Spontaneous recovery of long-term severe complex regional pain syndrome of the upper limb: A case report

Introduction

Complex regional pain syndrome (CRPS) is a “debilitating, painful condition associated with sensory, motor, autonomic, skin and bone abnormalities.” ¹ Signs and symptoms are categorised into sensory, vasomotor, sudomotor and motor changes. Often a patient will neglect the affected limb and develop severe limitations in function. Pain is usually the most predominant symptom and the psychological effects of chronic pain are evident. CRPS results from changes to the somatosensory, sympathetic and somatomotor systems. ² This leads to alterations in the way noxious, tactile and thermal sensations are interpreted and reacted upon. ² Although the cause of CRPS is unknown, the course is usually long term and 15% of sufferers will continue to have unrelenting pain and loss of function for over five years after onset with no known medical cure. ¹ It has been reported that patients who have the poorest outcomes have their upper limb affected. ³

The purpose of this article is to present an unusual case of spontaneous recovery of severe CRPS by reflecting on the diagnosis and describing the course of this patient’s treatment with clinical reasoning where appropriate. It is beyond the scope of this case report to give detailed pathophysiological theory regarding the cause and development of CRPS or critically evaluate the evidence for the treatment modalities that were used.

The case

This case report details the year-long progression of CRPS in a woman following a minor soft tissue injury which concluded with a sudden spontaneous resolution of symptoms.

The patient attended the Emergency Department (ED) on 3 June 2011 following a simple fall caused by slipping on wet decking in her garden the previous day. She sustained a closed injury to her right elbow whilst hitting a rabbit hutch during the fall. Radiographs revealed no bony injury; therefore, a soft tissue injury was diagnosed. The patient was provided with a collar and cuff and given an appointment in the ED clinic three days later. On attendance at the clinic, the patient complained of elbow pain, hand and wrist oedema, paraesthesia of all digits (including her thumb) and was subsequently referred to physiotherapy. The patient had a history of Myalgic Encephalitis (ME), asthma and depression. However, the only medication she required was a Ventolin inhaler. She was right handed and worked as a school midday supervisor. She lived with her partner and seven-year-old daughter. Her parents lived nearby and were very supportive. Her hobbies included her animals and sewing.

The patient attended for her initial physiotherapy assessment six days following the injury. Upon assessment, it was found that she had disproportionate pain in her hand, extensive oedema in her hand, forearm and elbow, parasthesia and allodynia of her hand and forearm. Her skin appeared tight and shiny, she had no active motion in her elbow, distal radio-ulna joint (DRUJ) and wrist and only minimal active motion in her digits. She was unable to tolerate functional use of the hand and was in a collar and cuff with her neck flexed, shoulder protracted, elbow at 90 degrees flexion, DRUJ in mid prone and wrist in ulnar deviation hanging out of the cuff. Treatment was commenced on the day of assessment and consisted of a full explanation of CRPS with reassurance regarding an improved prognosis with early diagnosis and commencement of treatment. A referral was made to her general practitioner (GP) for Amitriptyline as recommended in NICE guidelines for the management of neuropathic pain, ⁴ and a desensitising programme was initiated including looking and touching her hand and arm. She was also supplied with a supportive sling to wear when travelling but to leave off at home and an urgent referral was made to the local pain team.

Discussion

A search of the literature for complete recovery of CRPS revealed only one report which detailed the use of anaesthetic Ketamine and Midazolam to induce recovery. ¹⁰ However, this was a single case report which lacked objective outcome measures. No further literature reporting spontaneous recovery of CRPS was found.

Following this remarkable event, the clinicians have reflected upon their diagnosis and treatment of this patient. On the initial assessment in physiotherapy, six days following her injury, the diagnosis of CRPS that had been made was reviewed using the Budapest diagnostic criteria ¹ and supported the diagnosis (Table 1). The possible differential diagnoses ¹ (Table 2) were also considered, the only one of these that could be appropriate to this patient would be neuropathic pain due to peripheral nerve damage. The original injury was at the elbow near the route of the ulnar nerve; however, her symptoms did not correspond to ulnar nerve motor or sensory supply. The patient was reviewed and treated by a clinical specialist physiotherapist who first made the diagnosis of CRPS. This diagnosis was confirmed by the physiotherapy team, GP, pain consultant, pain team and hand surgeon.

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Table 1.

Comparison of patients symptoms with Budapest diagnostic criteria. ¹

All of the following statements must be met	Met by patient
The patient has continuing pain which is disproportionate to any inciting event	Yes
The patient has at least one sign in two or more of the categories below	Yes
The patient reports at least one symptom in three or more of the categories below	Yes
No other diagnosis can better explain the signs and symptoms	Yes
Category/sign and symptoms
1. Sensory – allodynia and/or hyperalgesia	Yes
2. Vasomotor – temperature asymmetry and/or skin colour changes and/or skin colour asymmetry	Yes
3. Sudomotor/oedema – oedema and/or sweating changes and/or sweating asymmetry	Yes
4. Motor/trophic – decreased range of motion and/or motor dysfunction and/or trophic changes	Yes

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Table 2.

Differential diagnosis for CRPS. ¹

Common conditions	Rare conditions
Bony or soft tissue injury	Gardner-Diamond syndrome
Neuropathic pain	Erythromelalgia
Arthritis/arthrodesis	Self-harm
Infection
Compartment syndrome
Arterial insufficiency
Raynaud’s
Lymphatic/venous obstruction
Thoracic outlet syndrome

Prompt diagnosis, early referral to physiotherapy, management by a specialist pain management programme and an interdisciplinary treatment approach including the four pillars of intervention which are patient information and education, pain relief, physical and vocational rehabilitation and psychological intervention are recommended. ¹ On review of the management of this case, it is clear that all these recommendations were met.

It is interesting that despite a year long history of reduced motion and disuse of the hand and arm associated with chronic oedema, there was no evidence of joint stiffness or soft tissue tightness at each administration of the nerve block. It would be expected that as full passive stretches of the joints were only permitted during the nerve blocks that the tissues would have contracted between these treatments.

The patient had numerous psychological risk factors that predicted chronicity ¹ such as her pre-existing ME, history of depression/anxiety, previous negative experiences with health professionals, illness behaviour, poor coping strategies, lack of willingness to set goals and being passive in treatment sessions. It has been demonstrated that the risk of CRPS is significantly increased in patients with high trait anxiety scores, although this study evaluated the development of CRPS following distal radius fracture. ¹¹ These psychological factors were recognised and, where appropriate, dealt with by the various members of the team as effectively as possible.

Due to this patient’s longevity of symptoms, previous medical history and interactions with medical staff as a child and as a ME sufferer it could be assumed that the activation threshold of her neurotags ¹² had become sensitised and disinhibited causing even the thought of motion to activate a pain neurotag. The somatosensory homunculus ¹³ would also have been altered to give a greater representation of the hand and upper limb. Therefore, it is even less likely that a spontaneous resolution of symptoms could occur.

When reviewing this patient’s symptoms and response to treatment in light of the pain conceptual change model which reflects upon the patient’s ability to take on the conception that pain is not necessarily harmful, ¹⁴ it would seem that reconceptualisation ¹⁵ through education, reassurance and graded motor imagery was blocked. Therefore, it could be surmised that symptom relief would not be achievable.

Conclusion

This is a very unusual case of a prolonged severe and progressive CRPS that resolved spontaneously despite the lack of effect of all previous medical, surgical and therapeutic interventions. It is the intention that this case report should stimulate debate by other therapists regarding the diagnosis, adherence to the recommended medical and therapeutic management and outcome for patients suffering with CRPS.