in 5 and a fall on the coccyx without fracture in 1.
characterized by severe pain with autonomic changes, such None of the patients had voiding symptoms immediately after
as vasomotor disturbances. Reflex sympathetic dystrophy is the initial injury. Urological symptoms became noticeable as
associated with dystrophic changes of the skin, bones or reflex sympathetic dystrophy manifested and progressed.
joints, which usually improve with sympathetic denerva­ A history was obtained, and physical examination, urinal­
tion. 1 The condition usually follows traumatic injury, such as ysis and urine culture, renal ultrasound, cystoscopy using
that occurring in a motor vehicle accident. Other precipitat­ local anesthesia and video urodynamic evaluation were done.
ing factors, including infection, malignancy and surgical pro­ Urine cytology was performed in 6 patients with significant
cedures, have been implicated as precipitating factors. Al­ urgency. Urinary tract infection was ruled out in all patients
though reflex sympathetic dystrophy most commonly affects before urodynamic evaluation, which included simultaneous
the extremities, pelvic and perineal pain has also been at­ video, pressure and electromyographic components. The uro­
tributed to this syndrome. 2 • 3 Galloway et al recently pro­ dynamic diagnoses were established based on International
posed reflex sympathetic dystrophy as a potential etiology for Continence Society standards.5 Filling pressures were con­
the syndrome of chronic interstitial cystitis. As urologists at sidered normal if they were less than 20 em. water at bladder
an institution serving as a regional center for the treatment capacity. Detrusor hyperreflexia was considered when invol­
of retlex sympathetic dystrophy, we have noted that voiding untary detrusor contraction occurred at 15 cm. water or
dysfunction is a significant complaint in some patients. We more. Detrusor-external sphincter dyssynergia was defined
report urological symptomatology in patients with reflex as persistent and involuntary electromyographic activity
sympathetic dystrophy. dUring an involuntary detrusor contraction. Oral terazosin (5
mg.) was given nightly for 1 month to patients 16 and 19 with
METHODS detrusor hyperreflexia (see table).
A total of 20 consecutive patients (16 women and 4 men)

with neurologically verified reflex sympathetic dystrophy

RESULTS
was referred by the regional reflex sympathetic dystrophy Mean patient age was 43.4 ± 10.2 years (range 28 to 58)
center for urological evaluation of severe voiding symptoms, and mean duration of urological symptoms was 4.9 ± 3.6
including frequency, urgency and/or urinary incontinence. In years (range 1 to 14). None of the patients had had voiding
each case lower urinary tract symptomatology did not occur· symptoms before the initial trauma that induced reflex sym­
until after the initial injury to which the development of pathetic dystrophy. The distribution of urological complaints
reflex sympathetic dystrophy was attributed. None of the varied, including frequency, urgency, urinary retention and
patients was diagnosed with reflex sympathetic dystrophy incontinence. Painful bladder symptoms were not significant
because of urological complaints. (see table).
The original iIUury that caused reflex sympathetic dystrophy Endoscopic evaluation failed to reveal bladder tumors, ul­
included blunt foot iIUury in 4 patients, blunt leg iI\jury in 5, ceration, pinpoint bleeding at cystoscopic capacity or other
blunt hand and arm injury in 2, blunt groin injury in 1, minor intravesical pathology and, therefore, bladder biopsy was not
lower extremities orthopedic surgery in 2, cervical and Iumbo- routinely perfonned. Urine cytology showed no malignancies.
No upper tract pathology, including hydronephrosis, nephro­
A«epted for publication June 2, 1995. lithiasis or solid masses, was seen on renal ultrasound. Uro­
634
 REFLEX SYMPATHETIC DYSTROPHY 635
Patknt demographies
Reflex
Pt.-Age-Sex Sympathetic Previous Cyatometric
Chief Urinary Urodynamic Urological
No. Dystrophy Intervention Capacity
Complaint (mi.)
Diagnllllia Treatment
Duratinn (yra.)

