Preoperative screening for sickle cell disease in children: clinical implications

Preoperative screening of at-risk patients for sickle cell disease (SCD) is recommended as a method to decrease perioperative morbidity. However, the effectiveness of pre-operative screening in accomplishing this goal has never been demonstrated. We undertook a retrospective study to determine the prevalence of positive test results among those screened preoperatively at our institution and to determine whether amendments to present screening guidelines can be recommended.

The hematology laboratory database of a university teaching hospital was searched to identify all patients who underwent preoperative screening for SCD from October 1999 to October 2003. The medical records of those patients testing positive were reviewed.

Of 1,906 children screened preoperatively, 79 (4.1%) were diagnosed as having sickle cell trait and three (0.16%) as having some form of SCD: one had homozygous hemoglobin S and two had sickle-hemoglobin C disease. Two of the three had a family history for SCD and none had a preoperative hemoglobin concentration < 10 g.dL(-1). No patient developed perioperative sickle-related complications.

Preoperative screening of 1,906 children identified only one asymptomatic child with undiagnosed SCD and a negative family history, suggesting that routine preoperative screening for SCD is rarely of significant clinical value in our population. Had preoperative screening not been performed, no child requiring preoperative transfusion would have been missed, representing a long-run probability of at least 99.84% that no at-risk child would require transfusion. We recommend that preoperative screening for SCD be undertaken selectively, giving consideration to the risks and benefits of screening to the individual patient.