Can children with sickle cell disease undergo open splenectomy without preoperative transfusion despite severe anemia? A report of three cases

Abstract

Preoperative red cells transfusion to correct anaemia and to reduce the proportion of sickle red cells is part of standard preparation of children with sickle cell anaemia (HbSS) for major procedures including open abdominal surgeries. We report three children with sickle cell anaemia presenting with chronic massive splenomegaly and hypersplenism. The children were initially denied surgery because of extremely low haemoglobin levels and the inefficacy of transfusion. Subsequently, they underwent successful open abdominal splenectomy without any red cells transfusion. These observations are important to paediatricians and surgeons in settings where HbSS is common. They highlight the fact that surgery should not be withheld from children with sickle cell anaemia and massive splenomegaly purely on the basis of difficulty in correcting anaemia before the procedure.

Keywords

• sickle cell disease
• surgery
• splenectomy
• transfusion