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Transnasal adenoidectomy in mucopolysaccharidosis
Rebecca Harrison1, Simone Schaefer1, Laura Warner1
1Royal Manchester Children's Hospital, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
Summary
Transnasal adenoidectomy using microdebrider or Coblation effectively treats obstructive sleep apnea (OSA) in Mucopolysaccharidosis (MPS) patients with difficult airways. This safe endoscopic approach improves OSA symptoms and oxygenation, even with challenging anatomy.
Area of Science:
- Otolaryngology
- Pediatric Surgery
- Metabolic Disorders
Background:
- Mucopolysaccharidosis (MPS) diseases cause GAG accumulation, leading to airway obstruction and obstructive sleep apnea (OSA).
- Conventional adenoidectomy for OSA in MPS patients is challenging due to restricted neck extension, macroglossia, and limited mouth opening.
- Endoscopic transnasal techniques offer an alternative for airway management in these patients.
Purpose of the Study:
- To evaluate the efficacy and safety of transnasal microdebridement and radiofrequency plasma ablation (Coblation) adenoidectomy for OSA in MPS patients.
- To assess the impact of these techniques on OSA severity and surgical complications.
- To explore the application of endoscopic techniques for adenoid hypertrophy in patients with restricted airway access.
Main Methods:
- Retrospective case review of MPS patients undergoing transnasal adenoidectomy (microdebrider or Coblation) for OSA between June 2015 and March 2017.
- Nasendoscopic guidance was used for all transnasal procedures.
- Primary outcome: OSA improvement via sleep oximetry and parental report; Secondary outcomes: surgical complications and risk factors for persistent OSA.
Main Results:
- Nine MPS patients (mean age 9 years) underwent the procedure.
- Six of eight patients showed improved oxygen desaturation index (ODI-4) post-surgery (mean ODI-4 decreased from 8.11 to 4.99).
- All parents reported improvement in OSA-related symptoms, with no identified risk factors for persistent OSA or surgical complications.
Conclusions:
- Transnasal Coblation and microdebrider adenoidectomy are safe and effective for treating OSA in MPS patients with adenoidal hypertrophy.
- These endoscopic techniques provide a viable surgical option for adenoidectomy when transoral access is limited or for debulking recurrent disease.
- Optimizing airway management, including novel endoscopic approaches, is crucial for the increasing lifespan of MPS patients.
