Definition
Also known as algodystrophy, Sudeck´s disease or sympathetic reflex dystrophy, this disorder is a highly unpleasant, unpredictable and painful complication of injuries and trauma especially fractures. The cause, development and effective treatment of CRPS are largely unknown. It has not been observed in children. Putative causes range from disorders of vegetative innervation to endocrine and psychosomatic disorders. Triggers include fractures, operations, infections and nerve injuries.
The severity of the underlying injury bears no apparent relationship to the sever- ity of the symptoms of Sudeck’s disease, which can be triggered even by trivial trauma. Most frequently effected are joints of the hand (90%), followed by ankle and knee joints. Two types are recognised: Type 1 develops after a trauma, while Type 2 is triggered by a peripheral nerve injury.
Clinical Findings
These consist of a triad of sympathetic, motoric and sensory manifestations with five characteristic symptoms:
▶ Disproportionately strong pain
▶ Swelling and unusual warmth of the effected area
▶ Skin discoloration of the effected area
▶ Increased hair growth on the area involved
▶ Stiffness of the joints involved
Diagnosis
The results of the following investigations contribute to the diagnosis:
▶ Thermography (area of overheating)
▶ Bone scan (area of increased uptake)
▶ X-ray (patchy rarefaction of bone) (Fig. 14.1)
▶ MRI (edematous areas around the joints involved)
CHAPTER 14 Complex Regional Pain Syndrome (CRPS)
140 Chapter 14 Complex Regional Pain Syndrome (CRPS)
▶ Alleviation of pain by sympathetic blockade – designated as “sympathetically maintained pain” (SMP) confirms the diagnosis
Course of Disease
This can be divided into three main stages (though questioned by some experts):
▶ Stage of inflammation (up to 3 months): Typical symptoms include localised pain, blue discoloration and overheating of the skin, dough-like edema and functional limitations of the joint. MRI shows presence of bone marrow edema.
▶ Stage of dystrophy (3 to 6 months): The dermatologic symptoms regress, leaving a trophic disorder of the skin. There is an increase in restriction of joint move- ment and spotty areas of demineralisation are seen on X-rays.
▶ Stage of atrophy (6 to 12 months): This end stage is characterised by generalised atrophy of skin, muscles and bone. Stiffness of the joint is further increased as massive rarefaction of bone occurs.
Fig. 14.1 Massive but spotty decalcifica- tion of the hand in CRPS (Sudeck’s disease), stage III
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Treatment Strategies
A relationship of confidence and trust must be established between patient and doctor, to ease the fear, tension and anxiety which always accompany this chronic condition. First, it is essential to break the vicious circle of pain and dystrophy by rest and physiotherapy. Surgery is only indicated for stabilisation of a fracture or later for correction of a deformity. However, it should be noted that early surgical intervention carries the risk of aggravating the condition.
Immobilisation, analgesics, anti-inflammatory drugs and cold dressings to counteract overheating are useful measures in stage I. Blockade of the sympa- thetic nerve supply (stellate) and calcitonin therapy have also been successful at this stage. Physiotherapy and exercises are strongly recommended in stages II and III.
Bisphosphonates
Since 1988, 4 international trials performed with pamidronate showed alleviation of pain in most cases and cure in some. This constitutes genuine progress in the treat- ment of M. Sudeck. Clodronate and alendronate were equally effective. Similar results were also achieved in patients treated with one of the following amino- bisphosphonates given for 4 to 6 months:
▶ Ibandronate (Bondronat®) Infusion of 6 mg monthly, 4–6 times, the first infusion only 2 mg in 100 ml NaCl solution over 15 min
▶ Pamidronate (Aredia®) Infusion of 60 mg monthly, 4–6 times, the first infusion only 30 mg in 500 ml NaCl solution over 30–60 min
▶ Zoledronate (Aclasta®) 5 mg infusion over 15 min given once only.
The initial low doses of 2 mg ibandronate or 30 mg pamidronate were used to avoid the possible acute-phase reactions (previously described), which could be much more pronounced in patients with Sudeck’s disease than in other patients. Occasionally acute-phase reactions occurred after the second infusion, but were milder. Many patients – some already morphine-dependent – have been cured by this therapy.
In others, the pain was alleviated to such a degree that analgesics were no longer required. Since the bisphosphonates have not yet been authorised for treatment of Sudeck’s disease, the patient’s informed consent must be obtained and docu- mented.
Bisphosphonates