1 -51-F AntichnUnergics, pelvic floor Urgency 120 Detrusor hyperreflexia Epidural bloek

stimulator

2 -39-F 9 Multiple orthopedic opera- Urinary retention 676 Detrusor aretlilDa Intermittent selfoQtheter­
tinna. dnrsal column stim- ization

ulator

3 -28-F Sympathetic blocks Urge incontinence 300 Detrusor hyperreflexia, Anticholinergica, pelvic

urethral hypennobilit;y tloor~

stress urinary inconti­

nence

4 -53-M 6 Anticholinergics Urgency, post-void 240 Detrusor hyperrefluia, Estemal sphinc:terotomy

fUllness detrusor-utemal

sphincter dyssyDerpa

5 -45-F 5 Epidural blocks Urge incontinence 200 Detrusor hyperret19ia AnticholiDergica, timed

voiding

6 -50-M 3 Lumbar laminectomy Urinary retention 500 Detrul!ol" areflexia Intermittent aelfoQtheter­
ization

7 -31-F 14 Dorsal column stimulator Miud incontinence 200 Detrusor hyperreftexia, Anticbalinergics. pelvic

urethral hypermobility Door uen:ises

stress urinary inconti.

nence

8 -55-F 7 Multiple orthopedic opera- Urgency 250 Sensory urgency Anticho1iDergica, timed
tions voiding
9 -42-F 1 Epidural block Urinary retention 570 Detrusor areflexia Intermittent selfoQtheter­
ization
10 -28-M 4 Epidural blocks Urgency, urge incouti- 300 Detrusor hyperref10ia AntichoUnergic:a, timed
nence voicIiDg
11 -33-F 8 Lamin~y Urgency 263 Sensory urgency Anticholinergics, timed
voicIiDg
12 -58-M 7 Multiple orthopedic opera- Nocturia 10 time&' 563 Detrusor areOnia Intermittent aelfoQtheter­
tions night ization
13 -54-F 10 Multiple orthopedic opera- Frequency. dysuria 600 Sensory urgency AnticbolineT1Pca. timed
tions voiding, fluid restriction
14-50-F 7 Multiple orthopedic opera- Urinary retention 600 Detrusor areflexia Intermittent BelfoQtheter­
tions ization
15 -32-F 4 Multiple orthopedic opera- Urinary retention 700 Detrusor areflexia Intermittent self-eatheter­
tiona ization
16 -51-F 5 Total abdominal bysterec- Urgency 370 .Detrusor bypenefluia, Antic:boUnergies. tBrazosin
tomy urethral bypel'lllObility
stress urinary inclonti·
nence
17 -42-F Epidural block Urinary retention 570 Detrusor areflexia Intermittent aelf-eatbeier­
ization
18 -29-F 2 Lumbar laminectomy Urinary retention 600 Detrusor areflilDa Intennittent self-catbeter­
ization
19 -42-F Sympathetic blocks Urg~, sensation of in- 416 Detrueor bypenefie»a Antiebolinergic:s. terazo8in,
complete emptying timed voiding
20 -55-F 2 Epidural blocks Urgency, severe in- 300 Detrueor hyperrefle»a, Anticbolinergica. periure­
continence intrinsic sphincteric tbra1 collagen iJljec:tion
deficiency 8b'e88 uri­
nary incontinence

