Complex regional pain syndrome

Classification according to ICD-10
G90.5-	complex regional pain syndrome, type I (since 2019) (until 2018: M89.0)
G90.6-	complex regional pain syndrome, type II (since 2019) (until 2018: G56.4 for CRPS II of the upper extremity, G57.8 for CRPS II of the lower extremity)
ICD-10 online (WHO version 2019)

The complex regional pain syndrome ( Complex regional pain syndrome , CRPS ) is a neurological-orthopedic-trauma disorders. The internationally standardized term is increasingly replacing the previously common and synonymously used terms reflex dystrophy , Sudeck 's disease , Sudeck dystrophy , algodystrophy and reflex sympathetic dystrophy .

The disease is characterized by the fact that after external influences (e.g. trauma , operations and inflammation ), dystrophy and atrophy of limb sections occur over a longer period of time . When symptoms occur swelling , pain , circulatory disorders , hypersensitivity to touch and skin lesions on the further course also functional limitations in the form of weakness and impaired mobility. The disease occurs more often on the upper limbs than the lower ones in adults; especially often after distal radius fractures . Women are more often affected.

If the origin mechanism has not yet been adequately clarified, a neuronal inflammatory reaction , both peripheral and central , in combination with a cortical reorganization is discussed as the cause. The therapy requires the collaboration of several specialist disciplines.

For a long time, CRPS was mainly treated with painkillers and nerve blocks. In 2013, an extensive data analysis by the Cochrane Collaboration revealed insufficient effectiveness of the frequently used sympathetic blockade . Therapy recommendations have been changed considerably since then. The warm CRPS, which is associated with classic inflammatory symptoms , is mainly treated with anti-inflammatory effects using cortisone shock therapy. Cold CRPS, which is more characterized by sensitivity disorders, is preferably treated with antineuropathic drugs such as gabapentin or pregabalin . In addition, in all cases of CRPS, intensive physiotherapy and occupational therapy should be used at an early stage in order to reduce swelling, compensate for pathological movement patterns and poor posture, reduce painful movement patterns and restore normal sensitivity.

The prognosis depends to a large extent on the time at which treatment is started. Complete healing is usually only possible if treatment is started early. Frequently, however, the symptoms are only assigned to the correct clinical picture several months after the onset of the disease, in a late stage, which in the vast majority of cases leads to lifelong impairments through pain and functional impairments.

Pathogenesis

The pathogenesis of CRPS is not yet fully understood. A disturbed healing process of the affected tissue is suspected. It is noteworthy that the occurrence of CRPS and its severity do not depend on the severity of the initial violation. The following development model is currently being discussed: An inflammatory reaction occurs in which inflammatory mediators ( substance P , CGrP) are released that are no longer broken down in sufficient quantities and thus prolong the neurogenic inflammatory reaction. This distribution of said inflammation-mediating substances (mediators) is carried out also in the central nervous system (CNS) , thus causing a sensitization of the central nervous pain processing cell groups ( neuron comes). The changes in blood circulation and the tendency of the skin to perspire are caused by a central malfunction of the sympathetic nervous system , although the exact mechanism is still in the dark. The narrowing of the vessels ( vasoconstriction ) and the formation of arterio-venous shunts lead to an insufficient supply of oxygen to the tissue (hypoxia), which leads to increased acidic degradation products ( acidosis ), which contribute to an intensification of pain.

As with phantom pain , cortical reorganization occurs in CRPS. This is a change in the individual representation areas (e.g. the hand) in the cerebral cortex , which can also explain the expansion of pain beyond a certain nerve supply area. There is evidence of a genetic disposition .

The role of psychological factors in the development of CRPS has long been controversial, the existence of a so-called "CRPS personality" is now largely rejected. However, several studies have shown an accumulation of stressful life events before the onset of the disease and an increased rate of post-traumatic stress disorders .

CRPS type I after distal radius fracture

The distal radius fracture is the most common trauma that precedes CRPS. With an incidence of 7% to 37%, it is a common complication of this fracture. However, the frequency has been decreasing continuously over the past 40 years. The equally increasing proportion of surgically stabilized radius fractures suggests that this can partially prevent CRPS. It is certain that some factors favor the development of CRPS. Above all, the following should be mentioned: Unstable retention, secondary displacement, multiple attempts at repositioning and constricting plaster casts that restrict the mobility of the fingers.

It most commonly affects postmenopausal women . The information on this varies between about 70% and 90%. In addition, this group of patients is also the group most likely to suffer radius fractures.

Subdivision

• CRPS type I (M. Sudeck): trauma without nerve damage
• CRPS type II (causalgia): trauma with nerve damage

Staging

Acute inflammation in CRPS

Inflammation in CRPS of the hands and wrists

The disease can become chronic if left untreated. A classification can be made according to the time course as well as according to symptoms.

