Effect of spinal cord stimulation in Type I complex ...

[Pages:5]J Neurosurg 110:274?278, 2009

Effect of spinal cord stimulation in Type I complex regional pain syndrome with 2 rare severe cutaneous manifestations

Case report

Kim Rijkers, M.D.,1 Jasper van Aalst, M.D.,1 Erkan Kurt, M.D.,2 Marc A. Daemen, M.D., Ph.D.,1 Emile A. M. Beuls, M.D.,1 and Geert H. Spincemaille, M.D., Ph.D.1

1Department of Neurosurgery, Maastricht University Hospital, Maastricht; and 2Department of Neurosurgery, Alkmaar Medical Centre, Alkmaar, The Netherlands

The authors present the case of a 49-year-old female patient with complex regional pain syndrome?Type I (CRPSI) who was suffering from nonhealing wounds and giant bullae, which dramatically improved after spinal cord stimulation (SCS). The scientific literature concerning severe cutaneous manifestations of CRPS-I and their treatment is reviewed. Nonhealing wounds and bullae are rare manifestations of CRPS-I that are extremely difficult to treat. Immediate improvement of both wounds and bullae after SCS, such as in this case, has not been reported previously in literature. Considering the rapidly progressive nature of these severe skin manifestations, immediate treatment, possibly with SCS, is mandatory. (DOI: 10.3171/2008.4.17506)

Key Words ? complex regional pain syndrome ? severe skin manifestation ? spinal cord stimulation

C omplex regional pain syndrome?Types I and II are neuropathic pain syndromes that typically occur after trauma and were formerly known as reflex sympathetic dystrophy. Complex regional pain syndrome? Type II develops after trauma involving major nerve damage, whereas CRPS-I develops after trauma not involving major nerve damage. The clinical presentation of CRPS-I varies, but the syndrome is diagnosed primarily by noting symptoms such as regional burning pain, hyperalgesia, edema, changes in skin color and temperature, hyperhidrosis, dystrophic/atrophic skin and nail changes, and movement disorders.17 These different symptoms are caused by the multifactorial pathophysiology in CRPS-I,23 in which peripheral somatosensory abnormalities,12 vasomotor disturbances,20 abnormal adrenergic innervation,13 and disturbances in peripheral and supraspinal regulatory mechanisms play a role.5

The initial diagnosis of CRPS-I was made based on the 4 criteria defined by the International Association for the Study of Pain (Table 1).11 The clinical course of CPRS is then divided into 3 stages: I) "warm," acute, or hyperemic stage characterized by symptoms of regional inflammation; II) "intermediate" stage within 2 years following

Abbreviations used in this paper: CRPS = complex regional pain syndrome; SCS = spinal cord stimulation.

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the original trauma, characterized by normalization of temperature and the beginning of dystrophic or ischemic changes; and III) "cold," chronic, or atrophic stage after 4 years with irreversible changes in skin and bones and the spreading of pain throughout the entire limb in a more neuropathic way. Blood flow and tissue-blood distribution are diminished, resulting in a decreased skin surface temperature. This division into 3 stages is mainly based on clinical observations.22

A recognized therapy for CRPS-I consists of a combination of pharmacotherapy, nerve blocks, and psychotherapy (when appropriate). Additional therapy, such as SCS, is proposed in patients who continue to experience symptoms that prevent function-restoring therapy.3 In this report we describe a patient suffering from Stage III CRPS-I who was treated with SCS. After the patient temporarily discontinued SCS, she developed nonhealing surgical (on a surgical scar) skin lesions on the abdomen and thorax, and giant bullae on the left lower limb. Both skin manifestations resolved immediately after adequate stimulation was restored. Although skin symptoms in CRPS-I are common, severe and unusual cutaneous manifestations are rare; we found only 6 articles on this subject in the scientific literature.14,15,18,19,21,22 The literature on severe cutaneous manifestations of CRPS-I and the treatment of these lesions is reviewed.

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Spinal cord stimulation in Type I complex regional pain syndrome

TABLE 1: Diagnostic criteria for CRPS*

Criteria No.

Criteria

1

Preceding noxious event w/out (CRPS-I) or w/ obvious

nerve lesion (CRPS-II)

2

Spontaneous pain or hyperalgesia/hyperesthesia not

limited to a single nerve territory and disproportionate

to the inciting event

3

Edema, skin blood flow (temperature) or sudomotor ab-

normalities, motor symptoms or trophic changes are

present on the affected limb, in particular distally

4

Exclusion of all other conditions that would explain the

symptoms

* From Merskey H, Bogduk N: Classification of Chronic Pain: Descriptions of Chronic Pain Syndromes and Definition of Pain Terms, ed 2. Seattle: IASP Press, 1994. Criteria 2?4 must be satisfied for a diagnosis of CRPS.

