Review Article

CME Article: Perioperative Management in Sickle Cell Disease

Authors: Rita D. Paschal, MD

Abstract

Many patients with sickle cell disease (SCD) will require surgical intervention during the course of their lifetime. Common surgeries include orthopedic and abdominal procedures. Perioperative complications occur commonly and can be related to the surgical procedure or the underlying hemoglobinopathy. The complication rate may be reduced by preoperative optimization of disease and careful attention to the patient in the postoperative period. This review examines the perioperative management of patients with SCD. For patients undergoing both elective and emergent surgery, attempts should be made to coordinate care with an SCD specialist.

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References

1. Steinberg MH. Management of sickle cell disease. N Engl J Med 1999;340:1021-1030.
 
2. US Department of Health and Human Services. Evidence-based management of sickle cell disease: expert panel report, 2014. https://www.nhlbi.nih.gov/sites/www.nhlbi.nih.gov/files/sickle-cell-disease-report.pdf. Accessed April 17, 2016.
 
3. Bunn HF. Pathogenesis and treatment of sickle cell disease. N Engl J Med 1997;337:762-769.
 
4. Steinberg MH. Pathophysiology of sickle cell disease. Baillieres Clin Haematol 1998;11:163-184.
 
5. Ballas SK, Mohandas N. Sickle red cell microrheology and sickle blood rheology. Microcirculation 2004;11:209-225.
 
6. Koshy M, Weiner SJ, Miller ST, et al. Surgery and anesthesia in sickle cell disease. Cooperative Study of Sickle Cell Diseases. Blood 1995;86:3676-3684.
 
7. Schmalzer EA, Lee JO, Brown AK, et al. Viscosity of mixtures of sickle and normal red cells at varying hematocrit levels. Implications for transfusion Transfusion 1987;27:228-233.
 
8. Howard J, Malfroy M, Llewelyn C, et al. The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial. Lancet 2013;381:930-938.
 
9. Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. The Preoperative Transfusion in Sickle Cell Disease Study Group. N Engl J Med 1995;333:206-213.
 
10. Firth PG. Anesthesia and hemoglobinopathies. Anesthesiol Clin 2009;27:321-336.
 
11. Firth PG, McMillan KN, Haberkern CM, et al. A survey of perioperative management of sickle cell disease in North America. Paediatr Anaesth 2011;21:43-49.
 
12. De Jong A, Futier E, Millot A, et al. How to preoxygenate in operative room: healthy subjects and situations "at risk.". Ann Fr Anesth Reanim 2014;33:457-461.
 
13. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group N Engl J Med 2000;342:1855-1865.
 
14. Naik RP, Streiff MB, Haywood C, Jr et al. Venous thromboembolism incidence in the Cooperative Study of Sickle Cell Disease. J Thromb Haemost 2014;12:2010-2016.
 
15. Stein PD, Beemath A, Meyers FA, et al. Deep venous thrombosis and pulmonary embolism in hospitalized patients with sickle cell disease. Am J Med 2006;119:897.e7-e11.
 
16. Novelli EM, Huynh C, Gladwin MT, et al. Pulmonary embolism in sickle cell disease: a case-control study. J Thromb Haemost 2012;10:760-766.
 
17. Ataga KI, Key NS. Hypercoagulability in sickle cell disease: new approaches to an old problem. Hematology Am Soc Hematol Educ Program 2007;:91-96.
 
18. Gould MK, Garcia DA, Wren SM, et al. Prevention of VTE in nonorthopedic surgical patients: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest 2012;141(2 Suppl):e227S-e277S.
 
19. Falck-Ytter Y, Francis CW, Johanson NA, et al. Prevention of VTE in orthopedic surgery patients: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest 2012;141(2 Suppl):e278S-e325S.