AIRWAY MANAGEMENT IN 7-WEEKS-OLD INFANT WITH PIERRE ROBIN SYNDROME AND CONGENITAL PYLORIC STENOSIS

Abstract

Pierre Robin Syndrome (PRS) is characterized by a sequence of events including mandibular hypotrophy (micrognathia), abnormal posterior placement of tongue (glossoptosis), and airway obstruction. Pyloric Stenosis on the other hand is the most common infant surgical condition which presents with episodes of projectile vomiting leading to dehydration and weight loss. Airway management in these patients is a true challenge for every anesthesiologist. The patient was 7 weeks old infant, weighted 3,1 kg, admitted in Intensive Care Department for surgical repair of pyloric stenosis, previously diagnosed with Pierre Robin Syndrome at birth. Preoperative preparation, intravenous rehydration and electrolyte substitution was obtained. Video laryngoscope was used for management of difficult airway. We had many attempts in visualization of the vocal cords, eventually we performed awake intubation with stylet uncuffed endotracheal tube size 3. Pyloromyotomy was performed. Maintenance of anesthesia was with Sevoflurane and bolus doses of Fentanyl as adjunct. Perioperative vital signs were within normal ranges. Awake extubation was performed. The facial malformation that appears in patients with Pierre Robin Syndrome makes visualization of the glottis extremely difficult to impossible. In cases where tracheal intubation is needed, awake fiberoptic intubation is recommended, but it can have many limitations. These two conjoined conditions present the quandary of safely managing an expected difficult airway in an uncooperative patient. With this case we can conclude that for children with Pierre Robin syndrome, video laryngoscopy should be considered as a first attempt intubation device both in the operating room and for emergent situations.