2016 FSA Posters

P041: A MULTIDISCIPLINARY PERIOPERATIVE MANAGEMENT OF A DIFFICULT AIRWAY IN A NEONATE WITH ROBINOW SYNDROME
Shazia Mohammad, MD, Sonia Mehta, MD, Sandra Gonzalez-Rodriguez, MD; University of Florida

Introduction: Robinow syndrome is a dwarfing syndrome presenting with malformations involving multiple systems where the more significant ones include craniofacial deformities, congenital heart defects, and genitourinary malformations. Characteristic facial features include marked midfacial hypoplasia, ankyloglossia, and micrognathia.1 Given the facial features described, patients are at increased risk of airway compromise of variable degree, which can be severe enough to require a surgical airway early in life.

Case: A 2-week old 2.9 kg neonate was born at 37 weeks gestational age; patient had stridor and desaturations, particularly with feeding.  Genetic testing confirmed Robinow syndrome.

Swallow study revealed aspiration, and a Nissen with gastrostomy tube placement was scheduled. Given his severe micrognathia, spontaneous ventilation was maintained with ketamine and sevoflurane during induction. Three attempts were made with C-mac video laryngoscopy.  Despite a grade II view, the endotracheal tube (ETT) was not advanced easily. A 3.0 uncuffed ETT was advanced into the trachea with extreme cricoid pressure, manipulation of stylet, and vigorous rotation at the ETT. The intraoperative course was uneventful and patient remained intubated postoperatively. Pediatric ENT recommended tracheostomy due to airway anatomy, but the neonate’s parents refused intervention. A creative multidisciplinary approach was taken to avoid tracheostomy in this patient. Plastic surgery created a lingual flap to advance the tongue forward in the patient’s mouth which was an effort to avoid obstruction post-extubation. The tongue flap dehisced which led to the tongue retracting back to its original posterior position in the neonate’s mouth. Patient remained intubated after the flap creation.  He was extubated five days later, but experienced airway obstruction and increased work of breathing. Three days later anesthesiology was called urgently to pediatric intensive care unit to secure the neonate’s airway. Initially neonate was maskable but unable to intubate despite multiple attempts with different tools. The patient then became unmaskable.  Pediatric ENT was emergently called to bedside.  A grade 3 view was obtained with a Cmac, and a 2.5 uncuffed ETT was passed. Due to persistent respiratory failure and extremely difficult airway, a tracheostomy was performed at 6 weeks of age.

Summary: Given the airway features of Robinow syndrome, adequate preparation to manage the airway is of the utmost importance.  Unlike many other difficult pediatric airways, patients with Robinow syndrome are always at risk of difficult intubation despite the age. In this patient with continued respiratory failure, a tracheostomy could have been done sooner to avoid the risks of further manipulation of a difficult airway. Moreover, it is important to have a plan amongst all specialties involved in case of emergency.

References:

1.Patton, M. A., & Afzal, A. R. (2002). Robinow Syndrome. J Med Genet, 39, 305-310.