dynamic evaluation demonstrated detrusor hyperreflexia in In cases of autonomic dysreflexia an acute noxious stimu­
8 patients, detrusor areflexia in 8, sensory urgency in 3 and lus applied to the body below the level of the injury, such as
detrusor hyperreflexia with detrusor-external sphincter dys­ bladder or bowel distension, results in a sudden massive
synergia in 1. Mean cystometric bladder capacity was 417 :t unchecked sympathetic discharge, which causes vasocon­
182 mJ. (range 120 to 700). In 4 women genuine stress uri­ striction below the level ofthe spinal cord injury. It manifests
nary incontinence was also documented urodynamically. as a dramatic increase in systemic blood pressure. Parasym­
Urological treatment included anticholinergic medications, pathetic reaction above the level of the lesion may cause
timed voiding, moderate fluid restriction, pelvic floor exercises, facial flusbing, diaphoresis and reflex bradycardia.7
terazosin, periurethral collagen injection, an epidural block and In contrast, reflex sympathetic dystrophy is a chronic sym­
extemal sphincterotomy, The 2 women who were given terazo­ pathetic nervous system dysfunction, which progresses
sin for voiding symptoms tolerated the drug well without side steadily in a series of identifiable stages.l The initiating
effects but they reported no subjective improvement. event is often a traumatic injury, such as the sudden decel­
eration encountered in a motor vehicle accident. Stage I
DISCUSSION reflex sympathetic dystrophy, the acute stage, is character­
Pain, edema and autonomic dysfunction are the chief ized by pain that is out of proportion to that expected for the
symptoms of early reflex sympathetic dystrophy. In later initiating injury. It is usually described as localized, deep
stages movement disorders and topical changes are promi­ burning or aching exacerbated by movement or emotional
nent. Bone changes, ranging from osteopenia to marked disturbance. Edema, hyperthermia or hypothermia and in­
demineralization or ankylosis, are often described. l Reflex creased nail growth are common. Roentgenography may
sympathetic dystrophy differs from autonomic dysreflexia, show evidence of demineralization even at this early stage.
Which is the sympathetic nervous system dysfunction most In stage II reflex sympathetic dystrophy, the dystrophic
commonly encountered by urologists in patients with spinal phase, burning pain increases and emotional disturbanees
cord injury above the 1'7 level.8 are common, such as anxiety and depression. Edematous
636 REFLEX SYMPATHETIC DYSTROPHY

areas may progress to the point of induration. The skin may empirical therapy of detrusor hyperreflexia in the 2 women
appear shiny, bronzed and cool with cyanosis and mottling. with reflex sympathetic dystrophy in our series.

Alopecia may develop in previously hair-bearing skin while To our knowledge our report represents the first series of

the nails become severely dull and brittle. urodynamically verified neurourological dysfunction associ­