Case Report

History and Presentation. This 49-year-old woman had suffered from severe Stage III CRPS-I for almost 40 years. Her symptoms consisted of pain, edema, and dystonia in all 4 limbs, resulting in wheelchair dependence. She also suffered from skin problems consisting of bullae and nonhealing wounds.

After several years of unsuccessful conservative treatment for her CRPS-I, SCS (Itrel II, Medtronic) was started in 1991. Spinal cord stimulation was performed with an epidurally placed electrode at the C4?5 level, which resulted in complete pain relief but only partial improvement of her dystonia. In November 2003 she developed a spontaneous lesion of the skin covering the SCS extension cable in the right scapular area. After 4 months of unsuccessful conservative treatment with antibiotics, several surgical explorations were performed to treat the nonhealing wound. In September 2004, using patch testing, a dermatologist excluded the possibility of a contact allergy caused by any of the 11 materials used (as well as those in the European Standard Series patch tests), or by the organic dye, plastics, and glue used in the SCS system. In February 2006, when the extension cable spontaneously extruded from the wound in the right scapular area, the plastic surgeon successfully performed a Z-plasty. A few weeks later, an old surgical scar over the pulse generator in the left hemiabdomen began to discharge fluid. After a few weeks of conservative antibiotic treatment by her general practitioner with a working diagnosis of low-grade infection, her situation had worsened and she was admitted to our department again when the pulse generator was visible through a skin defect.

Operation and Postoperative Course. In a final attempt to treat this skin problem, in May 2006 the complete SCS system was removed and successfully replaced by a new one (Synergy, Medtronic) with the electrode located at the cervical level and the pulse generator implanted in the right hemiabdomen. The stimulations induced by this system were inadequate, and only reached the patient's

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Fig. 1. Photograph depicting the right leg of our patient after discontinuation of SCS. The photograph was taken 2 weeks after the first surgery. The knee is visible in the upper-right corner of the photograph. Giant bullae on the right leg developed as a result of inadequate stimulation.

arms and shoulders, but not her legs. This new system immediately resulted in complaints of severe neuropathic pain in both legs. Within 3 days after discontinuation of stimulation, giant bullae developed on the patient's right lower leg, progressively increasing in size (Fig. 1).

Subsequently it was decided to implant additional thoracic electrodes to obtain adequate stimulation in the legs in an attempt to halt progression of the bullae and treat the pain. Adding these electrodes restored successful stimulation in both legs as well as in the abdominal area. Within 1 day after receiving additional stimulation the pain resolved. Moreover, the bullae on the patient's right leg resolved within days (Fig. 2) and even the 2-monthold nonhealing skin lesion on the left side of the abdomen resolved. One year later, SCS continued to provide adequate pain relief to the patient in both her arms and legs, and no new skin lesions or bullae have developed.

Discussion The unusual cutaneous lesions described in this re-

Fig. 2. Photograph depicting the right leg of our patient, taken 5 days after additional adequate stimulation in the lower limbs.

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K. Rijkers et al.

TABLE 2: Summary of patients with CRPS-I suffering from bullae

Case CRPS-I Authors & Year No. Stage Sex

Sundaram & Webster, 1 unknown unknown

2001

2 unknown unknown

3 unknown unknown

Webster et al., 1993 4 III

F

5 III

F

Wagner & Hathaway, 6 I

F

1995

present study

7 III

F

Age (yrs) unknown unknown unknown

40 42 33

49

Culture Light Microscope unknown unknown

Biopsy Results Immunofluorescence unknown

Electron Microscope unknown

unknown unknown unknown unknown negative

negative

unknown unknown epidermal necrosis, dermal fibrosis stasis dermatitis

superficial & deep perivascular in filtrate not performed

unknown unknown negative fibrin in dermal vessels negative

not performed

unknown unknown unknown

basement membrane disruption unknown

not performed

port developed in the context of CRPS-I. Severe unusual cutaneous manifestations of CRPS-I have been described in the scientific literature.14,15,18,19,21,22 To the best of our knowledge, only 44 patients with these unusual manifestations have been described in the literature thus far, of whom 6 suffered from bullae (Table 2),15,19,21 and 1 patient from nonhealing wounds.15 Small bullae in posttraumatic pain-affected limbs have been reported in 2 additional patients.1,2 However, these patients both suffered from major nerve damage and would be considered to be affected by CRPS-II at present.

