Perioperative management of children with sickle hemoglobinopathy

Area of Science:

• Pediatric Surgery
• Hematology
• Transfusion Medicine

Background:

• Major hemoglobinopathies like sickle cell disease (SS), SC disease, and S-thalassemia pose surgical risks.
• Previous surgical preparation methods for these patients carried risks of morbidity and mortality.

Purpose of the Study:

• To evaluate the safety and efficacy of a specific preoperative transfusion protocol for children with major hemoglobinopathies undergoing surgery.
• To compare this protocol with alternative transfusion strategies.

Main Methods:

• A cohort of 35 children with major hemoglobinopathies underwent 46 surgical procedures between 1967 and 1978.
• Patients received preoperative packed erythrocyte transfusions to achieve a hematocrit of at least 36% (15-20 cc/kg).
• Surgery was performed immediately after transfusion for urgent cases and the next morning for elective cases.

Main Results:

• No mortality or unusual morbidity was observed in the 46 operations.
• The transfusion protocol was easily applied in both urgent and elective surgical settings.
• Achieving a minimum hematocrit of 36% via transfusion was a key factor in patient preparation.

Conclusions:

• Preoperative transfusion to a hematocrit of at least 36% is a safe and effective method for preparing children with major hemoglobinopathies for surgery.
• This transfusion approach is more desirable than two-volume exchange or delayed transfusions (10-15 days prior).
• The protocol's simplicity and excellent outcomes support its widespread adoption in pediatric surgical care for these patients.