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Treatment of otitis media with effusion in children with mucopolysaccharidoses
M Motamed1, S Thorne, A Narula
1Department of Otolaryngology Head and Neck Surgery, Leicester Royal Infirmary, UK.
Insights
Children with mucopolysaccharidoses (MPS) often have otitis media with effusion (OME) and difficult airways. For managing OME in MPS patients, long-term ventilation tubes or hearing aids are preferred over short-term options to reduce anesthetic risks.
Area of Science:
- Pediatric Otolaryngology
- Genetics and Rare Diseases
- Anesthesiology
Background:
- Otitis media with effusion (OME) is a consistent finding in children diagnosed with mucopolysaccharidoses (MPS).
- Children with MPS often present anesthetic challenges due to potential airway difficulties.
- Management of OME in this population requires careful consideration of both hearing and anesthetic risks.
Purpose of the Study:
- To review the management of OME in patients with MPS over a seven-year period.
- To evaluate the effectiveness of different OME treatment strategies in children with MPS.
- To determine optimal interventions for OME in MPS patients to minimize anesthetic risks.
Main Methods:
- A retrospective review of medical records for patients diagnosed with MPS and OME.
- Analysis of the number and type of ventilation tube insertions and hearing aid usage.
- Assessment of hearing loss and compliance with hearing aids post-diagnosis.
Main Results:
- Nine patients with MPS and OME were identified, all requiring multiple short-term ventilation tube insertions prior to MPS diagnosis.
- Following MPS diagnosis, three patients needed repeated short-term tubes, while four received single long-term tube insertions.
- Five children with hearing loss were fitted with hearing aids, but compliance was an issue in two cases.
Conclusions:
- For children with MPS and OME, long-term ventilation tubes or hearing aids (if compliant) are superior to short-term tubes post-diagnosis.
- These interventions aim to minimize the significant anesthetic risks associated with repeated procedures in MPS patients.
- A 'watch and wait' approach for OME management in children with MPS is not recommended due to potential complications.
Abstract:
Otitis media with effusion (OME) is a constant finding in children with mucopolysaccharidoses (MPS). Affected children may also present the anaesthetist with a difficult airway. A 7-year retrospective review of the management of OME in individuals with MPS was carried out. Nine patients were identified. All had a number of short-term ventilation tube insertions (one to four, mean two) before a diagnosis of MPS was made. Following diagnosis three required repeated short-term ventilation tubes insertions (two to four, mean three), four had long-term ventilation tube insertions once only. Five children who had residual hearing loss were provided with hearing aids but compliance was poor in two. Once a diagnosis of MPS has been made, a hearing aid, if compliant, or a long-term ventilation tube would be a better option than a short term one in order to minimise the anaesthetic risk. A 'watch and wait' policy is not recommended.
