Surgical procedures and outcomes among children with sickle cell disease.

Background: Although children with sickle cell disease often undergo surgery, there are limited current epidemiological data for this pediatric population. We performed a database analysis to estimate population characteristics, surgical procedures, and perioperative outcomes in this population.

Methods: We queried the Nationwide Inpatient Sample Database from 2000 to 2010 for discharges pertaining to patients <18 years of age having a diagnosis of sickle cell disease who underwent 1 or more surgical procedures during that admission. We abstracted surgical procedures using the Clinical Classifications Software procedure codes and the ICD-9-CM procedure codes. We described characteristics of patients undergoing the 6 most common procedures.

Results: During 2000 to 2010, 3.6 % (SE 0.12) of individual hospital discharges were of children with sickle cell disease who had undergone surgical procedures. The most frequent surgical procedures were cholecystectomy (1.47% [0.08]), tonsillectomy/adenoidectomy (0.81% [0.06]), splenectomy (0.62% [0.06]), repair of umbilical hernia (0.19% [0.02]), and appendectomy (0.17% [0.02]). Acute chest syndrome was recorded among 3.08% (0.60) of patients undergoing elective surgery. The incidence of stroke was 0.20% (0.11); death was reported in <11 patients (<0.20%).

Conclusion: Surgical procedures such as cholecystectomy, tonsillectomy, splenectomy, hernia repair, and appendectomy account for a small but significant proportion of hospital admissions in children with sickle cell disease. Acute chest syndrome is among the most common complications of elective surgery, while stroke and death are rare.