BACKGROUND: Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting approximately 100 000 Americans, disproportionately affecting Black individuals. Sickling of hemoglobin S red blood cells due to conditions in the perioperative period including hypoxemia, hypothermia, surgical stress, and pain can reduce organ perfusion and lead to adverse outcomes including pain crisis, acute chest syndrome, and stroke.
AIMS: This study evaluates perioperative outcomes and risk factors for sickle cell crisis in children with SCD undergoing common inpatient surgical procedures.
METHODS: A retrospective cohort was created using the Healthcare Cost and Utilization Project (HCUP) Kids' Inpatient Database (KID) 2003-2019. Hospital admissions for children < 18 years of age undergoing a selected group of surgical procedures (cholecystectomy, appendectomy, congenital cardiac surgery, and posterior spinal fusions) commonly requiring inpatient stay were included. Diagnoses and procedures were identified using ICD-9/ICD-10 codes. Perioperative outcomes included post-operative length of stay (LOS), blood transfusions, hematologic, and infectious complications. Children with SCD were compared to those without SCD using multivariable Poisson regression to adjust for surgical procedure and sociodemographic, clinical, and hospital characteristics.
RESULTS: Of 5 75 005 children studied, 2357 (0.4%) had SCD. Relative to children without SCD, those with SCD had a longer post-operative LOS (adjusted incidence rate ratio [aIRR]: 1.29; 95% CI [1.26-1.32], p < 0.001). Children with SCD hospitalized for a surgical procedure were also more likely to receive a blood transfusion (adjusted risk ratio [aRR]: 13.1; 95% CI [12.1-14.2], p < 0.001). Significantly increased associated risks of hematologic and infectious complications, however, were not observed. Of children with SCD, 17.5% experienced a sickle cell crisis during hospitalization. The odds of sickle cell crisis in non-elective admissions were more than three times as high as in elective admissions (aOR 3.36; 95% CI [2.46-4.60], p < 0.001). Children with sickle cell crisis had a longer post-operative hospital stay (aIRR: 1.58; 95% CI [1.49-1.67]) than those without a crisis.
CONCLUSIONS: The perioperative course in children with SCD was associated with longer postoperative length of stay and higher blood transfusion rates relative to those without SCD undergoing similar surgical procedures. Perioperative sickle cell crisis was present in more than one out of six admissions, more common in non-elective admissions, and is associated with excess length of stay.