Stage III, the atrophic phase, is characterized by further ated with reflex sympathetic dystrophy. Our study illus­
increases of pain resulting in severe, often crippling hyperas­ trates that significant lower urinary tract dysfunction may
thesia. Motor changes are common, including weakness, develop as a direct result of or in association with sympa­
tremor. spasm. dystonia and increased deep tendon reflexes. thetic nervous system dysfunction. Our patients had complex
The fascial tissues lose thickness, and cartilage. muscle and histories, and underwent a number of invasive and noninva­
joints atrophy, resulting in contracture of the extremities. sive treatment modalities for pain. It is possible that pre­
Roentgenography often reveals marked bony demineraliza­ vious injuries and invasive therapies rather than reflex
tion in the fully manifested syndrome. sympathetic dystrophy alone contributed to the voiding
The diagnosis of reflex sympathetic dystrophy is clinical symptoms. We attempted to include only those in whom
and currently there is no consensus for its diagnosis.'! Roent­ voiding symptoms developed concurrently with progressive
genography, bone scintigraphy and differential sympathetic reflex sympathetic dystrophy symptoms. Patients with reflex
neural blockade have been used to support the diagnosis. A sympathetic dystrophy but more severe initial injury, caus­
number of pain syndromes, such as causalgia, Sudeck's atro­ ing herniated intervertebral disks in the cervical, thoracic,
phy and algoneurodystrophy, are considered clinical variants lumbar or sacral spine, were not included. Furthermore,
of reflex sympathetic dystrophy. I patients with acute development of voiding symptoms after
The etiology and pathogenesis of this disorder are unclear. back surgery were not included. Treatment for reflex sympa­
Numerous theories have been suggested to account for the thetic dystrophy, including anticholinergic therapy for pa­
manifestations of the disease process. The common features tients with detrusor hyperreflexia and intermittent catheter­
of reflex sympathetic dystrophy (burning pain, hyperalgesia ization programs for those with detrusor areflexia, was based
and dystrophic changes accompanied by vasomotor distur­ on urodynamic results and was largely successful. The 2 most
bances after nerve plexus or soft tissue injury) may improve common treatment options in our series were anticholinergic
with sympathetic intervention. Effective therapy includes drugs and intermittent self-catheterization. 14
the blockade of sympathetic activity using epidural or re­ A theory of the pathogenesis of reflex sympathetic dystro­
gional injections. systemic sympathetic antagonists or surgi­ phy pain is that tissue injury sensitizes C-fiber nociceptors
cal sympathectomy. I via al-adrenoceptors. In undamaged nerves sympathetic
There are few previous reports of the urological manifes­ stimulation has a suppressive effect on C-fiber activity. In­
tations of reflex sympathetic dystrophy. To our knowledge no volved tissue may have an extra increase in adrenoceptors to
previous evaluations of patients with reflex sympathetic dys­ increase the discharge rate in response to sympathetic stim­
trophy have included a thorough documentation of urody­ ulation. is We are presently studying intravesical capsaicin, a
namic diagnoses of the voiding dysfunction that may be as­ C-fiber neurotoxin, for the treatment of reflex sympathetic
sociated with reflex sympathetic dystrophy. Chalkley et al dystrophy and interstitial cystitis.
described a case of suspected reflex sympathetic dystrophy of The urological symptoms and urodynamic findings of the
the penis that developed 1 year after transurethral prosta­ reflex sympathetic dystrophy patients in our study are sim­
tectomy.9 The symptoms were disabling burning pain in the ilar to those of other neurologically impaired patients with
penis accompanied by penile hypothermia. This condition voiding symptoms. Notably pelvic or suprapubic pain was not
was successfully treated using epidural nerve blocks. Olson a significant complaint. There is no proved explanation for
reported on another patient with apparent reflex sympa­ the urological dysfunction among our patients. However, our
thetic dystrophy who had a severe perineal and penile burn­ study suggests that sympathetic dysfunction can result in
ing sensation after the treatment of metastatic colon cancer the development of neurogenic lower urinary tract dysfunc­
with surgery and radiation. 2 Symptoms were alleviated by tion.
bilateral lumbar sacral sympathectomy. Recently Stevens et Parallels may be drawn to the autonomic neuropathy of
al reported on a patient with a sympathetically maintained diabetes mellitus, which can affect the bladder and urethral
pain syndrome who was given terazosin orally.lo The condi­ sphincter. Detrusor hyperreflexia and especially areflexia
tion responded well to sympathetic blocks for short periods are common urological sequelae of diabetes mellitus. 16. 17 The
but not to oral opioids, anti-inflammatory medications and implication of detrusor hyperreflexia in diabetes cystopathy
muscle relaxants. Symptoms rapidly resolved with the initi­ is that cortical or spinal regulatory tracts have been affected.
ation of terazosin therapy. The implication of abnormal a­ In conclusion, reflex sympathetic dystrophy may have a
receptor activity in these chronic pain syndromes is sup­ profound effect on detrusor and sphincter function. The spec­
ported by the finding of increased responses of various a-ad­ trum and severity of lower tract dysfunction in reflex sym­
renoceptors to locally infused norepinephrine in the affected pathetic dystrophy patients vary markedly.
compared to unaffected limbs and the normal contralateral
limb of these patients. II
Ghostine et al reported on 40 patients with sympathetically
mediated pain who were treated with 40 to 120 mg. phenoxy­ REFERENCES
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toms. 12 Followup ranged from 6 months to 6 years with no Neurol. Neurosurg., 6: 531, 1993.
reported recurrences. When reflex sympathetic dystrophy has 2. Olson, W. L., Jr.: Perineal reflex sympathetic dystrophy treated

been relieved by sympathetectomy, intradermal injection of with bilateral lumbar sympathectomy. Ann. Intern. Med., 113:

phenylephrine hydrochloride (predominantly al agonist) but 633.1990.

