Children with sickle cell disease (SCD) require specific perioperative care, and clinical practice in this area remains poorly defined. We aimed to conduct a systematic, PRISMA-based review of the literature, available clinical guidelines and practice recommendations. We also aimed to extract any valuable information for the "best of available-evidence"-based prevention of perioperative adverse events in children with SCD, and highlight the most urgent priorities in clinical research. As data sources, USWe also included institutional, consortia and expert group guidelines. Included were reports/guidelines in English, French, German, and Italian. Excluded were reports on obstetrical and fetal management. We identified 202 reports/guidelines fulfilling the criteria outlined above. A majority focused on visceral, cardiovascular and orthopedic surgery procedures, and only five were multicenter randomized controlled trials and two prospective randomized studies. After grading of the quality of the evidence, the extracted data was summarized into clinical recommendations for daily practice. Additionally, we designed a risk-grading algorithm to identify contexts likely to be associated with adverse outcomes. In conclusion, we provide a systematic PRISMA-based review of the existing literature and ancillary practice and delineate a set of clinical recommendations and priorities for research.

We tested whether prophylactic droperidol and ondansetron, in combination with a moderate dose of dexamethasone, were equally effective in reducing nausea and vomiting after tonsillectomy in children and that both were superior to saline with dexamethasone. We randomly allocated 300 children to intravenous saline, droperidol 10 μg.kg or ondansetron 150 μg.kg , after induction of anaesthesia and the administration of intravenous dexamethasone 250 μg.kg . The rates (95%CI) of nausea or vomiting within 24 postoperative hours were: 42/91 after saline, 46% (36%-57%); 43/87 after droperidol, 49% (39%-60%); reduced to 18/84 by ondansetron, 21% (13%-32%), p < 0.001. There were no differences in the rates of side-effects between groups. We conclude that ondansetron is more effective than saline in preventing nausea or vomiting after paediatric tonsillectomy when given with a moderate dose of dexamethasone, whereas droperidol was not.

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.

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