MANAGEMENT AND THERAPY OF SICKLE CELL DISEASE

NIH Publication No. 95-2117, Revised December 1995 (Third Edition) National Institutes of Health, National Heart, Lung, and Blood Institute

Chapter 21--Surgery and Anesthesia

Patients with sickle cell disease who undergo surgery have an increased risk of perioperative complications. Careful preoperative preparation of the patient by a team consisting of a surgeon, hematologist, and anesthesiologist will minimize or eliminate these complications.

PREEXISTING HEALTH OF THE PATIENT

Patients with organ damage and/or coexistent disease must be identified because they are at increased risk for perioperative complications. In particular, older patients, those with a history of pulmonary or CNS disease, recurrent hospitalizations, and/or those who have been previously heavily transfused are at high risk for perioperative complications, especially acute chest syndrome and vaso-occlusive events. A physical examination and chart review should be supplemented by the following tests:

*Arterial oxygen pressure or oxygen saturation measured by pulse oximetry.

* Pulmonary function tests with bronchodilator response analysis for patients who have acute chest syndrome, asthma, or other pulmonary complications.

* Echocardiogram.

* Renal and liver functions.

TYPES OF SURGERY

Surgical procedures that have an increased probability of ischemia or hypoxia deserve special attention. These include cardiothoracic surgery; techniques associated with hypotension, hypothermia, and hyperventilation; and vascular surgery. Laparascopic surgery appears to lower the postoperative complications of sickle cell disease and should be used in appropriate settings.

PREOPERATIVE CARE

All patients should be evaluated by the anesthesiologist the day before surgery. Patients requiring general anesthesia should receive maintenance fluids at least 12 hours before surgery, with strict attention paid to urinary output and weight. Preoperative assessment of the patient should include checking for signs of vaso-occlusion, fever, infection, and dehydration. The laboratory and physical examination results should be reviewed to identify abnormalities in the heart, liver, kidneys, brain, and lungs.

INTRAOPERATIVE MANAGEMENT

All patients should be monitored with at least an EKG and have a determination of inspired oxygen concentration by pulse oximetry or blood gas testing. Measurements of electrolyte and urine output and invasive hemodynamic monitors may be required, depending on the patient's clinical status and the type of surgery. A warm temperature should be maintained in the operating room. General anesthesia should aim for a mild respiratory alkalosis (pH about 7.45) and a normothermic, well-hydrated patient. The patient should receive a minimum of 50 percent oxygen in combination with the anesthetic agent.

Blood replacement for significant intraoperative blood loss is recommended. Intraoperative blood salvage techniques (cell savers) are not recommended. Postoperatively, oxygen should be administered until the effects of anesthesia have worn off. Patients who have surgical wounds that interfere with respiration may require an extended use of oxygen. Continued monitoring by oximetry is recommended in the recovery room and/or intensive care unit. Postoperative parenteral hydration should keep the patient at 1 to 1 1/2 times maintenance. Aggressive respiratory care is necessary in sickle cell disease to minimize pulmonary complications.

BIBLIOGRAPHY

Bischoff RJ, Williamson A III, Dalali MJ, et al. Assessment of the use of transfusion therapy perioperatively in patients with sickle cell hemoglobinopathies. Ann Surg 1988;207:434-8.

Burrington JD. Elective and emergency surgery in children with sickle cell disease. Surg Clin North Am 1976;56:55-71.

Esseltine DW, Baxter M, Bevan JC. Sickle cell states and the anesthesiologist. Can J Anaesth 1988;35:385-403.

Fullerton MW, Philippart AI, Sarnaik S, Lusher J. Preoperative exchange transfusion in sickle cell anemia. J Ped Surg 1981;16:297-300.

Gibson J. Anesthesia for sickle cell disease and other hemoglobinopathies. Semin Anesth 1987;6:27-35.

Homi J. General anesthesia in sickle cell disease. Br Med J 1979;1:1599-1601.

Janik J, Seeler RA. Perioperative management of children with sickle hemoglobinopathy. J Pediatr Surg 1980;15:117-20.

Koshy M, Weiner SJ, Miller ST, et al. Surgery and anesthesia in sickle cell disease. Blood 1995;86(10). National Institutes of Health. NIH Consensus Development Conference Statement From Perioperative Red Cell Transfusion. Vol. 7, No. 4, June 27-29, 1988.

Searle JF. Anesthesia in sickle states. Anaesthesia 1973;28:48-58.

Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell disease. N Engl J Med 1995;333(4):206-13.

Ware R, Filston HC, Schultz WH, Kinney TR. Elective cholecystectomy in children with sickle hemoglobinopathies: successful outcome using preoperative transfusion regimen. Ann Surg 1988;208:17-35.

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