not clonidine hydrochloride (predominantly a2 agonist) into the 3. Schwartzman, R, J. and Mclellan, T. L.: Reflex sympathetic

formerly painful area causes reactivation of pain, suggesting dystrophy. A review. Arch. Neurol., 44: 555, 1987.
that 01 receptors may mediate sympathetically maintained 4. Galloway, N. T., Gabale. D. R. and Irwin, P. P.: Interstitial
cystitis or reflex sympathetic dystrophy of the bladder? Sem.
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reflex sympathetic dystrophy have been described in the head, 5. Abrams, P., Blaivas, J. G., Stanton, S. L. and Andersen. 'J. T.:
neck and trunk. Terazosin is reportedly effective in the treat­ Standardisation of terminology oflower U1;nary tract function.
ment of autonomic dysreflexia l:l but it was not effective for Neurourol. Urodynam., 7: 403, 1988.
 REFLEX SYMPATHETIC DYSTROPHY 637
6. Erickson, R. P.: Autonomic hyperreflexia: pathophysiology and
Alar, C. G.: Phenoxybenzamine in the treatment of causalgia.
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Report of 40 cases. J. Neurosurg., 80: 1263, 19M.
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Jr.: Prospective evaluation of terazosin for the treatment of

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autonomic dysret1exia. J. UfO!., 151: 111, 1994.

8. Ochoa, J. L.: Reflex sympathetic dystrophy; a disease of medical

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understanding. Clin J. Pain, 8: 363, 1992.

Institute ofArthritis, Diabetes, Digestive and Kidney Diseases

9. Chalkley, J. E., Lander, C. and Rowlingson, J. C.: Probable reflex

workshop on interstitial cystitis, National Institutes of Health,

sympathetic dystrophy of the penis. Pain, 2&: 223, 1986.

Bethl!8da, Maryland, August 28-29,1987. J. UraL, 140: 203,

10. Stevens, D. S., Robins, V. F. and Price, H. M.: Treatment of 1988.

sympathetically maintained pain with teraz08in. Reg. Anesth., 15. KDltzenburg, M. and McMahon, S. B.: The enigmatic role of the

18: 318, 1993.

sympathetic nervous system in chronic pain. Trends Pharma­

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col. Sci., 12: 399, 1991.

Carruthers, S. G.: Increased venous alpha·adrenoceptor re­

16. Ellenberg, M.: Development of urinary bladder dysfunction in

sponsiveness in patients with reflex sympathetic dystrophy.

diabetes mellitus. Ann. Intern. Meel., part 2, BZ: 321, 1980.

Ann. Intern. Med.• 118: 619, 1993.

17. Frimodt-M.Uer, C.: Diabetic cystopathy: epidemiology and re­

12. Ghostine, S. Y., Comair, Y. G., Turner, D. M., Kasaell, M. F. and lated disorders. Ann. Intern. Mee!., part 2, 92: 318, 1980.

Spinal Cord Stimulation in the Treatment

of Complex Regional Pain Syndrome
(CRPS) of the Lower Extremity:
A Case Report
Julie Saranita, 00, 1 Douglas Childs, DPM, FACFAS, 2 and
Anthony D. Saranita, DPM, FACFAS2
Complex regional pain syndrome (CRPS) is a condition that is often associated with the extremities. This
chronic pain syndrome, when localized to the lower extremity, includes peripheral changes such as
edema, temperature alterations, limited range of motion, loss of or excessive perspiration, pain out of
proportion to any stimulus, and trophic alterations of the skin, hair, and nails. In this report, we describe
the case of a patient who developed complex regional pain syndrome following an ankle injury and
surgery. This case report highlights treatment options that are available to patients experiencing complex
regional pain, including the use of a spinal cord stimulator. Level of Clinical Evidence: 4 (The Journal of
Foot & Ankle Surgery 48(1):52-55,2009)

