Paeds Vivas · rheumatology-musculoskeletal-and-sports
Pain amplification, juvenile fibromyalgia and complex regional pain syndrome: Viva
Branching clinical structured oral on amplified musculoskeletal pain, juvenile fibromyalgia and complex regional pain syndrome, covering the central sensitization mechanism, the 2010 American College of Rheumatology criteria, the Budapest clinical criteria, the red flags that mandate re-investigation, and the multidisciplinary management.
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Target exams
This oral is built to probe the reasoning that holds the central sensitization at the centre, and to expose the candidate who has memorised the pain label without the mechanism and the red flags. The questions escalate from the classification and the exclusion to the mechanism and the management of the two main phenotypes. [7]
Opening question: the classification and the exclusion
The examiner opens with the presentation and asks how you classify her pain and what you exclude before applying the amplified pain label. [3]
A strong answer names the juvenile fibromyalgia by the 2010 American College of Rheumatology preliminary diagnostic criteria, with the Widespread Pain Index and the Symptom Severity Scale, and then the exclusion of the juvenile idiopathic arthritis, the leukaemia and the infection through the focused history, the examination and the first-line blood panel. [1][3]
Model answer. This girl has the juvenile fibromyalgia by the 2010 criteria, with the widespread pain for at least three months, the Widespread Pain Index of seven or more and the Symptom Severity Scale of five or more, and no other disorder explaining the pain. The juvenile idiopathic arthritis is excluded by the absence of the morning stiffness and the synovitis, and the leukaemia is excluded by the normal full blood count and the absence of the systemic features. The amplified pain label is applied only after the exclusion. [1][9]
Pitfall probe. What are the red flags that exclude the primary amplified pain diagnosis? The objective synovitis, the persistent fever, the weight loss, the night pain that wakes the child, the progressive neurological deficit and the cytopenias. The amplified pain label is never applied to the child with an unexplained red flag. [3][9]
Probe one: the central sensitization mechanism
The examiner presses for the mechanism that underpins the amplified pain. [7]
A strong answer explains the central sensitization as the amplified processing of the nociceptive signals in the central nervous system with the reduced descending inhibition, producing the allodynia and the hyperalgesia, and the vicious cycle of the pain, the fear, the avoidance and the deconditioning that sustains the syndrome. [3][7]
Pitfall probe. Why does the pain not respond to the opioids? Because the opioids act on the peripheral and the spinal nociceptive pathways, and they do not reverse the central sensitization. The opioids are ineffective and they cause the dependence and the hyperalgesia, which is why they are avoided. [3]
Probe two: the multidisciplinary management
The examiner asks for the stepwise management of this girl. [6]
A strong answer names the four pillars of the multidisciplinary rehabilitation. The education on the central sensitization, the graded aerobic exercise increased through the pain, the cognitive behavioural therapy confirmed by the Cochrane review, and the sleep hygiene. The school reintegration is the urgent functional goal, the opioids and the surgery are avoided, and the parental catastrophizing is addressed because it drives the functional disability. [6][8]
Pitfall probe. Why is the school reintegration the treatment and not the reward for the recovery? Because the child who waits for the pain to resolve before the return to the school waits indefinitely, and the return to the function drives the recovery of the deconditioned body and the desensitised nervous system. The functional goals replace the pain intensity goals. [7]
Branch one: the complex regional pain syndrome
The examiner pivots to the scenario where the pain is a severe burning regional pain in a single limb with the allodynia and the skin colour change, and asks how you classify and manage it. [9]
A strong answer names the complex regional pain syndrome by the Budapest clinical criteria, with the continuing disproportionate pain plus the symptoms in three of the four categories and the signs in two, and the management by the early mobilisation and the desensitisation and the graded motor imagery. The immobilisation is reversed, the opioids are avoided, and the intensive functional restoration is the escalation. [9]
Pitfall probe. Why is the immobilisation of the limb with the complex regional pain syndrome harmful? Because the immobilisation drives the contracture, the muscle atrophy and the permanent disability. The early mobilisation is the prompt action, even though it hurts, because the pain of the movement is the central sensitization and not the tissue damage. [9]
Branch two: the refractory case
The examiner pivots to the scenario where the child has not responded to the outpatient management and the school absence is prolonged, and asks for the escalation. [7]
A strong answer names the intensive functional restoration programme of the Sherry model, the inpatient or the day-programme rehabilitation that delivers the daily physiotherapy, the psychology and the schooling over several weeks, and achieves the return to the full function in the great majority. [7]
Pitfall probe. What is the role of the sympathetic nerve blocks and the neuromodulation in the pediatric complex regional pain syndrome? They are rarely needed, and they are reserved for the refractory case that has not responded to the functional restoration. The first-line is always the multidisciplinary rehabilitation. [9]
Closing question: the single framework
The examiner closes and asks for the single framework that carries the whole topic. [3]
A strong answer states that the amplified musculoskeletal pain is the chronic pain driven by the central sensitization, presenting as the widespread juvenile fibromyalgia or the regional complex regional pain syndrome. The diagnosis is the pattern and the exclusion, the red flags are excluded, and the management is the multidisciplinary rehabilitation of the education, the exercise, the cognitive behavioural therapy and the sleep hygiene, with the explanation of the central sensitization as the first treatment and the functional restoration as the goal. The candidate who holds the central sensitization framework, the two criteria sets, the four pillars and the avoidance of the opioids and the surgery has the corners that the examination rewards. [3][7]
References
- [1]Ting TV, Barnett K, Zelikovsky N, et al 2010 American College of Rheumatology Adult Fibromyalgia Criteria for Use in an Adolescent Female Population with Juvenile Fibromyalgia J Pediatr, 2016.PMID 26545727
- [3]Kashikar-Zuck S, Ting TV Juvenile fibromyalgia: current status of research and future developments Nat Rev Rheumatol, 2014.PMID 24275966
- [6]Eccleston C, Palermo TM, Williams AC, Lewandowski A, Morley S Psychological therapies for the management of chronic and recurrent pain in children and adolescents Cochrane Database Syst Rev, 2012.PMID 23235601
- [7]Sherry DD, Sonagra M, Milojevic D The spectrum of pediatric amplified musculoskeletal pain syndrome Pediatr Rheumatol Online J, 2020.PMID 33046102
- [8]Dougherty BL, Zelikovsky N, Schurman JV, Williams SE Longitudinal Impact of Parental Catastrophizing on Child Functional Disability in Pediatric Amplified Pain J Pediatr Psychol, 2021.PMID 33491076
- [9]Low AK, Ward K, Wines AP Pediatric complex regional pain syndrome J Pediatr Orthop, 2007.PMID 17585269