Adenotonsillectomy in children with sickle cell disease: a systematic review.

OBJECTIVE: Adenotonsillectomy (ADT) is a common indication for children with sickle cell disease (SCD) and comorbid obstructive sleep apnea (OSA) or recurrent infections (adenoidits and tonsillitis), despite the paucity of data in this population. This review aims to evaluate the surgical outcomes of ADT in pediatric patients with SCD. METHODS: We conducted a systematic review by searching the PubMed and Embase electronic databases. The PRISMA guidelines were followed, and the review protocol was registered in PROSPERO. A qualitative analysis was performed. RESULTS: Ten studies, comprising a total of 454 subjects, were included. The mean age ranged from 6 to 12 years, with a comparable sex distribution between groups. The combined cohort consisted of 303 cases of HbSS, 24 of HbSC, 16 of HbS/β-thalassemia, and 3 cases of HbSS-HPFH. Baseline hemoglobin (Hb) levels ranged from 6.7 to 9.3 g/dL, while mean Hb levels at the time of surgery varied between 8.9 and 11.4 g/dL. The proportion of patients receiving preoperative transfusions ranged from 72% to 100%. Follow-up duration spanned from 1 month to 1 year. The primary surgical indications were OSA and recurrent infections. Regarding surgical outcomes, the improvement in the AHI ranged from 50.56% to 82.90%, with mean hospital stays of 2 and 3.5 days. Postoperative acute chest syndrome and emergency department visits decreased significantly, whereas the reduction in vaso-occlusive crises was not significant. Finally, surgical complications were reported in 5.2% to 11.3% of cases. CONCLUSION: ADT may provide clinical benefits for children with SCD. However, given the limited evidence, outcomes must be interpreted with caution, and a multidisciplinary approach is required to manage potential complications.