Management of Infants with Pierre Robin Sequence

Abstract

Objective Several methods of treating babies with Pierre Robin sequence have been described since the condition itself was first documented in 1923. The main aim of treatment has been to relieve upper airway obstruction. Treatment methods used range from positioning of the baby to invasive surgery. The aim of this article was to describe the assessment, treatment, and monitoring methods used for babies referred with Pierre Robin sequence (PRS). Setting/Patients From December 1995 to May 2000, 22 consecutive patients were admitted to Birmingham Children's Hospital with PRS. Their airway and nutritional status were assessed and continuously monitored. Interventions Treatment concentrated on the relief of airway obstruction with a nasopharyngeal airway (NPA) and nutritional support of the babies until they grew out of their respiratory and feeding difficulties. Main Outcome Measures Outcome measures were oxygen saturation, growth of the babies, and the need for surgery. Results All babies were managed successfully with an NPA and nutritional support. No baby required surgery, and the majority showed good weight gain. Conclusion Relieving airway obstruction by NPA is an effective and safe treatment for babies with PRS until they have grown out of their respiratory and feeding difficulties. It avoids the need for surgery and can be used on neonatal wards using the monitoring described.

Pierre Robin sequence: An institutional experience in the multidisciplinary management of airway, feeding and serous otitis media challenges

“…Other authors have reported the success of non-invasive methods [12][13][14]. Wagener et al showed that in all cases in their series of 22 patients, a NPA provided adequate airway management [15].…”

Percutaneous gastrostomy tubes in children with Pierre Robin sequence: efficacy, maintenance and complications

“…Children with PRS have both respiratory and feeding difficulties of varying levels of severity [7]; both play a role in determining the severity of their clinical presentation. Failure to thrive has been shown to be strongly correlated with the severity of airway obstruction.…”

A rudimentary epiglottis associated with Pierre Robin sequence

“…Several methods of treating babies with Pierre Robin sequence have been described since the condition was first documented 9 . The main aim of treatment is to provide relief from the upper-airway obstruction, with attention to feeding the second most-important factor.…”