The Science Journal of the American Association for Respiratory Care

2012 OPEN FORUM Abstracts

TREATMENT OF SEVERE OBSTRUCTIVE APNEA AND MICROGNATHIA WITH PLACEMENT OF AN INTERNAL JAW IN A NEWBORN.

Leane Soorikian1, Janet Lioy2, Steven Sobol3, Jesse Taylor4, Joanne Stow3, Natalie Napolitano1; 1Respiratory Care Department, The Children’s Hospital of Philadelphia, Philadelphia, PA; 2Neonatology, The Children’s Hospital of Philadelphia, Philadelphia, PA; 3Otorhinolaryngology, The Children’s Hospital of Philadelphia, Philadelphia, PA; 4Plastic Reconstructive Surgery, The Children’s Hospital of Philadelphia, Philadelphia, PA

Introduction: infants with severe apnea as a result of micrognathia were traditionally treated with a tracheostomy and a wait-and-grow plan of care. Technology dependent patients have their own sets of challenges and place significant financial and time burdens on the family. Select children’s hospitals now offer an alternative to a tracheostomy in mandibular distraction osteogenesis. Case Summary: The patient is a term infant with a fetal diagnosis of micrognathia/retrognathia. At birth, the condition was found to be complicated by a cleft palate. The infant was born at an outlying hospital and transferred to CHOP for evaluation of increased respiratory distress with stridor and retractions when supine. The infant was kept in the prone position until workup was complete and plan of care decided. A 16-channel sleep study was performed and showed severe obstructive apnea with a hypopnea index in the 120’s; patient was placed on CPAP of 5 and ultimately NIMV via RAM cannula through the Evita XL ventilator to control the apnea. A microlaryngoscopy and bronchoscopy was performed prior to surgery which revealed severe tongue-based upper airway obstruction with no lower anomalies. Internal mandibular distracters were surgically placed. The distracters were turned 3 times daily by nursing staff to allow separation of the jaw and osteogenesis for formation of a traditional size mandible. The infant remained intubated for 1 week after surgery and was extubated to room air without any complications. Discussion: This infant’s case was complicated by the mother’s ability to care for the child after discharge. At the time of treatment mom was homeless and living in a shelter. The internal jaw was determined the best treatment option for long term care in the unknown home environment. The internal jaw is less visible but does have the limitations of only being able to move the jaw in one direction and for a definitive additional length (under 25 mm) and can require more significant surgery for removal. This infant’s obstructive apnea was clinically resolved after 1 week and now requires no pulmonary support. This patient is being followed by the neonatal multidisciplinary critical airway team to optimize care and outcomes. Sponsored Research - None