Key Words: causalgia, complex regional pain syndrome, CAPS, reflex sympathetic dystrophy, spinal
cord stimulation

failed to respond to conservative modalities such as physical

Complex regional pain syndrome (CRPS), formerly therapy and pharmacological interventions were left to deal
known as reflex sympathetic dystrophy, is often a devastat­ with continued and debilitating pain. Currently, the treat­
ing neuropathic condition that has, in recent years, been ment of CRPS includes early intervention and implantation
recognized with increasing frequency in the lower extrem­ of a spinal cord stimulator, as soon as it becomes apparent
ities. Patients with CRPS, who are not diagnosed and treated that less invasive modalities fail to diminish and provide
in a timely fashion, may worsen to such a degree that the pain relief (2). Unfortunately, delaying the diagnosis and
individual may never return to a satisfactory and productive treatment of CRPS can adversely affect the response to
life. Spinal cord stimulation (SCS) has been used in the treatment (2). Many clinicians feel that an early and accu­
treatment of neuropathic pain since 1967 (l). Although it rate diagnosis of the condition provides the greatest chance
was infrequently used during the 1970s and 1980s, SCS has of a full recovery and an improved quality of life.
gained in popularity over the past 15 years because of
technological progress that has directly impacted the use of
implantable systems. Before the use of SCS, patients who
Case Report

Address correspondence to: Julie Saranita. DO. Diplomate. American A 69-year-old female presented to her podiatric physician
Board of Anesthesiology. Director of South Lake Pain Institute. P.A.• 845 for a work-related crush injury to the left foot. The injury
Oakley Seaver Drive. Clermont. FL 34711. E-mail: jsarunita@ was initially treated by a different physician, who had been
yahoo.com
1Diplomate, American Board of Anesthesiology; Subspecialty Certifi­ appointed by the patient's workers' compensation adjustor.
cation in Pain Medicine; Director of South Lake Pain Institute. P.A.• The initial surgeon had already performed a primary repair
Clermont. FL.
of the patient's ruptured lateral ankle ligaments. After the
2Diplomates. ABPS. Teaching Faculty at Florida Hospital East Orlando
Podiatric Surgical Residency Program; Private Practice Corporate Office: initial surgical intervention and subsequent recovery, the
Orlando Foot and Ankle Clinic, Orlando, FL. patient was discharged from the previous surgeon's prac­
Financial Disclosure: Drs. Julie and Anthony Saranita have received tice. Several months thereafter, she presented to our practice
lecture fees from Boston Scientific. formerly known as Advanced Bionics.
None of the authors have stock, equity. or other financial interests in with a complaint of continued pain and instability involving
Boston Scientific. Dr. Childs has no relevant disclosures. her left anl.$:le. Following historical interview and physical
Conflict of Interest: None reported. examination, a diagnosis of chronic left ankle lateral liga­
Copyright © 2009 by the American College of Foot and Ankle Surgeons
1067-2516/09/4801-0009$36.00/0 mentous instability was made and, after considering treat­
doi: 1O.1053/j.jfas.2008.1O.003 ment options, the patient underwent revisionallateral ankle

52 THE JOURNAL OF FOOT & ANKLE SURGERY

FIGURE 1 Anteroposterior fluoroscopic view showing spinal cord FIGURE 2 Lateral fluoroscopic view showing the spinal cord stim­
stimulator lead in the epidural space at the cephalic aspect of the ulator lead In the posterior epidural space.
10th thoracic vertebra.

