Management of infants with Pierre Robin sequence

• Published in: The Cleft palate-craniofacial journal 2003-03, Vol.40 (2), p.180-185
• Main Authors: WAGENER, Silke, RAYATT, Sukh S, TATMAN, Andy J, GORNALL, Peter, SLATOR, Rona
• Format: Article
• Language: English
• Subjects: Airway Obstruction - etiology; Airway Obstruction - therapy; Biological and medical sciences; Complex syndromes; Dentistry; Female; Head and neck surgery. Maxillofacial surgery. Dental surgery. Orthodontics; Humans; Infant, Newborn; Intubation, Gastrointestinal; Intubation, Intratracheal; Male; Medical genetics; Medical sciences; Nasopharynx; Nutrition Disorders - etiology; Nutrition Disorders - therapy; Oximetry; Pierre Robin Syndrome - complications; Pierre Robin Syndrome - therapy; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the upper aerodigestive tract
• ISSN: 1055-6656; 1545-1569
• DOI: 10.1597/1545-1569(2003)040<0180:MOIWPR>2.0.CO;2

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). 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. 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. Outcome measures were oxygen saturation, growth of the babies, and the need for surgery. All babies were managed successfully with an NPA and nutritional support. No baby required surgery, and the majority showed good weight gain. 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.