Stickler syndrome is an autosomal-dominant disorder caused by mutation of pro-collagen genes and characterized by bone malformation, eye disease, mandibular micrognathia, and cleft palate in the oral and maxillofacial region. Palatoplasty is associated with the risk of difficulty of intubation because of the small jaw and airway obstruction caused by postoperative edema during the peri-surgical period.
Here, we describe the case of a 4-month-old girl with Stickler syndrome with a past history of being intubated and sedated because of an airway obstruction caused by glossoptosis and laryngomalacia who was intubated and sedated after palatoplasty. To prevent complications, in consensus with pediatricians and anesthesiologists, the patient was managed postoperatively with intubation and sedation in the pediatric intensive care unit. Evaluation of the level of swelling and extubation was performed in 2 days. She was discharged from the hospital with no complications 7 days post-surgery as is done in usual cases of palatoplasty. We consider that the combined use of intubation and sedation is effective in preventing peri-operative complications after palatoplasty in a patient with Stickler syndrome.