Temporal course

• Acute inflammatory swelling (0-3 months) with local signs of inflammation (swelling, redness, warmth, pain, dysfunction)
• Dystrophy (three to six months) with stiff joints
• Atrophy (six to twelve months) = end stage (no longer functioning)

This classification was introduced by Paul Sudeck at the beginning of the 20th century. However, it is now very controversial, as many cases show a different course of the disease. In current CRPS research, staging hardly plays a role anymore.

Symptoms

The symptoms are initially unspecific so that the diagnosis can be delayed. The course of the disease also varies greatly from person to person, with all gradations being found from spontaneous total remission to progression with not inconsiderable impairment of quality of life.

Motor disorders

In more than three out of four patients there is a weakness of the affected extremity, which is caused by pain and edema in the acute stage and by contractures and fibrosis in the chronic stage . Tremor occurs in half of the patients and myoclonus occurs in one in three .

Sensory disorders

In many patients there is what is known as hyperalgesia , i.e. a greatly increased sensitivity to pain, usually to mechanical stimuli or allodynia, i. H. Pain when the sensation is actually not painful (e.g. wiping the skin with a cotton ball). Approx. Three out of four patients experience pain at rest, which varies greatly from person to person (or burning or tingling). In rarer cases, numbness or a feeling of alienation in the affected extremity occurs.

Autonomous disorders

In the early stages, there is almost always reddening, swelling (edema) and heating of the skin (signs of inflammation), which changes to a blue color and a feeling of cold if it persists for a long time (chronification). In half of the cases there is an increased tendency to sweat ( hyperhidrosis ).

Changes in the x-ray

Irregular decalcification of some bones in the right hand and forearm bones

During the transition to the chronic stage a few months after the onset of the disease, the X-ray image usually shows blotchy lightening , which is due to a reduction in the calcium salt content in the bone, which makes it more permeable to X-rays. The decalcification of the bones increases with progressive chronification until the picture of a high-grade inactivity osteoporosis is present.

Trophic disorders

In many cases there is increased nail and hair growth in the acute stage, in the chronic stage it changes into the opposite. In severe cases, the muscles can break down (atrophy) with the formation of contractures and consequent restricted mobility.

Diagnosis

The diagnosis of CRPS is primarily based on clinical criteria. Apparatus methods (e.g. x-ray, scintigraphy) can provide additional information, but neither prove nor exclude CRPS.

Clinical diagnosis

The clinical diagnosis is made today according to the revised criteria of the IASP (International Association for the Study of Pain), which were drawn up in Budapest in 2003 and therefore also known as the “Budapest Criteria” or “Harden-Bruehl Criteria” (according to the authors of the publication ) are designated. To this end, the symptoms of CRPS are divided into four categories. In the anamnestic (in the medical history) there must be one symptom from three of the four categories; the examination must show symptoms from two categories.

Modified Budapest criteria
A.	Persistent pain that cannot be explained by the initial trauma.
B.	at least one symptom from 3 of the 4 following categories in both the anamnesis and at the time of the examination:
		1.	Hyperalgesia (hypersensitivity to pain stimuli) or	Hyperesthesia (hypersensitivity to touch) or	Allodynia
		2.	Skin temperature asymmetry or	Change of color
		3.	Asymmetry in sweating or	edema
		4th	reduced mobility, or dystonia	Tremor , " paresis ", or weakness	Changes in hair or nail growth.
C.	Another disease does not adequately explain the symptoms.

For the clinical diagnosis of CRPS, all points A, B and C must be met.

laboratory

Routine laboratory values ​​show no abnormalities with CRPS. Laboratory tests are only indicated to rule out other diseases (e.g. infection).

X-ray image

In the X-ray image , spot-shaped decalcifications near the joints are traditionally shown. However, these appear no earlier than two months after the onset of the disease and can also be completely absent. In later stages, the changes cannot be clearly distinguished from other bone atrophies. Therefore, the X-ray image only plays a subordinate role in diagnostics.

Magnetic resonance imaging

In MRI soft tissue edema, skin thickening, joint effusion, and later fibrotic changes may be visible. However, the sensitivity is low; H. many CRPS cases are not recognized. The importance of the magnetic resonance tomogram therefore lies mainly in the differential diagnosis (excluding other diseases).

Skeletal scintigraphy

The value of skeletal scintigraphy lies in the fact that characteristic changes (band-shaped excess storage near the joint) are visible relatively early on (maximum sensitivity after about six weeks). Sensitivity and specificity are better than with the aforementioned methods, but are also not sufficient for a reliable diagnosis. As the disease progresses (after more than 12 weeks), the diagnostic value of scintigraphy decreases considerably.

therapy

Treatment is often lengthy and can be frustrating for both patients and therapists. The prognosis is best when the disease is recognized and treated early. Chronic severe courses are generally rare with less than 2% of cases.

