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Adenotonsillectomy in children with sickle cell disease: a systematic review

Francesca Galluzzi1, Werner Garavello2

  • 1Department of Otorhinolaryngology, Fondazione IRCCS San Gerardo dei Tintori, Via Pergolesi, 33, 20900, Monza, MB, Italy.

American Journal of Otolaryngology
|August 20, 2026
PubMed

Insights

Adenotonsillectomy (ADT) offers benefits for children with sickle cell disease (SCD) and sleep apnea or infections. While improvements in apnea-hypopnea index were noted, careful management is crucial due to limited evidence and potential complications.

Area of Science:

  • Pediatric Otolaryngology
  • Pediatric Hematology
  • Sleep Medicine

Background:

  • Adenotonsillectomy (ADT) is frequently indicated for pediatric patients with sickle cell disease (SCD) experiencing obstructive sleep apnea (OSA) or recurrent adenoiditis and tonsillitis.
  • Despite its common use, there is a lack of comprehensive data evaluating the surgical outcomes of ADT in this specific high-risk population.

Purpose of the Study:

  • To systematically review and evaluate the surgical outcomes of adenotonsillectomy (ADT) in pediatric patients diagnosed with sickle cell disease (SCD).
  • To synthesize existing evidence on the efficacy and safety of ADT for indications such as obstructive sleep apnea (OSA) and recurrent tonsillitis/adenoiditis in children with SCD.

Main Methods:

  • A systematic review was conducted, adhering to PRISMA guidelines, by searching the PubMed and Embase electronic databases.
  • The review protocol was registered with PROSPERO, and a qualitative analysis of the included studies was performed.
  • Ten studies involving 454 pediatric patients with various SCD genotypes (HbSS, HbSC, HbS/β-thalassemia, HbSS-HPFH) were analyzed.

Main Results:

  • The primary indications for ADT were obstructive sleep apnea (OSA) and recurrent infections.
  • Significant improvements were observed in the apnea-hypopnea index (AHI), ranging from 50.56% to 82.90%, with mean hospital stays of 2-3.5 days.
  • Postoperative acute chest syndrome and emergency department visits decreased significantly; however, reductions in vaso-occlusive crises were not statistically significant. Surgical complications occurred in 5.2% to 11.3% of cases.

Conclusions:

  • Adenotonsillectomy (ADT) may confer clinical benefits for children with sickle cell disease (SCD), particularly for obstructive sleep apnea (OSA).
  • The current evidence is limited, necessitating cautious interpretation of outcomes and emphasizing the need for a multidisciplinary approach to manage potential perioperative and postoperative complications.
  • Further research is warranted to establish definitive guidelines for ADT in pediatric SCD patients.
Abstract

Keywords:
AdenotonsillectomyChildrenObstructive sleep apneaReviewSickle cell disease

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