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The Role of Airway Management on Feeding Difficulties in Children With Pfeiffer Syndrome
Joseph Salem1, Wendy Blumenow2, Anne Markey1
1Department of Paediatric ENT Surgery, Alder Hey Children's NHS Foundation Trust.
Insights
Children with Pfeiffer syndrome needing airway support experience feeding issues. Airway adjuncts, especially nasopharyngeal airways (NPAs), may worsen these feeding difficulties, requiring intervention.
Area of Science:
- Pediatric craniofacial anomalies
- Airway management in children
- Pediatric feeding disorders
Background:
- Pfeiffer syndrome presents with craniosynostosis, mid-face hypoplasia, and airway obstruction.
- Airway management, including positive airway ventilation, nasopharyngeal airway (NPA), or tracheostomy, is frequently necessary.
Purpose of the Study:
- To evaluate the effect of airway adjuncts on feeding difficulties in children with Pfeiffer syndrome.
Main Methods:
- Retrospective review of 11 Pfeiffer syndrome patients from 1998-2020 at Alder Hey Children's Hospital.
- Data collected from Speech & Language Therapy and medical notes.
- Oral Feeding Score from the UK Craniofacial Outcome Score was utilized.
Main Results:
- Six patients (55%) did not require airway adjuncts; 3 had tracheostomy (27%) and 2 had NPA (18%).
- Patients with airway adjuncts required percutaneous endoscopic gastrostomy/jejunostomy feeding.
- Mean Oral Feeding Score was 4.60 (no adjunct), 2.4 (pre-airway adjunct), 2.0 (NPA group post-adjunct), and 3.0 (tracheostomy group post-adjunct).
Conclusions:
- Children with Pfeiffer syndrome requiring airway intervention exhibit more severe feeding problems.
- Airway adjuncts, particularly NPAs, may contribute to feeding difficulties in this population.
Background:
Pfeiffer syndrome is characterized by craniosynostosis, mid-face hypoplasia, broad thumbs, and often multilevel airway obstruction. Airway management is often required, including the use of positive airway ventilation, nasopharyngeal airway (NPA), or tracheostomy.
Objective:
The objective of this study was to assess the impact an airway adjunct can have on feeding difficulties in children with Pfeiffer syndrome.
Methods:
Retrospective review of patients diagnosed with Pfeiffer syndrome from January 1998 to January 2020 at one of England's 4 supraregional Craniofacial Units, Alder Hey Children's Hospital. Speech & Language Therapy case notes and medical notes were used to gather data, as well as the Oral Feeding Score component of the UK Craniofacial Outcome Score.
Results:
Eleven patients were included. Six patients had no airway adjunct (55%): 3 had tracheostomy (27%) and 2 patients had NPA (18%). All patients with airway adjuncts were percutaneous endoscopic gastrostomy/percutaneous endoscopic jejunostomy fed. Those who did not require an airway adjunct had an Oral Feeding Score of 4.60 (SD: 0.49). The children who went on to have an airway adjunct had a mean preintervention Oral Feeding Score of 2.4 (SD: 0.8). The mean feeding score (postairway adjunct) in the NPA group was 2.0, compared with the tracheostomy group scoring 3.0.
Conclusions:
Children with Pfeiffer syndrome who require airway intervention have more significant feeding problems requiring feeding intervention. Although there were small numbers included in this study, there is a suggestion that airway adjuncts can contribute to feeding difficulties, particularly NPAs.
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