Complex regional pain syndrome type I in children: What is new?

Complex regional pain syndrome type I (CRPS-I), although first described by the French surgeon Ambroise Paré as far back as the 16th century, nevertheless remains shrouded in mystery. The most common symptoms are pain in an entire hand or foot, allodynia, functional impairment induced by the pain, local oedema and skin color changes and transient sweating abnormalities. Most cases occur after a minor injury (i.e., a sprain or fracture), although there may be no identifiable triggering event, particularly in children. Primarily cold CRPS-I is by far the most common variant in children. Development of the Budapest criteria has benefited the diagnosis. These criteria are clinical and no specific diagnostic investigation is available. In vitro and in vivo studies have established that several pathogenic mechanisms can be involved concomitantly. However, there is no satisfactory explanation to the full clinical spectrum. Blood tests and imaging studies are useful for ruling out other diagnoses then monitoring the course of the condition, which may involve the development of demineralisation or osteopenia. High-resolution peripheral quantitative computed tomography may be helpful, as it provides quantitative assessments of the cortical and trabecular bone. CRPS-I has several specific characteristics in children compared to adults and whether the condition is the same entity in these two age groups is a legitimate question.

The optimal management involves an early diagnosis followed by a multidisciplinary management programme of functional rehabilitation therapy and cognitive behavioral therapy. Analgesics are useful only during the phase of acute pain and to facilitate physical therapy. Studies in adults showed that bisphosphonates were effective within the first 12 months after symptom onset and calcitonin in longer-lasting cases. No high-quality clinical research studies into the aetiopathogenesis and treatment of CRPS-I in children and adolescents are available to date.