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. Tw o 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.
Résumé
Objectif
Le dépistage préopératoire des patients à risque de drépanocytose est recommandé pour réduire la morbidité périopératoire. Ľefficacité de ce dépistage n’a pourtant jamais été démontrée. Notre étude rétrospective voulait déterminer la prévalence de résultats positifs aux tests préopératoires à notre institution et pour déterminer si nous pouvons recommander des modifications aux présentes normes de dépistage.
Méthode
La base de données du laboratoire ďhématologie ďun hôpital universitaire a été revue à la recherche des patients soumis à un dépistage préopératoire de la drépanocytose entre octobre 1999 et octobre 2003. Ľexamen des dossiers médicaux présentant des tests positifs a été fait.
Résultats
Des 1 906 enfants vus en dépistage préopératoire, 79 (4,1 %) présentaient un trait drépanocytaire et 3 (0,16 %) une forme de drépanocytose : un avait une hémoglobinose S homozygote et 2 avec falciformation-hémoglobine C. Deux des trois avaient des antécédents familiaux de drépanocytose et aucun n’avait une concentration préopératoire ďhémoglobine < 10 g·dL-1 . Aucune complication de falciformation ne s’est manifestée chez ces patients.
Conclusion
Du dépistage préopératoire de 1 906 enfants, il y avait un enfant asymptomatique atteint de drépanocytose non diagnostiquée et sans antécédents familiaux. Donc, le dépistage préopératoire de routine de la drépanocytose n’a que rarement une valeur clinique significative dans notre population. Même sans le dépistage préopératoire, aucun enfant nécessitant une transfusion préopératoire n’aurait été oublié, ce qui représente une probabilité à long terme ďau moins 99,84 % qu’aucun enfant à risque aurait eu besoin de transfusion. Le dépistage préopératoire sélectif de la drépanocytose est recommandé, tenant compte des risques et des avantages du dépistage ďun patient en particulier.
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References
Serjeant GR, Serjeant BE. Sickle Cell Disease, 3rd ed. Oxford: Oxford University Press; 2001: 23–8.
Motulsky AG. Frequency of sickling disorders in U.S. blacks. N Engl J Med 1973; 288: 31–3.
Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease. Rates and risk factors. N Engl J Med 1991; 325: 11–6.
Banerjee AK, Layton DM, Rennie JA, Bellingham AJ. Safe surgery in sickle cell disease. Br J Surg 1991; 78: 516–7.
Pemberton PL, Down JF, Porter JB, Bromley LM. A retrospective observational study of pre-operative sickle cell screening. Anaesthesia 2002; 57: 334–9.
Baraka A, Haddock G, Roussis X, Leventis J, Fyfe AH. An audit of sickle cell screening in a paediatric hospital population. Scott Med J 1994; 39: 84–5.
Scott-Conner CE, Brunson CD. The pathophysiology of the sickle hemoglobinopathies and implications for perioperative management. Am J Surg 1994; 168: 268–74.
Eichhorn JH. Preoperative screening for sickle cell trait. JAMA 1988; 259: 907.
Means LJ, Ferrari LR, Fisher QA, Kingston HG, Schreiner MS. American Academy of Pediatrics, Section on Anesthesiology. Evaluation and preparation of the pediatric patients undergoing anesthesia. Pediatrics 1996; 98: 502–8.
Marchant WA, Walker I. Anesthetic management of the child with sickle cell disease. Pediatr Anaesth 2003; 13: 473–89.
Firth PG, Head CA. Sickle cell disease and anesthesia. Anesthesiology 2004; 101: 766–85.
Lorey F, Cunningham G, Shafer F, Lubin B, Vichinsky E. Universal screening for hemoglobinopathies using high-performance liquid chromatography: clinical results of 2.2 million screens. Eur J Hum Genet 1994; 2: 262–71.
Sickle Cell Disease Guideline Panel. Sickle cell disease: screening, diagnosis, management, and counseling in newborns and infants. Clinical practice guideline number 6, Agency for Health Care Policy and Research, publication number 93-0562, Public Health Services, U.S. Department of Health and Human Services; April 1993.
Shafer FE, Lorey F, Cunningham GC, Klumpp C, Vichinsky E, Lubin B. Newborn screening for sickle cell disease: 4 years of experience from California’s newborn screening program. J Pediatr Hematol Oncol 1996; 18: 36–41.
Koshy M, Weiner SJ, Miller ST, et al. Surgery and anesthesia in sickle cell disease. The Cooperative Study of Sickle Cell Disease. Blood 1995; 86: 3676–84.
Delatte SJ, Hebra A, Tagge EP, Jackson S, Jacques K, Biemann Othersen H jrJr. Acute chest syndrome in the postoperative sickle cell patient. J Pediatr Surg 1999; 34: 188–92.
Wales PW, Carver E, Crawford MW, Kim PC. Acute chest syndrome after abdominal surgery in children with sickle cell disease: is a laparoscopic approach better? J Pediatr Surg 2001; 36: 718–21.
Crawford MW, Speakman M, Carver ED, Kim PC. Acute chest syndrome shows a predilection for basal lung regions on the side of upper abdominal surgery. Can J Anesth 2004; 51: 707–11.
Fleming AF. The presentation, management and prevention of crisis in sickle cell disease in Africa. Blood Reviews 1989; 3: 18–28.
Oduro KA, Searle JF. Anaesthesia in sickle-cell states: a plea for simplicity. Br Med J 1972; 4: 596–8.
Platt OS, Brambilla DJ, Rosse WF, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med 1994; 330: 1639–44.
Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease. Incidence and risk factors. Blood 1994; 84: 643–9.
Bunn HF. Pathogenesis and treatment of sickle cell disease. N Eng J Med 1997; 337: 762–9.
Githens JH, Lane PA, McCurdy RS, Houston ML, McKinna JD, Cole DM. Newborn screening for hemoglobinopathies in Colorado. The first 10 years. Am J Dis Child 1990; 144: 466–70.
Kolk AM, van Hoof R, Fiedeldij Dop MJ. Preparing children for venepuncture. The effect of an integrated intervention on distress before and during venepuncture. Child Care Health Dev 2000; 26: 251–60.
O’Connor ME, Drasner K. Preoperative laboratory testing of children undergoing elective surgery. Anesth Analg 1990; 70: 176–80.
Charpak Y, Blery C, Chastang C. Designing a study for evaluating a protocol for the selective performance of preoperative tests. Stats Med 1987; 6: 813–22.
Macpherson DS. Preoperative laboratory testing: should any tests be "routine" before surgery? Med Clin North Am 1993; 77: 289–308.
Newborn screening for sickle cell disease and other hemoglobinopathies. Consensus conference. JAMA 1987; 258: 1205–9.
Vichinsky E, Hurst D, Earles A, Earles A, Kleman K, Lubin B. Newborn screening for sickle cell disease: effect on mortality. Pediatrics 1988; 81: 749–55.
Ohene-Frempong K. Selected testing of newborns for sickle cell disease. Pediatrics 1989; 83(5Pt2): 879–80.
Mack AK. Florida’s experience with newborn screening. Pediatrics 1989; 83(5Pt2): 861–3.
Davies SC, Cronin E, Gill M, Greengross P, Hickman M, Normand C. Screening for sickle cell disease and thalassaemia: a systematic review with supplementary research. Health Technol Assess 2000; 4: 1–99.
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Crawford, M.W., Galton, S. & Abdelhaleem, M. Preoperative screening for sickle cell disease in children: clinical implications. Can J Anesth 52, 1058–1063 (2005). https://doi.org/10.1007/BF03021605
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DOI: https://doi.org/10.1007/BF03021605