Parental perceptions and morbidity: tracheostomy and Pierre Robin sequence.

OBJECTIVE: Evaluate parental perceptions associated with tracheostomy morbidity and quality of life in the management of Pierre Robin Sequence (PRS).

STUDY DESIGN: Retrospective review/survey.

METHODS: 42 Pierre Robin patients were identified, records were reviewed and airway assessments evaluated relative to airway compromise. Twenty patients had undergone tracheostomy. Perceptions of quality of life/morbidity related to tracheostomy were assessed using parental surveys.

RESULTS: 31/41 (76%) patients participated in the survey. 15/31 (48%) of survey participants required tracheostomy and were decannulated after a mean of 28 months. Of the patients who had undergone tracheostomy, 10/15 (67%) had isolated Pierre Robin (iPRS) and the remaining 5/15 (33%) had syndromes associated with Pierre-Robin (sPRS). 9/10 (90%) iPRS and 4/5 (80%) sPRS families' expectations were met regarding expected duration of tracheostomy although 3/5 (60%) sPRS, and 8/10 (80%) iPRS described the overall experience as difficult. Of the 2/15 patient's families who were dissatisfied 1 patient had iPRS and the other sPRS. 9/15 (60%) required multiple > or = 3) hospitalizations. 3/13 (23%) reported airway problems after decannulation and 2/15 (13%) remained tracheostomy dependent at the time of survey. Prolonged tracheostomy duration represented a significant parental concern.

CONCLUSIONS: A subset of patients required extended duration of tracheostomy; some continued to have airway problems after decannulation and/or distraction. Although some patients benefit from early mandibular distraction other Pierre Robin patients have multi-level obstruction requiring additional therapies and often tracheostomy. Parental concerns and perceptions relative to tracheostomy have not been adequately studied for Pierre Robin airway obstruction. Of those responding to this survey, the majority of parents' expectations were met regarding tracheostomy. Of those whose expectations were not met, it seems that better pre-intervention counseling regarding length of tracheostomy tube dependence, as well as a discussion about potential complications and hospitalizations frequently associated with prolonged tracheostomy, may lead to improved parental expectations.