Anesthesia

Pierre Robin Sequence (PRS) is a rare congenital condition of facial abnormalities, defined by a triad of micrognathia, retroglossoptosis and airway obstruction. PRS may have varied presentations due to associations with syndromes. Hence, the consensus in management remains elusive, with no definitive treatment protocols. We describe a case from a resource-‐ scarce setting that highlights the use of a less commonly performed surgical procedure which is simpler and cheaper than the gold-‐ standard surgery in PRS. An 18-‐ month-‐ old boy with PRS presented to A&E with airway obstruction and hypoxia due to retroglossoptosis. He was resuscitated immediately and intubated. Gold-‐ standard treatment was surgery: Mandibular Distraction Osteogenesis (MDO). However, as the patient was unable to afford the surgery, a cheaper procedure called tongue-‐ lip adhesion (TLA) was performed. The procedure involved pulling the base of the tongue anteriorly and tying to the hyoid bone. This maintained airway patency and patient was extubated. Mother was given feeding and child positioning advice. It is expected that the mandibular growth will eventually catch up with the tongue growth. The surgical procedures used to relieve airway obstruction in PRS include TLA, MDO and tracheostomy. In this case, TLA was chosen due to affordability issues. Three different TLA techniques, previously described in the literature for PRS, were discussed. Given the nutritional status of the patient, we decided to avoid extensive dissection. We required a technique that would not restrict mobile segments of the tongue, to allow for normal speech development and feeding. In addition, sutures on the tongue should not be damaged by biting, in the teething child. Lapidot and Ben-‐ Hur technique (briefly described in the case) satisfied above-‐ mentioned requirements and was hence chosen. Overall, this case is of great value in exploring different surgical techniques for PRS management, not widely explained in the literature.