Corporation, Valencia, CA) SCS. The patient responded

ligamentous reconstruction using a split peroneus brevis
favorably to the SCS trial, reporting a 75% reduction of her
tendon graft. Initially, the patient appeared to respond fa­
pain. Based on her response to the SCS trial, a permanent
vorably to the procedure and temporarily returned to work.
SCS (Figures I and 2) was implanted 8 weeks following
Over time, however, she displayed increased anxiety and
removal of the trial leads. Over the ensuing several weeks,
her left foot exhibited persistently worsening edema and
the patient reported clinically significant pain reduction,
discoloration. She subsequently reported progressive burn­
improved sleep, and increased activity level after implanta­
ing pain in the left foot. Despite extensive physical therapy,
tion of the permanent SCS system. At the time of her last
anxiolytic therapy, peripheral nerve blocks, and adjunctive
follow-up evaluation, 6 months following implantation of
pharmacological management, her condition failed to im­
the permanent SCS, she related that her subjective VAS
prove. The presence of hyperalgesia, edema, allodynia, and
pain scale score was 2 out of 10.
skin discoloration, raised concerns about the possibility of
CRPS, and the patient was referred to an interventional pain
medicine physician for further evaluation and treatment at Discussion
approximately 4 weeks following the second left ankle
operation. CRPS can be debilitating, and is often difficult to treat.
At the time of her initial evaluation with the pain medi­ The controversial role of sympathetic nervous system in­
cine specialist, a diagnosis of CRPS was confirmed. Her volvement in reflex sympathetic dystrophy (RSD), lack of
pre-procedural subjective visual analog scale (VAS) pain evidence for a reflex mechanism, and the small subgroup of
core was reported by the patient to be 8 out of 10 in patients who present with dystrophy, led to a revision of the
intensity. Chronic pain therapy was initiated with the use of terminology used to describe this condition (3) and, in 1994,
an anticonvulsant (gabapentin titrated over 3 weeks, up to the International Association for the Study of Pain (IASP)
600 mg orally 3 times daily), a tricyclic antidepressant changed the terminology so that RSD would thereafter be
(nortriptylene, 50 mg orally at bedtime), topical com­ referred to as complex regional pain syndrome type I (CRPS I),
pounded analgesic cream (ketamine, clonidine, capsaicin and causalgia would thereafter be referred to as CRPS
amitriptyline, and ketoprofen applied to the affected area 3 type II (4).
to 5 times daily), an opioid analgesic (methadone, 5 mg It is interesting, moreover, to note that although CRPS I
orally every 8 hours), and 4 separate lumbar sympathetic has been a recognized clinical entity for more than a cen­
nerve blocks (LSB). These treatment interventions failed to tury, even today early diagnosis is often missed. One of the
provide satisfactory pain relief after 12 weeks, and she key clinical features of CRPS I is the presence of pain out
experienced a number of adverse side effects related to the of proportion to the stimulus, with a nondermatomal distri­
medications. Moreover, she experienced only temporary bution. It was once thought that these patients had a psy­
pain relief following LSB. In an effort to improve her chogenic disorder, but to date no empirical evidence has
response to therapy, the decision was made to perform a substantiated this claim (5). A retrospective study found that
5-day SCS trial using the Precision Plus (Boston Scientific patients with CRPS had, on average, seen 4.8 different