• Physiotherapy : Physiotherapy exercise treatment is undisputed in prophylaxis after recent injuries, but only physiotherapy adapted to the stage helps in therapy. Exercise on the affected extremity should be avoided in the acute area affected by edema, redness, and pain. At this stage, treatment is given by immobilization and elevation, if necessary on a positioning rail. Lymph drainage , local cooling and possibly “descending baths” are used to treat the edema . At the end of this stage, contralateral active exercise treatment can also be started. The physiotherapeutic activation of the affected joints only takes place when the pain has subsided. Medical monitoring of the effect and, if necessary, adjustment of the prescription are necessary.
• The occupational therapy is the restoration of everyday function, initially starting with the reduction of painful movement patterns. In order to achieve the most normal possible sensitivity, the affected areas of the skin are actively desensitized with the aim of getting used to everyday contact. Fine motor skills are initially trained without resistance and later with resistance, and finally, if necessary, the posture of the joints can be gradually corrected.
• Bisphosphonates such as pamidronate, neridronate, and alendronate are commonly used. The effect is well proven in CRPS after fractures. Several studies show a good effect within 4 to 10 days and a significant improvement within weeks or months. Four 100 mg neridronate infusions over 4 to 10 days (400 mg total dose) are now standard treatment in university hospitals in northern Italy. It should be noted, however, that bisphosphonates are not approved for this application. The frequently discussed risk of bone damage (e.g. to the jaw) is only of importance in high-dose therapy.
• Corticoids in tablet form (e.g. prednisolone ) are mainly used in the early phase. The status of studies on this is not entirely clear, but very often there is rapid improvement in pain, swelling and movement.
• Pain management: tricyclic antidepressants , non-opioid analgesics ( NSAIDs , e.g. ibuprofen , diclofenac, or metamizole ) or opioids (such as morphine ).
• Gabapentin is used for neurological symptoms, but there are hardly any studies on this.
• In two placebo-controlled studies, ketamine showed a positive effect, so its use is recommended in the current version of the DGN guidelines (2012) if other therapies are not sufficiently effective. However, the therapy is relatively complex and has many side effects, liver damage has been observed. Ketamine is not approved for the CRPS.
• local application of steroid- containing ointment or DMSO ( dimethyl sulfoxide as a radical scavenger).
• In certain cases (Harden / Swan) a blockade of the sympathetic stellate ganglion is indicated. If possible, it should take place within the first six months of the duration of the illness, since only then can recovery rates of up to 70% be achieved.
• The early use of calcitonin (nasal spray) can possibly have a positive effect on the course. However, the study results on this are contradictory. Calcitonin is no longer available as a nasal spray in Germany and has to be injected.
• Rheologics like Haes are sometimes used, but there is no scientific basis for doing so.
• Spinal cord stimulation can be used to treat chronic pain .

The therapy definitely belongs in the hands of specialists for orthopedics and trauma surgery, physical and rehabilitative medicine, neurology and anesthesiology, who are experienced in pain therapy , in an interdisciplinary exchange.

forecast

The course of the disease of CRPS is very different and ranges from complete healing to severe chronic course with permanent disability and pain.

There is broad consensus that starting therapy as early as possible improves the prognosis; however, there is no reliable data on this.

In a long-term study of over 100 patients, almost 85% of the patients improved to such an extent that the diagnostic criteria for CRPS were no longer met. However, pain of varying degrees was still present in 41% of the cases. The median time to "healing" was 11 months, but improvements still occurred after three years. However, these are only data from a single center; there are no major studies.

prevention

It has been known for a long time that CRPS occurs less frequently after operations under regional anesthesia than after operations under general anesthesia. An observational study confirms this, but randomized studies do not exist. However, the use of regional anesthesia is usually recommended.

However, the preventive effect of vitamin C in operations on the hand or foot has been proven by several studies (500–1,000 mg daily for 50 days from the day of the operation). A summarizing analysis showed a reduction in the CRPS risk of around three quarters. Vitamin C therapy is therefore an effective preventive measure.

