Our Summary

Mucopolysaccharide (MPS) diseases are a group of inherited diseases where the body accumulates partially broken-down complex sugars, causing a range of problems including sleep apnea due to obstructed airways. Traditionally, surgery involving removal of the adenoids and tonsils has been used to improve sleep apnea in MPS patients, but this can be difficult due to issues such as limited neck movement and a small mouth opening.

This study reviewed cases of MPS patients who had adenoid surgery to treat sleep apnea between June 2015 and March 2017. The surgeries were performed using a less invasive method that involves going through the nose guided by a small camera (endoscopy). The tools used were either a microdebrider, which cuts and sucks away tissue, or a Coblation wand, which uses radiofrequency energy to remove tissue.

Nine patients were included in the study, with ages ranging from 3 to 14. After surgery, sleep study data showed that six out of eight patients saw improvements in their sleep apnea. All parents reported improvement in their children’s sleep apnea symptoms after the surgery. There were no complications reported and no risk factors identified for persistent sleep apnea after surgery.

The study concludes that this less invasive adenoidectomy method is a safe and effective treatment for sleep apnea in MPS patients. These techniques offer a new way to manage enlarged adenoids when traditional surgery is not possible, or to remove recurring disease that would be difficult to remove with traditional surgery.

FAQs

  1. What are Mucopolysaccharide (MPS) diseases and how do they relate to sleep apnea?
  2. How is the less invasive adenoidectomy method performed to treat sleep apnea in MPS patients?
  3. What were the results of the study on the effectiveness of the less invasive adenoidectomy method in treating sleep apnea in MPS patients?

Doctor’s Tip

The doctor may suggest to the patient that this less invasive method of adenoidectomy using endoscopy and specialized tools could be a safe and effective option for treating sleep apnea caused by enlarged adenoids. This technique may be particularly beneficial for patients with conditions like MPS, where traditional surgery may be challenging. It is important for the patient to discuss this option with their doctor to determine if it is the right choice for them.

Suitable For

Overall, patients with Mucopolysaccharide diseases who have sleep apnea due to obstructed airways are typically recommended adenoidectomy. This less invasive method using endoscopy and tools like a microdebrider or Coblation wand can be a safe and effective option for improving sleep apnea symptoms in these patients.

Timeline

Overall timeline for a patient before and after adenoidectomy:

Before surgery:

  • Patient experiences symptoms of obstructive sleep apnea, such as snoring, difficulty breathing during sleep, and daytime fatigue.
  • Patient undergoes evaluation by a healthcare provider, which may include a sleep study to diagnose sleep apnea.
  • Surgery is recommended as a treatment option for the patient’s sleep apnea.
  • Patient and their family discuss the risks and benefits of surgery with the healthcare team.

After surgery:

  • Patient undergoes adenoidectomy using a less invasive method guided by endoscopy.
  • Patient is monitored in the recovery room following surgery.
  • Patient may experience some pain and discomfort in the throat area after surgery.
  • Patient is discharged home with instructions for post-operative care, including pain management and follow-up appointments.
  • Patient’s sleep apnea symptoms improve, as reported by both sleep study data and the patient’s family.
  • Patient and their family report overall improvement in quality of life due to the successful treatment of sleep apnea.

Overall, the patient’s experience before and after adenoidectomy involves a comprehensive evaluation, surgical intervention, and post-operative care to improve symptoms and quality of life related to sleep apnea.

What to Ask Your Doctor

  1. What are the potential risks and benefits of undergoing adenoidectomy surgery for my child with MPS?
  2. How will the surgery be performed, and what techniques will be used?
  3. How long is the recovery period expected to be, and what post-operative care will be required?
  4. Will this surgery completely resolve my child’s sleep apnea, or are there potential risks for persistent symptoms?
  5. Are there any alternative treatments or therapies that could be considered for managing my child’s sleep apnea?
  6. How frequently will follow-up appointments be needed after the surgery, and what will be monitored during these visits?
  7. Are there any specific precautions or considerations that need to be taken into account for my child with MPS undergoing adenoidectomy surgery?
  8. Can you provide information about the success rates of this less invasive adenoidectomy method in MPS patients, based on your experience or previous cases?
  9. Are there any specific lifestyle changes or accommodations that should be made to help manage my child’s sleep apnea after the surgery?
  10. Is there any additional information or resources that you recommend for educating myself and my family about adenoidectomy surgery for MPS patients with sleep apnea?

Reference

Authors: Harrison R, Schaefer S, Warner L, Mercer J, Jones S, Bruce I. Journal: Int J Pediatr Otorhinolaryngol. 2018 Aug;111:149-152. doi: 10.1016/j.ijporl.2018.04.028. Epub 2018 May 1. PMID: 29958599