VOLUME 48, NUMBER 1, JANUARY/FEBRUARY 2009 53

physicians and had received an average of 5 different types clude anticonvulsants, tricyclic antidepressants, nonsteroi­
of treatments before being referred to a pain center, and the dal anti-inflammatory drugs (NSAlDS), corticosteroids, and
mean duration of symptoms before evaluation by a pain topical compounded creams. Intrathecal therapy may also
specialist was approximately 30 months (6). be effective in certain cases where intolerable side effects
Currently, there is limited epidemiological data pertain­ occur with high doses of opioids. Sympathetic nerve blocks
ing to the incidence of CRPS. A population-based study at have also been used to reduce the pain associated with
the Mayo Clinic found that the median age of onset of CRPS, to facilitate physical therapy, and to aid in the
CRPS was 46 years, and that it occurred 4 times more determination of whether or not the sympathetic nervous
frequently in females than males (7). Furthermore, the de­ system is involved in the maintenance of pain. Sympathetic
velopment of CRPS is usually associated with trauma or blocks are performed under fluoroscopic guidance by inject­
surgery, although the condition can arise without any pre­ ing a local anesthetic agent on the stellate ganglion for
cipitating traumatic event. According to the IASP, the clin­ treatment of the upper extremity, or on the lumbar sympa­
ical features to be taken into account for diagnosing CRPS thetic chain for treatment of the lower extremities. In some
type I include the presence of regional and continued pain cases, surgical sympathectomy has been shown to be ben­
disproportionate to any inciting event, sensory changes such eficial in the treatment of sympathetically maintained pain
as allodynia and hyperalgesia, sudomotor alterations, (9); however, this procedure is generally reserved for cases
edema, vasomotor instability (temperature changes and skin in which extensive conservative treatment has failed. Phys­
discoloration), and exclusion of any other condition that ical therapy, as well as cognitive and behavioral therapies
would account for the above-mentioned signs and symp­ are important adjunct modalities that should also be consid­
toms. CRPS n (causalgia) includes the aforementioned fea­ ered in the multidisciplinary approach to the treatment of
tures accompanied by a specific peripheral nerve lesion (3), CRPS.
and the sensory changes that usually accompany this diag­ Since its first use in 1967 by Shealy et al (1), SCS has
nosis include burning, aching, pain to light touch, and an been widely used for the treatment of chronic pain. A spinal
exaggerated response to noxious stimuli. As CRPS persists, cord stimulator is an implantable medical device that gen­
trophic nail and hair alterations, as well as skeletal muscle erates electrical pulses that stimulate the dorsal column
weakness, tremor, and dystonia may develop, and radio­ fibers of the spinal cord. The electrical current produced by
graphs often display patchy demineralization of long bones. an implantable pulse generator (IPG) is carried through
In severe cases involving the lower extremity, contractures either a single- or dual-lead cathode to the spinal cord. The
may be observed, and dystonia may present with resultant location of the lead(s) in the epidural space effects stimu­
equinovarus position of the foot. Still further, sudomotor lation of the desired dermatome. Following the SCS trial,
dysfunction, manifested as hyper- or hypohidrosis (3), as the percutaneous leads are removed from the patient and, as
well as peripheral edema, which conveys a glossy, swollen such, are not considered a permanent implant. SCS technol­
appearance. ogy has been used effectively in the management of chronic
The signs and symptoms of CRPS result from dysfunc­ pain related to diabetic peripheral neuropathy (DPN), failed
tion of the peripheral and central components of the nervous back surgery syndrome (FBSS), CRPS, phantom limb pain,
system. Nociceptors are peripheral nerve fibers that transmit postamputation stump pain, and arachnoiditis; and, after
pain signals to the spinal cord. These fibers are capable of more than 30 years of experience, SCS has come to be a first
releasing inflammatory mediators, such as substance P and line intervention for cases of CRPS that have not satisfac­
calcitonin gene-related peptides, into the peripheral tissues. torily responded after 12 to 16 weeks of conservative ther­
The release of these mediators is believed to trigger neuro­ apy (2).
genic inflammation through capillary leakage and activation Foot and ankle surgeons faced with patients who fail to
of inflammatory cells in peripheral tissues (8). Abnormal progress as anticipated after surgical intervention, and who
interactions subsequently develop between the sensory and display persistent pain, pain out of proportion to a stimulus,
mechanical nociceptors that are responsible for normal sen­ burning sensation, edema, and limited range of motion,
sations such as touch and vibration. Allodynia results when should be alerted to the possibility that the patient may be
abnormal connections are established between the axons of developing CRPS. If CRPS is suspected, then consideration
nociceptors and mechanosensory fibers, resulting in noci­ should be given to the potential benefits of a timely referral
ceptor excitation from tactile fiber stimulation (8). These to an interventional pain medicine physician for further
abnormal interactions result in the interpretation of nonpain­ evaluation and potential management. The treatment of
ful stimuli as painful, with amplified pain perception fol­ CRPS is considered by many clinicians to be most effective
lowing an injury; such interactions can evolve following when it is multifaceted and undertaken as early as possible.
even relatively minor trauma. ' As demonstrated in the patient described in this case report,
Various opioids and adjuvant medications have been SCS can provide a safe and minimally invasive modality for
successfully used to treat CRPS. Adjuvant medications in- the treatment of CRPS.

54 THE JOURNAL OF FOOT & ANKLE SURGERY

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The authors express appreClaUon to Michael Smith.
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DPM. and Ani C. Khodavirdi. PhD. for their independent review of the adult and paediatric literature. Pain 49:337-347, 1992.
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