Robert Blake Windsor1
1) United States
Introduction: This is a case of an atypical presentation of complex regional pain syndrome with development of digital necrosis and gangrene that prompted secondary evaluations of neuropathic pain. Peripheral nerve catheters were employed in an uncommon use to attempt limb salvage while undergoing diagnostic evaluation, with post-intervention angiography potentially demonstrating effects.
Case Description: A 10 year old female presented with diagnosis of CRPS due to episodes of allodynia, cyanosis, and inability to walk starting at age 5. Her episodes were cyclic, approximately 3-4 episodes per year, lasted 1 month, and with no pain or sensory changes between episodes. She had bilateral hand and feet complex syndactyly repairs early in life presumed to contribute to her diagnosis. She entered an episode and developed right lower extremity digital necrosis and gangrene that prompted a diagnostic evaluation for secondary causes of pain. She was ultimately diagnosed with medium vessel vasculitis. Because she had progressive digital necrosis, right sciatic and saphenous nerve catheters were used to provide sympathectomy for limb salvage during the diagnostic process. Following this intervention, her contralateral leg became threatened with poor perfusion. Subsequent angiography revealed unexpectedly normal perfusion to the necrotic foot, and poor perfusion to the contralateral leg without catheters.
Discussion: This case represents a diagnostic dilemma in the diagnosis of CRPS in children and the use of peripheral nerve catheters in vasculitis to attempt limb salvage through peripheral sympathectomy. Vasculitis is an important consideration in the differential diagnosis of CRPS and this case highlights the need for attention to factors such as age of onset, growth parameters, periodicity of symptoms, and inter-episodic sensory examination. It is unclear if sympathectomy provided salvage to the necrotic foot, but angiography was surprisingly normal considering the degree of necrosis, and the unilateral intervention in a bilateral process provides some ability for comparison.
