Perioperative steroid prophylaxis for adrenal insufficiency, a single‐centre experience

Perioperatively, patients with adrenal insufficiency are at risk of adrenal crisis due to an attenuated cortisol response during periods of physiological stress. Primary and secondary adrenal insufficiency occur at rates of 1 per 5000–10 000 children.1 Adrenal crises are potentially life-threatening emergencies with cardinal features of profound hypotension and hypoglycaemia.1, 2 Like all rare but potentially catastrophic crises perioperatively, appropriate awareness, knowledge, and protocols are required to safely manage patients. Stress dosing of hydrocortisone, often combined with dextrose containing intravenous fluids perioperatively, is the primary management strategy.1, 3 In addition to a weight-based approach, dosage calculations per body surface area (BSA) are common in pediatrics to address potential height-related differences. Pediatric-specific data on perioperative glucocorticoid management is very limited with current recommendations primarily based on hospital protocols or extrapolated from adult data. Based on available evidence (UK and Endocrine Society guidelines for emergency and perioperative management of adrenal insufficiency in children and young people: BSPED consensus guidance Developed by the Pediatric Adrenal Insufficiency Group on behalf of the British Society of Pediatric Endocrinology & Diabetes (BSPED)1, 3 an institutional guideline was developed: Major surgery; induction hydrocortisone (intravenous) 50–100 mg/m2 or 2–4 mg/kg followed by postsurgical stress dosing of 25 mg/m2/day (intravenous) 6-h for 24-h then 12.5 mg/m2/day (intravenous) 6-h for 24-h then once oral route established, 7.5 mg/m2/day 6-h for 48-h. Minor surgery; induction hydrocortisone (intravenous) 50 mg/m2 or 2 mg/kg followed by postsurgical stress dosing of triple the normal daily dose or 30 mg/m2/day in four equal doses over 24-h.1-3 Prior to guideline implementation a retrospective analysis of data on perioperative dosing of hydrocortisone was completed for all patients with diagnosed adrenal insufficiency (primary or secondary) undergoing general anesthesia over a period of 24 years (11/9/1997-17/2/2021, approval-No: 2023-000442), 100 endocrinology patients were identified with conditions requiring regular steroid medication, with 70 diagnosed with adrenal insufficiency on regular steroid supplementation (congenital n = 56, acquired n = 13, unclassified n = 1). General anesthesia (143 episodes) was classified as major surgery (procedure lasting >1 h, expected delay in ability to establish oral intake, n = 52) or minor surgery/imaging (lasting <1 h with oral intake expected immediately postsurgery, n = 40/51). Table 1 summarizes steroid dosing (mg/kg) at induction and postoperatively (average and range, grouped in comparison to recommendations from the proposed new guideline) and clinical complications. We included patients with adrenal insufficiency on regular steroid medication with available weight-based dosage calculations (n = 143). Large variabilities in steroid dosing were observed. In 20% of major, 27% of minor, and 66% of imaging only events no hydrocortisone was given at induction. Postoperatively, hydrocortisone was not administered in only one minor surgery event. In 56% of major, 65% of minor, and 95% of the imaging-only procedures low doses of hydrocortisone were prescribed (Table 1). Notably, in the imaging group no complications were noted; complications occurred in 5 major procedures with low, 1 with adequate dose and the minor event without hydrocortisone (4.8%). Five presented as hypotension unresponsive to fluid therapy; 1 with additional bradycardia, 1 with isolated hypernatraemia. Detailed data on procedures, comorbidities and additional regular medication could not be included in this report. Our data set provides a reasonable sample size in the context of a low incidence condition. The study is limited by not having explicitly looked at signs of steroid-overdosing. This would have been important for events with high hydrocortisone dosing and in particular for the imaging-only group as these are known to be at risk for high cumulative steroid doses.4, 5 This project highlights significant variability in perioperative stress steroid dosing in children with adrenal insufficiency. This was associated with a low incidence of adverse events. The true adverse event rate might be higher (reporting bias and retrospective study design). The data presented adds to the limited research and raises awareness of the issues associated with managing adrenal insufficiency perioperatively. The project highlights the importance of institutional guideline development for not only common, but rare conditions and also highlights the need for further audits in this field. The findings are intended to better understand and thus improve perioperative management of adrenal insufficiency in the pediatric population. On a departmental level, guideline implementation, and education were actioned, and this will be followed by re-audit to assess quality improvement. Cooperation and direct communication between endocrinologists and anesthetists involving patients and their families are essential. Furthermore, patient and family education needs to be updated on a regular basis and dosing recommendations need to be regularly reviewed. Accurate emergency and surgical management plans need to be provided to families and patients as well as considering school management plans. This project highlights that prospective studies are needed to provide pediatric evidence-based clinical guidelines for perioperative steroid dosing in adrenal insufficiency. Open access publishing facilitated by The University of Western Australia, as part of the Wiley - The University of Western Australia agreement via the Council of Australian University Librarians. BSvUS is part funded by the Stan Perron Charitable Foundation and through a National Health and Medical Research Council Investigator Grant (2009322). The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.