In 1993, Webster and colleagues21 were the first to report on recurrent bullous skin lesions in 2 patients with CRPS-I from a group of 9 with severe cutaneous manifestations. These patients, similar to our patient, suffered from longstanding CRPS-I. Wagner and Hathaway19 have described 1 patient whose primary presentation of CRPS-I consisted of burning pain associated with erythema, edema, hyperpigmentation, and bullous lesions on her left arm.

Sundaram and Webster15 summarized the severe skin lesions of 26 patients with CRPS-I and found poor wound healing in 1 patient and bullae in 3. All patients suffered from longstanding CRPS-I. It is not clear whether the 3 patients mentioned in the paper by Sundaram and Webster include the 2 patients described earlier by Webster et al.21 If this is the case, then only 4, not 6, patients with CRPS-I and bullae have been reported.

In all of the patients described, viral, bacterial, and fungal cultures were negative. Our patient underwent repetitive cultures of the nonhealing wounds. We decided not to perform a biopsy procedure of the bullae in our patient so as not to compromise the integrity of the bullae because of their giant size and risk of infection. More importantly, a biopsy procedure itself can induce CRPS-I and is therefore not indicated in this group of patients. Histological examination of the skin lesions in 1 of Webster et al.'s patients (Case 5; Table 2) with recurrent bullae revealed chronic stasis dermatitis and numerous ultrastructural abnormalities, consisting of areas in which the basement membrane contained no identifiable anchoring

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fibrils, and segments of basement membrane that showed decreased electron density and focal disruption.21 In the patient reported by Wagner and Hathaway19 (Case 6; Table 2) a biopsy specimen of the lesions revealed a superficial and deep perivascular mononuclear infiltrate. Antinuclear antibodies and immunofluorescent studies were negative.

There is no information available on the contents of the bullae in the 6 reported cases. We decided not to puncture the bullae in our patient for the same reasons we did not perform a biopsy procedure. Though not comparable with spontaneous bullae, Huygen and colleagues6 and Heijmans-Antonissen et al.4 studied blister content in patients with CRPS-I using the "suction blister technique" to make artificial blisters. A significant increase in proinflammatory cytokines was found in artificial blisters on the involved extremity compared with the uninvolved extremity.4,6

Hence, both structural changes in skin architecture as well as inflammatory changes appear to play a role in the development of severe cutaneous manifestations in patients with CRPS-I. However, the literature on patients with CRPS-I who are suffering from bullae is scarce and no definite conclusions can be drawn from these reports. It is even possible that bullae in CRPS-I are not a manifestation of the syndrome but rather are a dermatological symptom provoked by CRPS-I.

Treatment Options for Severe Cutaneous Manifestations

As stated before by others,15,19,21,22 the most favorable therapy for the severe skin problems in CRPS-I is to control the CRPS-I. A recognized therapy for CRPS-I consists of a combination of pharmacotherapy, nerve blocks. and (when appropriate) psychotherapy. Additional therapy is proposed in patients who continue to experience symptoms that prevent function-restoring therapy.3 Based on our observations and on the literature, severe cutaneous manifestations are a further indication for additional therapy. Considering the progressive nature of the bullae in our patients, these severe cutaneous manifestations should be treated as an emergency.

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Spinal cord stimulation in Type I complex regional pain syndrome

The treatment of severe cutaneous manifestations of CRPS-I is very difficult due to the inability of the patient to comply with topical treatment because of hyperalgesia in the involved area. There was no effect of the use of topical steroids in combination with oral prednisone (up to 40 mg/day) in Webster et al.'s patients.21 According to Webster and associates, therapy for bullous CRPS-I lesions has been largely ineffective.21

Successful treatment of the bullae in the patient described by Wagner and Hathaway19 consisted of a complete continuous brachial plexus block for 7 days. A relapse of CRPS-I, with both pain and bullae, was successfully treated with a T-3 costotransversectomy and sympathetic ganglionectomy. The patient was the only one of the 6 patients with CRPS-I suffering from bullae who was treated successfully.19

Spinal Cord Stimulation

Spinal cord stimulation is another (reversible) option in patients with CRPS who continue to experience symptoms that prevent function-restoring therapy.16 In the most recent systematic review on SCS for CRPS,16 a high success rate of SCS in CRPS-I is described, from both the 1 randomized controlled trial7,8 and from the 25 case series published. On average, 67% of patients undergoing SCS attain pain relief of at least 50%.16

