The use of partial exchange blood transfusion and anaesthesia in the management of sickle cell disease in a perioperative setting: two case reports

Abstract

IntroductionHomozygous sickle cell carriers have an increased perioperative mortality. Some indications may justify an exchange blood transfusion to reduce the proportion of haemoglobin S. The advantages of general blood transfusion in a perioperative setting have not been proven and thus remain controversial. It is not clear whether reducing the proportion of haemoglobin S minimizes perioperative complications or whether patients with sickle cell disease in a stable clinical condition benefit from an exchange blood transfusion in a perioperative setting.Case presentationWe report the case of two Angolan children aged 10 and 11 respectively, of African origin with sickle cell anaemia who underwent surgery to treat chronic necrosis, fistula of the bones and bone destruction. This presentation describes the perioperative course, including general anaesthesia. A partial exchange blood transfusion decreased S-haemoglobin levels from 81% to 21% and simultaneously treated the anaemia.ConclusionThere is a consensus that imbalances in homoeostasis, including operative procedures, can cause a critical exacerbation of sickle cell disease. The case presented here illustrates a strategy for successfully managing sickle cell disease in the perioperative period to minimize its complications. It is important for the anaesthesiologist to carefully manage pulmonary gas exchange and to ensure sufficient tissue perfusion, balanced fluid resuscitation and normothermia, while keeping in mind the level of organ impairment in order to prevent an acute exacerbation of sickle cell disease.We performed a partial exchange blood transfusion due to the following factors: high haemoglobin S-fraction, anaemia, operating procedure at several sites, and difficult management of body temperature. Esmarch ischemia is an established tool for preventing uncontrolled blood loss. There is no known contraindication for this, but attention must be paid to prevent uncontrolled tissue ischemia and acidosis. The use of regional anaesthesia should be considered for postoperative pain management.

Perioperative care of children with sickle cell disease: A systematic review and clinical recommendations

“…Some authors advocate for preoperative transfusion without giving a threshold, despite evidence from the abovementioned RCTs. Exchange transfusions (manual and automated) for a leve of HbS <30% is recommended for high risk surgery either preoperatively, or on cardiopulmonary bypass . In cardiac pulmonary bypass, some authors suggest a stricter HbS limit of <5% .…”

“…Intraoperative monitoring varies. Per standard anesthetic care, blood pressure, cardiac rhythm and rate and oxygenation are monitored strictly to avoid hypoxia . Some groups perform more invasive systematic monitoring such as the continuous measurement of central venous pressure, arterial blood pressure, regular arterial blood gas exchange and/or urine output determination (by Foley catheterization) .…”

Manual RBC exchange transfusion in a patient of sickle-beta thalassemia syndrome presenting in crisis: a case report

“…SCD causes progressive damage to multiple organs. 2 Manual RBC exchange is a simple and safe procedure that can be performed in the setting of acute complications of SCD and in patients with vaso occlusive crisis, previous stroke, to reduce HbS. It can be an additional therapy for refractory VOC and for respiratory improvement of acute thoracic syndrome, besides simple transfusion.…”

“…Clinical symptoms include relapsing ischemic episodes, chronic haemolysis and a specific type of anaemia termed sickle cell anaemia. 2 Homozygous Patients are in danger of increased perioperative mortality and have a reduction in the life expectancy. Hypothermia, hypoxia, acidosis and dehydration in the perioperative period can cause an acute exacerbation of the disease.…”

“…Hypothermia, hypoxia, acidosis and dehydration in the perioperative period can cause an acute exacerbation of the disease. 2 Transfusion may be aimed at either to correct an aggravation of anaemia or to provide non-deformable red cells to deliver oxygen to ischemic tissues in cases of vaso-occlusion. In the latter setting, an attention must be paid not to raise haematocrit above 35%, in order to prevent hyper viscosity.…”