Our Summary

This research paper discusses the case of a 9-year-old boy suffering from Crouzon syndrome, a very rare condition that affects the growth of the skull. This can cause deformities in the face and nose, which in this case resulted in sleep apnea, a disorder that causes breathing to stop and start during sleep.

The boy had been snoring during sleep for six years and his condition had worsened significantly over the past month. The doctors diagnosed him with severe sleep apnea and low oxygen levels in his blood due to the Crouzon syndrome.

The doctors performed a surgery to remove his adenoids (lymph tissue in the throat) and put him in the pediatric intensive care unit with a ventilator to help him breathe. Initially, he had trouble maintaining his blood oxygen levels after trying to remove the breathing tube and had to be put back on the ventilator. However, after further treatment, they were able to successfully remove the tube.

Three months after the surgery, his nasal wound healed well and his sleep improved significantly. The researchers concluded that for patients like this boy, it might be beneficial to use the ventilator for a longer period after surgery and to pay more attention to managing the airway during and after surgery to reduce the risk of complications.

FAQs

  1. What is Crouzon syndrome and how does it affect the patient’s sleep?
  2. What interventions were taken for the 9-year-old boy diagnosed with Crouzon syndrome and severe obstructive sleep apnea?
  3. What were the outcomes after the adenoidectomy and what lessons were learned from the case?

Doctor’s Tip

A doctor might advise a patient undergoing adenoidectomy to follow postoperative care instructions carefully, including staying hydrated, avoiding strenuous activities, and taking prescribed medications as directed. They may also recommend keeping the nasal passages clear by using a saline nasal spray and avoiding irritants such as smoke. It is important to follow up with the doctor for any concerns or complications that may arise after the procedure.

Suitable For

Patients with severe obstructive sleep apnea, especially those with craniofacial dysplasias such as Crouzon syndrome, are typically recommended adenoidectomy. Adenoidectomy can help improve airflow in the nasal cavity and posterior nasal meatus, reducing the severity of sleep apnea and improving overall sleep quality. It is important to carefully monitor these patients postoperatively, as they may be at higher risk for complications such as hypoxemia. Strengthening perioperative airway management and prolonging the time of ventilator-assisted breathing may help reduce the risk of postoperative complications in these patients.

Timeline

  • Before adenoidectomy:

    • Patient experiences sleep snoring for 6 years
    • Progressive aggravation in the past 1 month
    • Accompanied by apnea during sleep
    • Diagnosis of Crouzon syndrome complicated with severe obstructive sleep apnea and severe hypoxemia

  • After adenoidectomy:

    • Admitted to pediatric intensive care unit with ventilator-assisted respiration
    • Blood oxygen saturation fluctuates greatly
    • Difficulty maintaining blood oxygen after extubation, requiring re-intubation
    • Successful extubation after active treatment
    • Wound of nasopharynx recovers well
    • Sleep state significantly improved 3 months postoperation

Lessons learned:

  • Prolonged time of ventilator-assisted breathing may be necessary
  • Strengthen perioperative airway management to reduce risk of postoperative complications.

What to Ask Your Doctor

  1. What are the risks and benefits of adenoidectomy for my child with Crouzon syndrome?
  2. What is the expected recovery time after the surgery?
  3. How will the surgery affect my child’s breathing and sleep patterns?
  4. What measures will be taken to ensure proper airway management during and after the surgery?
  5. What are the potential complications that could arise from the surgery?
  6. Will my child need any additional treatments or interventions following the adenoidectomy?
  7. How will the adenoidectomy impact my child’s overall quality of life and long-term health outcomes?
  8. Are there any specific precautions or lifestyle changes my child should follow post-surgery?

Reference

Authors: Yu L, Zhao Y. Journal: Medicine (Baltimore). 2024 Jun 7;103(23):e38534. doi: 10.1097/MD.0000000000038534. PMID: 38847734