Spinal cord stimulation is a neuromodulation therapy that has been available since the 1970s and is based on the gate theory of Melzack and Wall.10 According to this theory, pain transmission is inhibited at the level of the dorsal horns by electrical stimulation of the large afferent fibers. More recently it was found that SCS leads to release of -aminobutyric acid inside the dorsal horns. Gamma-aminobutyric acid is the neurotransmitter of the inhibitory pathway coursing in the dorsal horns. Activation of this pathway reduces pain.16 In addition, SCS may suppress sympathetic activity,9 which means that SCS can relieve both pain and symptoms related to sympathetic activity in CRPS. With the more common but less severe cutaneous manifestations of CRPS-I, such as skin color changes, it has been shown that they can rapidly resolve after SCS or sympathectomy.19

Conclusions

Severe cutaneous manifestations in CRPS-I are rare. Of all patients described with these manifestations, only 6 patients suffered from bullae, and only 1 patient from nonhealing wounds. Of these 6 patients, only 1 patient was treated successfully, using sympathetic ganglionectomy. To our knowledge, SCS has not been described as an adequate treatment for nonhealing wounds and bullae as manifestations of CRPS-I. Moreover, the extreme reaction after discontinuing SCS has not been described in the literature before. However, the immediate and impressive response to adequate stimulation suggests a major role for SCS in the treatment of these and perhaps other severe skin manifestations of CRPS-I.

We prefer to use SCS compared with sympathectomy. Class II level of evidence is available for SCS as a treatment for CRPS-I, whereas for sympathectomy this level

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of evidence is not available. Moreover, SCS is safe and reversible. Considering the rapidly progressive nature of the bullae in our patient, these cutaneous manifestations should be treated as an emergency. Even though SCS in CRPS-I is normally performed as an elective type of surgery, these kinds of rapidly progressive skin lesions demand urgent treatment, meaning SCS should be applied or restored immediately.

Disclaimer

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Acknowledgment

We thank Dr. Van Hilten for his valuable comments on the manuscript.

References

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2. Doupe J, CUllen CH, Chance GQ: Post-traumatic pain and the causalgic syndrome. J Neurol Neurosurg Psychiatry 7: 33?48, 1944

3. Harden RN: Complex regional pain syndrome. Br J Anaesth 87:99?106, 2001

4. Heijmans-Antonissen C, Wesseldijk F, Munnikes RJ, Huygen FJ, van der Meijden P, Hop WC, et al: Multiplex bead array assay for detection of 25 soluble cytokines in blister fluid of patients with complex regional pain syndrome type 1. Mediators Inflamm 2006:K1?K8, 2006

5. Hilten JJ: Factor IV: movement disorders and dystrophia-- pathophysiology and measurement, in Wilson PR, StantonHicks M, Hardon RN (eds): CRPS: Current Diagnosis and Therapy. Seattle: IASP Press, 2005, Vol 32, pp 119?138

6. Huygen FJ, De Bruijn AG, De Bruin MT, Groeneweg JG, Klein J, Zijistra FJ: Evidence for local inflammation in complex regional pain syndrome type 1. Mediators Inflamm 11: 47?51, 2002

7. Kemler MA, Barendse GA, van Kleef M, de Vet HC, Rijks CP, Furnee CA, et al: Spinal cord stimulation in patients with chronic reflex sympathetic dystrophy. N Engl J Med 343:618? 624, 2000

8. Kemler MA, Reulen JP, Barendse GA, van Kleef M, de Vet HC, van den Wildenberg FA: Impact of spinal cord stimulation on sensory characteristics in complex regional pain syndrome type I: a randomized trial. Anesthesiology 95:72?80, 2001

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10. Melzack R, Wall PD: Pain mechanisms: a new theory. Science 150:971?979, 1965

11. Merskey H, Bogduk N: Classification of Chronic Pain: Descriptions of Chronic Pain Syndromes and Definition of Pain Terms, ed 2. Seattle: IASP Press, 1994

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15. Sundaram S, Webster GF: Vascular diseases are the most common cutaneous manifestations of reflex sympathetic dystrophy. J Am Acad Dermatol 44:1050?1051, 2001

16. Taylor RS, Van Buyten JP, Buchser E: Spinal cord stimulation for complex regional pain syndrome: a systematic review of the clinical and cost-effectiveness literature and assessment of prognostic factors. Eur J Pain 10:91?101, 2006

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Manuscript submitted February 15, 2007. Accepted April 8, 2008. Please include this information when citing this paper: published online October 17, 2008; DOI: 10.3171/2008.4.17506. Address correspondence to: Kim Rijkers, M.D., Department of Neurosurgery, Maastricht University Hospital, P. Debyelaan 25, PO Box 5800, 6202 AZ Maastricht, The Netherlands. email: kimrijkers @.

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