Neurogenic Edema from Complex Regional Pain Syndrome Resulting in Fulminant Infection Necessitating Below Elbow Amputation

Abstract

We report a case of severe upper extremity complex regional pain syndrome type 1 (CRPS-1) and neurogenic edema that ultimately led to a medically necessary below-elbow amputation. The patient presented with a history of remote bilateral carpal tunnel release complicated by debilitating and recalcitrant bilateral CRPS-1. Following years of severe neurogenic edema of the left upper extremity, the patient had full-thickness skin sloughing on the dorsum of her hand due to massive edema. This subsequently led to maggot infestation of the soft tissues of the left hand ultimately necessitating amputation. We present the case as an illustration of an extreme case of neurogenic edema, a potential physical manifestation of CRPS-1. The case presented discusses upper extremity amputation as an end treatment option for CRPS-1, though in this case amputation was primarily indicated secondary to medical necessity.

Keywords

amputation - carpal tunnel syndrome - complex regional pain syndrome - infection - neurogenic edema

• References

• 1 de Mos M, Huygen FJ, van der Hoeven-Borgman M, Dieleman JP, Ch Stricker BH, Sturkenboom MC. Outcome of the complex regional pain syndrome. Clin J Pain 2009; 25 (07) 590-597
• 2 de Mos M, de Bruijn AG, Huygen FJ, Dieleman JP, Stricker BH, Sturkenboom MC. The incidence of complex regional pain syndrome: a population-based study. Pain 2007; 129 (1–2): 12-20
• 3 Harden RN, Bruehl S, Stanton-Hicks M, Wilson PR. Proposed new diagnostic criteria for complex regional pain syndrome. Pain Med 2007; 8 (04) 326-331
• 4 Bean DJ, Johnson MH, Kydd RR. The outcome of complex regional pain syndrome type 1: a systematic review. J Pain 2014; 15 (07) 677-690
• 5 Allen G, Galer BS, Schwartz L. Epidemiology of complex regional pain syndrome: a retrospective chart review of 134 patients. Pain 1999; 80 (03) 539-544
• 6 Petersen PB, Mikkelsen KL, Lauritzen JB, Krogsgaard MR. Risk factors for post-treatment complex regional pain syndrome (CRPS)—an analysis of 647 cases of CRPS from the Danish Patient Compensation Association. Pain Pract 2017; DOI: 10.1111/papr.12610.
• 7 Savaş S, İnal EE, Yavuz DD, Uslusoy F, Altuntaş SH, Aydın MA. Risk factors for complex regional pain syndrome in patients with surgically treated traumatic injuries attending hand therapy. J Hand Ther 2017; DOI: 10.1016/j.jht.2017.03.007.
• 8 Cossins L, Okell RW, Cameron H, Simpson B, Poole HM, Goebel A. Treatment of complex regional pain syndrome in adults: a systematic review of randomized controlled trials published from June 2000 to February 2012. Eur J Pain 2013; 17 (02) 158-173
• 9 Krans-Schreuder HK, Bodde MI, Schrier E. , et al. Amputation for long-standing, therapy-resistant type-I complex regional pain syndrome. J Bone Joint Surg Am 2012; 94 (24) 2263-2268
• 10 Midbari A, Suzan E, Adler T. , et al. Amputation in patients with complex regional pain syndrome: a comparative study between amputees and non-amputees with intractable disease. Bone Joint J 2016; 98-B [phone]
• 11 Geertzen JH, Rietman JS, Smit AJ, Zimmerman KW. A young female patient with reflex sympathetic dystrophy of the upper limb in whom amputation became inevitable. Prosthet Orthot Int 1997; 21 (02) 159-161
• 12 Stanton-Hicks M, Jänig W, Hassenbusch S, Haddox JD, Boas R, Wilson P. Reflex sympathetic dystrophy: changing concepts and taxonomy. Pain 1995; 63 (01) 127-133
• 13 Merskey H, Bogduk N. Classification of Chronic Pain: IASP Task Force on Taxonomy. 2nd ed. IASP Press: Seattle, Washington: IASP Press; 1994: 40-43
• 14 Parkitny L, McAuley JH, Di Pietro F. , et al. Inflammation in complex regional pain syndrome: a systematic review and meta-analysis. Neurology 2013; 80 (01) 106-117
• 15 Weber M, Birklein F, Neundörfer B, Schmelz M. Facilitated neurogenic inflammation in complex regional pain syndrome. Pain 2001; 91 (03) 251-257
• 16 Fischer SG, Zuurmond WW, Birklein F, Loer SA, Perez RS. Anti-inflammatory treatment of complex regional pain syndrome. Pain 2010; 151 (02) 251-256
• 17 Taskaynatan MA, Balaban B, Karlidere T, Ozgul A, Tan AK, Kalyon TA. Factitious disorders encountered in patients with the diagnosis of reflex sympathetic dystrophy. Clin Rheumatol 2005; 24 (05) 521-526
• 18 MacDonald RI, Lichtman DM, Hanlon JJ, Wilson JN. Complications of surgical release for carpal tunnel syndrome. J Hand Surg Am 1978; 3 (01) 70-76
• 19 Bean DJ, Johnson MH, Heiss-Dunlop W, Lee AC, Kydd RR. Do psychological factors influence recovery from complex regional pain syndrome type 1? A prospective study. Pain 2015; 156 (11) 2310-2318
• 20 Rome L. The place of occupational therapy in rehabilitation strategies of complex regional pain syndrome: comparative study of 60 cases. Hand Surg Rehabil 2016; 35 (05) 355-362
• 21 Veizi IE, Chelimsky TC, Janata JW. Complex [corrected] regional pain syndrome: what specialized rehabilitation services do patients require?. Curr Pain Headache Rep 2012; 16 (02) 139-146
• 22 Zollinger PE, Tuinebreijer WE, Kreis RW, Breederveld RS. Effect of vitamin C on frequency of reflex sympathetic dystrophy in wrist fractures: a randomised trial. Lancet 1999; 354 (9195): 2025-2028
• 23 Evaniew N, McCarthy C, Kleinlugtenbelt YV, Ghert M, Bhandari M. Vitamin C to prevent complex regional pain syndrome in patients with distal radius fractures: a meta-analysis of randomized controlled trials. J Orthop Trauma 2015; 29 (08) e235-e241
• 24 Grunert BK, Smith CJ, Devine CA. , et al. Early psychological aspects of severe hand injury. J Hand Surg Br 1988; 13 (02) 177-180
• 25 Tennent DJ, Wenke JC, Rivera JC, Krueger CA. Characterisation and outcomes of upper extremity amputations. Injury 2014; 45 (06) 965-969