See also

• Volkmann's contracture
• Hand surgery

literature

• Guideline "Diagnostics and Therapy of Complex Regional Pain Syndromes (CRPS)" of the DGN
• RN Harden, M. Swan, et al. a .: Treatment of Complex Regional Pain Syndrome: Functional Restoration. In: Clinical Journal of Pain. 2006 Jun, 22 (5), pp. 420-424.
• DV Nelson, BR Stacey: Interventional Therapies in the Management of Complex Regional Pain Syndrome. In: Clinical Journal of Pain. 2006 Jun, 22 (5), pp. 438-442.
• J. Klingelhöfer, M. Rentrop: Clinical Guide "Neurology, Psychiatry". P. 252.
• In-house training in the Department of Neurology . Landgrebe, BB R. Hospital, April 2005.
• FU Niethard, J. Pfeil: Orthopedics. 4th edition. 2003, ISBN 3-13-130814-1 .
• Thomas von Rothkirch, Walter Blauth, Bodo Helbig: The Sudeck syndrome of the hand. Overview, treatment concept and results . Hand surgery, microsurgery and plastic surgery 21 (1989), pp. 115-126.
• G. Wasner, J. Schattschneider et al. a .: The complex regional pain syndrome. In: The anesthesiologist. 2003 Oct, 0 (10), pp. 883-895.
• M. Rizzo, SS Balderson, DH Harpole, LS Levin: Thoracoscopic sympathectomy in the management of vasomotor disturbances and complex regional pain syndrome of the hand. In: Orthopedics. January 2004, 27 (1), pp. 49-52.
• Healing on the verge of death. In: Image of Science . 8/2006.

Web links

Wiktionary: CRPS  - explanations of meanings, word origins, synonyms, translations

Individual evidence

1. ↑ Sudeck's disease - Functional therapy is essential , accessed on July 12, 2018.
2. ↑ E. Scola: Diagnosis and therapy of algodystrophy. In: Trauma and Occupational Disease. 2006, 8, Suppl. 2; pp. 225-228, doi: 10.1007 / s10039-005-1088-5 .
3. ↑ JH Geertzen u. a .: Stressful life events and psychological dysfunction in Complex Regional Pain Syndrome type I. In: Clin J Pain. 1998 Jun, 14 (2), pp. 143-147.
4. ↑ H. Brehmer u. a .: CRPS in children: What role do psychological factors and critical life events play? In: pain. 26, 2012, Suppl. 1 (Poster at the German Pain Congress 2012)
5. ^ V. Speck u. a .: Increased prevalence of post-traumatic stress disorder in patients with CRPS. In: pain. 26, 2012, Suppl. 1 (Poster at the German Pain Congress 2012)
6. ↑ RM Atkins, T. Duckworth, JA Kanis: Algodystrophy following Colles' fracture. In: J Hand Surg. (Br) 1989; 14, pp. 161-164.
7. ↑ E. Peltz, F. Seifert, C. Maihöfner: Guide to the diagnosis of the complex regional pain syndrome. In: Akt Rheumatol. 36, 2011, pp. 28-34.
8. ↑ Andreas Böger: Sudeck's disease. Diagnosis possible without apparatus. In: Deutsches Ärzteblatt . tape 116 , no. 1 , February 1, 2019, p. A204-A205 .
9. ↑ Massimo Varenna, F. Zucchi, D. Ghiringhelli, L. Binelli, M. Beviacqua, P. Bettica, L. Sinigaglia: Intravenous clodronate in the treatment of reflex sympathetic dystrophy syndrome. A randomized, double blind, placebo controlled study . In: J Rheumatol . tape 27 , no. 6 , June 2000, p. 1477-1483 , PMID 10852274 .
10. ↑ Massimo Varenna, S. Adami, M. Rossini, D. Gatti, L. Idolazzi, F. Zucchi, N. Malavolta, L. Sinigaglia: Treatment of complex regional pain syndrome type I with neridronate: a randomized, double-blind, placebo-controlled study . In: Rheumatology . tape 52 , no. 3 , March 2013, p. 534-542 , doi : 10.1093 / rheumatology / kes312 , PMID 23204550 .
11. ↑ WWA Zuurmond u. a .: Treatment of acute reflex sympathetic dystrophy with DMSO 50% in a fatty cream. In: Acta Anesthesiologica Scandinavica. 40/1996, pp. 364-367, doi: 10.1111 / j.1399-6576.1996.tb04446.x .
12. ↑ A. Rácz, L. Göderer, p Dworsky, F. Birklein, V. Bacon, C. Maihöfner: long-term prognosis of the CPRS - a follow-up study. In: pain. 26, 2012, Suppl. 1 (Poster at the German Pain Congress 2012)
13. ↑ SS Reuben, R. Pristas, D. Dixon, S. Faruqi, L. Madabhushi, S. Wenner: The incidence of Complex Regional Pain Syndrome after fasciectomy for Dupuytren's contracture: a prospective observational study of four anesthetic techniques. In: Anesthesia and Analgesia. 2006 Feb, 102 (2), pp. 499-503.
14. ↑ N. Shibuya, JM Humphers, MR Agarwal, DC Jupiter: Efficacy and safety of high-dose vitamin C on complex regional pain syndrome in extremity trauma and surgery - systematic review and meta-analysis. In: J Foot Ankle Surg. 2013 Jan-Feb, 52 (1), pp. 62-66.

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