Obstructive Sleep Apnea in Hurlers Syndrome: Polysomnographic Evidence of 58 Patients

Monday, October 19, 2026

11:54 AM - 12:00 PM PT

Location: 409AB

Presenting Author(s)

Evan N. Herschi, BA

Medical Student
University of Minnesota Medical School, Masonic Children's Hospital
Prior Lake, MN, United States

Senior Author(s)

LJ

Luke A. Jakubowski, MD

Dr
Department of Otolaryngology, University of Minnesota, Children's Minnesota Hospital, MN, USA
Minneapolis, MN, United States

Co-Author(s)

TD

Thamiris Dias Delfino Cabral, MD

Dr
University of Minnesota Departent of Otolaryngology, Head and Neck Surgery
minneapolis, MN, United States
PO

Paul Orchard, MD

MD
Department of Hematology, University of Minnesota, MN, USA, United States
Lauren Buchholz, BA

Medical Student
University of Minnesota, Children's Minnesota Hospital
Minneapolis, Minnesota, United States

Disclosure(s):

Evan N. Herschi, BA:

faculty for this accredited education activity has no relationship(s) with ineligible companies to disclose.

Introduction: Hurler syndrome (HS) is a rare lysosomal storage disorder characterized by progressive glycosaminoglycan deposition, leading to upper airway obstruction and obstructive sleep apnea (OSA). We evaluated OSA burden and airway outcomes following adenoidectomy-alone (AA), adenoidectomy and tonsillectomy (AT), or either airway surgery in the largest pediatric HS cohort to date.

Methods: A retrospective database study of HS patients evaluated at a tertiary center between 2000 and 2024 was performed. Demographics, polysomnograms (PSG), and airway surgery data were collected. Outcomes included change in apnea-hypopnea index (AHI), mean oxygen saturation (SpO2), and SpO2 Nadir. Nonparametric and exact tests were used for subgroup analysis, and exploratory linear regression evaluated predictors of postoperative AHI change.

Results: Among 134 pediatric HS patients, 90 underwent sleep evaluation, including 58 with formal PSG (28 female, 30 male). Baseline OSA grade was absent (8.62%), mild (41.38%), moderate (18.97%), and severe (31.03%). At first PSG, 6 patients had prior AT, 11 AA, and 41 neither. Median baseline AHI was 2.1 (95% CI 0.3-17.2) in prior AT, 3.6 (95% CI 0.8-6.4) in prior AA, and 5.5 (95% CI 2.1-14.3) without prior surgery. Mean age at AT was 66.9 months (SD 42.71), AA 41.5 months (SD 45.78), or either airway surgery 55.33 (SD 45.91). Paired pre- and postoperative PSGs had improvement in AHI and thus OSA grade. AT demonstrated significant median AHI reduction of –9.8 (95% CI –16.2 to –3.4, p< 0.05), whereas AA had no significant change (–2.1 median; 95% CI -5.4-1.2, p>0.05). Mean SpO2 increased modestly after AA (+0.9%, p>0.05), with variable changes in SpO2 nadir (p>0.05). Higher baseline AHI predicted greater postoperative improvement (β=-0.83, 95% CI –1.31 to –0.36, p=0.0002); baseline SpO2 was not predictive.

Conclusions: OSA is common in HS and improves following airway surgery, particularly after AT. Greater baseline OSA severity predicts larger postoperative improvement, supporting surgical intervention in selected patients. Subgroup analyses were limited by small sample sizes.