Amputations in Sickle Cell Disease: Case Series and Literature Review

Abstract

In this study, we describe four new patients with sickle cell disease who had limb amputations. Two of the patients had sickle cell anemia [Hb S (HBB: c.20A > T) (β^S/β^S)] with refractory leg ulcers that required amputations. The third patient had sickle cell trait with an extensive leg ulcer that was associated with epidermoid carcinoma. The fourth patient had amputations of both forearms and feet due to a misdiagnosis of dactylitis. Review of the literature showed that the indications for amputations in sickle cell disease included three distinct categories: mythical beliefs, therapeutic and malpractice. All therapeutic amputations were for severely painful, large, recalcitrant leg ulcers that failed non-interventional therapies. Amputation resulted in pain relief and better quality of life. Phantom neuropathic pain was not a major issue post-operatively. It was absent, transient or well controlled with antidepressants. Limb function was restored post-amputation with prosthetic artificial limbs, wheelchairs or crutches. Malpractice amputations were due to misdiagnosis or to cryotherapy by exposing the painful limb to ice water resulting in thrombosis, gangrene and amputation. We strongly suggest that leg amputations should be considered in the management of certain patients with severe extensive refractory leg ulcers, and topical cryotherapy should never be used to manage sickle cell pain.

• Amputation
• leg ulcers
• phantom pain
• sickle cell anemia
• sickle cell trait

Acknowledgements

CM contributed Cases 1 and 3 to the study, reviewed and edited the manuscript; STOS contributed Case 2 to the study, reviewed and edited the manuscript; AMMQ coordinated the study, reviewed and edited the manuscript; CL obtained funding for the study, reviewed and edited the manuscript, SKB contributed Case 4, designed the study, obtained funding for the study, wrote and edited the manuscript.

Declaration of interest

Supported in part by the Office of the Secretary of Health for the State of Rio de Janeiro and the Brazilian Ministry of Health and in part by the Sickle Cell Program of the Department of Health of the Commonwealth of Pennsylvania, Harrisburg, PA, USA. The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.