Preoperative screening for sickle cell disease in children: clinical implications

Abstract

Purpose: Preoperative screening of at-risk patients for sickle cell disease (SCD) is recommended as a method to decrease perioperative morbidity. However, the effectiveness of preoperative 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. Methods: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.Results: 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. Conclusion: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. Objectif

Congenital malformations and medical conditions associated with orofacial clefts in children in Burkina Faso

“…Anaesthetics and operative risks are so low that preoperative screening for sickle cell disease in children, in general and among those with CL/Ps in particular, would not be of great utility [33, 34]. …”

Effectiveness of preoperative screening for sickle cell disease in a population with a newborn screening program: a cohort study

“…These findings suggest that selective preoperative screening of children based on at-risk ethnicity is None SCD = sickle cell disease; SS = homozygous hemoglobin S; RCC = red cell concentrate; Hb = hemoglobin; HbS = sickle hemoglobin; HbA = hemoglobin A; HbA2 = hemoglobin A2; HbF = fetal hemoglobin an ineffective method of identifying children with SCD in a population that has a newborn sickle hemoglobinopathy screening program. 10 The previously undiagnosed case of SCD in this cohort prompted the introduction of a policy mandating that all newborns transferred to our hospital at birth must undergo the newborn screen upon admission to hospital, regardless of any history of newborn screening at the transferring hospital. The aim of this policy is to ensure that all newborns transferred to our hospital for emergency care undergo the newborn screen.…”

“…2 Prior to the introduction of newborn screening in Ontario, selective preoperative screening identified fewer than two new cases of SCD per 1,000 at-risk children (0.16%) at our institution, and screening did not alter the perioperative course of any child not previously diagnosed with SCD. 10 Following the introduction of newborn screening, the benefit of a continued policy of selective preoperative screening of all children of at-risk ethnicities has diminished.…”

Preoperative screening for sickle cell disease in children: a pragmatic solution in a UK district hospital

“…Had no preoperative testing been performed, no child requiring preoperative transfusion would have been missed. The study concludes that routine testing is not necessary and should only be undertaken on a selective basis (8).…”