Abstract
Background:Children with sickle cell disease (SCD) frequently require in‐ or outpatient surgical procedures for various indications, including complications secondary to SCD itself. To prevent adverse events associated with an increased morbidity/mortality, these vulnerable patients need specific perioperative care. However, clinical practice in this area remains poorly defined, and is rarely based on high‐grade evidence.Aims:We aimed to a) collect evidence in a systematic, PRISMA‐based review of the literature and of available clinical guidelines, b) define care recommendations, and c) identify the most urgent clinical research priorities in this area. Additionally, we sought to construct an algorithm for clinical risk assessment.Methods:Records in the MEDLINE/Pubmed, National Guideline Clearinghouse, International Guideline Network and TRIP databases and Google/Google Scholar engines matching our search strategy were collected until January 2019. We also screened SCD patient advocacy and institutional healthcare group websites for any relevant recommendations. Data were extracted manually from full‐text sources in English, French, German, Italian. Excluded were reports on obstetrical and fetal management, for limited applicability to pediatric SCD care.Results:We identified 203 papers fulfilling our inclusion criteria. The majority of papers focused on visceral, cardiovascular and orthopedic surgery procedures. Seven studies had a RCT design, all the others were observational studies or recommendations. One hundred and fifty‐three studies provided evidence or recommendations for pediatric SCD (75%). After a careful analysis of all included studies, we identified the necessity for a) multidisciplinary collaboration and communication, b) the preemptive establishment of individualized perioperative care plans including initial investigations, optimized transfusion (if necessary) and anesthesia strategies, as well as postoperative pain and respiratory management. In addition, and whilst all procedures should ideally be performed by an experienced team, we found no evidence to restrict the use of routine surgical or anesthetic techniques in children with SCD. Careful operating room set‐up and intraoperative monitoring, including for blood loss, are necessary. Postoperatively, pre‐emptive opioid‐based analgesia and respiratory physiotherapy to prevent pulmonary complications are strongly recommended. In addition, the need for ICU admission and thromboprophylaxis should be discussed well in advance. Careful discharge planning is required to avoid re‐admission or post‐operative morbidity. Based on this data, we also designed an algorithm for procedural risk definition in SCD patients. Finally, we identified the most urgent research priorities including transfusion strategies for patients receiving hydroxyurea, HbS threshold definitions for high‐risk procedures, optimal postoperative respiratory management and opioid‐free analgesia plans.Summary/Conclusion:We performed a systematic review of the existing literature describing the care of children with SCD during the perioperative period, and outlined a set of clinical recommendations supported by the best available evidence.
Published Version
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