Respiratory management of Pierre Robin sequence using nasopharyngeal airway with Kirschner wire☆

Summary 

Background

The Pierre Robin sequence (PRS) is a relatively rare symptom complex characterised by glossoptosis, micrognathia and respiratory obstruction. The initial problem that children with PRS face is obstructive dyspnoea, which can result in death without appropriate respiratory management. We designed and used a modified airway with a Kirschner wire (K-airway) in children with PRS who suffered from dyspnoea that did not improve with conservative treatment.

Methods

The subjects were four children diagnosed with PRS at the Department of Plastic Surgery, Shizuoka Children's Hospital, from February 2007 to December 2008. Since dyspnoea was not improved by conservative treatment, a φ0.8-mm Kirschner wire was set inside a nasopharyngeal airway bent in a form to lift the root of the tongue in order to prevent glossoptosis. The respiratory condition was evaluated with a test for sleep apnoea.

Results

Successful improvement in dyspnoea with the K-airway was noted in all cases. In Case 1, the subject was discharged from hospital without using the K-airway (92 days of age). In Case 2, the subject was discharged from hospital using the airway only at nighttime (122 days of age).

Conclusions

This method is safe because it is less invasive, and its effects can be easily evaluated, suggesting that it is a good method to try prior to surgical treatment.

Keywords: Pierre Robin sequence, Respiratory management, Airway, Kirschner wire