Perioperative Management of Patients with Sickle Cell Anemia

Department of Surgery, Dar A lAlafia Medical Company, Qatif, Saudi Arabia


  • Sickle cell anemia (SCA), which is due to homozygous inheritance of the HbS variant, is one of the commonly inherited hemoglobinopathies worldwide.

  • Patients with SCA often require operative interventions to treat SCA-related complications.

  • This however is associated with an increased risk of perioperative complications particularly vaso-occlusive crisis and acute chest syndrome.

  • Anesthesia and surgery are historically associated with an increased incidence of morbidity and mortality.

  • Recent multicenter data still found perioperative complications in 22 % of sickle cell patients undergoing elective surgery.

  • Optimal surgical outcome requires careful preoperative, intraoperative, and postoperative management by a team consisting of a surgeon, anesthesiologist, and hematologist.

  • Acute chest syndrome (ACS) is a well-documented postoperative complication in patients with SCA with an incidence ranging from 0.4 to 10 %.

  • The acute chest syndrome can be mild, moderate, or severe (Figs. 18.1, 18.2, and 18.3).


    Fig. 18.1
    Chest X-ray showing mild postoperative acute chest syndrome


    Fig. 18.2
    Chest X-ray showing a moderate postoperative acute chest syndrome in patient with sickle cell anemia


    Fig. 18.3
    A chest X-ray showing a severe postoperative acute chest syndrome which developed following splenectomy

  • A high HbF level (HbF level >20 %) has a protective effect and reduces the risk of postoperative ACS.

  • For many years, surgery on patients with SCA was associated with an unacceptably high complication rate, sometimes as high as 50 %.

  • These complications are part of the disease process and usually develop secondary to intraoperative or immediate postoperative events such as hypoxia, hypovolemia, hypothermia, and acidosis.

  • Although these complications can be seen following any operative procedure, they are more commonly seen in those undergoing thoracotomy or laparotomy (Figs. 18.4 and 18.5).


    Fig. 18.4
    A clinical intraoperative photograph showing laparotomy to remove a large spleen in a child with sickle cell anemia


    Fig. 18.5
    A clinical postoperative photograph showing a large abdominal wound following splenectomy and cholecystectomy. Such a large wound may be complicated by a postoperative acute chest syndrome

  • Nowadays and in spite of better understanding of SCA, improved anesthetic and surgical techniques, and careful perioperative management, the perioperative complication rate in these patients is still high.

  • Earlier studies reported a perioperative mortality rate as high as 10 % and a postoperative complication rate of up to 50 %, and because of this unacceptably high complication rate, they advocated against surgery except in symptomatic patients.

  • To minimize these risks, a variety of perioperative management protocols, including different preoperative blood transfusion regimens, have been adopted.

  • Blood transfusion is considered part of the preoperative management of patients with sickle cell anemia.

  • Perioperative blood transfusion to correct the anemia and decrease the percentage of HbS level is beneficial in reducing the rate of SCA-related complications, but the amount of blood transfusion necessary for safe surgery is not known.

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Jun 6, 2017 | Posted by in ONCOLOGY | Comments Off on Perioperative Management of Patients with Sickle Cell Anemia

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