Children with mucopolysaccharidosis Undergoing Adenotonsillectomy have Increased Postoperative Complications: Study

MPS is a collection of rare genetic conditions stemming from the failure of the body to metabolize glycosaminoglycans, leading to systemic manifestations such as abnormalities of airways. Adenotonsillectomy, a routine surgery done to excise the tonsils and adenoids, is more risky in children with compromised airways, i.e., in children with MPS. In order to clarify the burden and risks of this surgery in children with MPS, investigators used three editions (2009, 2012, and 2016) of the Healthcare Cost and Utilization Project Kids' Inpatient Database (HCUP KID) that comprised a combined total of 24,700 pediatric AT cases, including 40 children with MPS.

The authors performed a retrospective comparison of children with MPS (n = 40) and children without MPS (n = 24,660)** who received AT. Outcomes included respiratory complications, hemorrhage after AT, hospital stay, and overall hospital charges. Comparisons also were made of demographic variables, and statistical adjustment was made to control for potential confounding variables like sex.

Key Findings

The outcomes showed that MPS children had far poorer surgical results than their counterparts:

• Respiratory complications arose in 15% of patients with MPS (6 out of 40) versus 2.4% of children without MPS (586 out of 24,660), with sex-adjusted odds ratio 6.88 (95% CI: 2.87–16.46; P < 0.001), reflecting almost sevenfold increased risk.

• Postoperative hemorrhage occurred in 10% of MPS children (4 out of 40) versus 1.8% in the non-MPS children (444 out of 24,660), adjusted odds ratio 5.97 (95% CI: 2.12–16.86; P < 0.001).

• The median hospital stay for MPS children was 3 days (IQR 1–4), compared to 1 day (IQR 1–2) in the non-MPS group (P < 0.001).

• Median hospital costs were significantly higher in children with MPS: $33,016 (IQR $23,208.50–$72,280.50) versus $15,383 (IQR $9937–$24,462) for non-MPS children (P < 0.001).

• Children with MPS were also disproportionately male (P < 0.017), although this was controlled for in the statistical modeling.

Children with mucopolysaccharidosis who receive adenotonsillectomy are at higher risk for respiratory complications and perioperative bleeding, have longer lengths of stay, and have higher healthcare costs compared to children who do not have MPS. These results emphasize the necessity for personal surgical planning and increased perioperative care in this group in an effort to enhance outcomes and decrease healthcare burdens.

Reference:

Elwell, Z., Mancuso, D., Wolter, N.E. et al. Post-tonsillectomy outcomes in children with mucopolysaccharidosis and obstructive sleep apnea. J of Otolaryngol - Head & Neck Surg 52, 87 (2023). https://doi.org/10.1186/s40463-023-00685-y