A Beckwith-Wiedemann Syndrome Patient Who Needed Special Respiratory Care after Tongue Reduction

Beckwith-Wiedemann syndrome (BWS) is characterized by exomphalos, macroglossia, and gigantism. Macroglossia is observed in 97.5% of BWS cases. Here we report the case of a patient with BWS who needed respiratory management following tongue reduction. An 18-year-old man with macroglossia was referred to our department due to concerns of relapse after orthodontic treatment. He had a history of tongue reduction in infancy, and recurrent macroglossia was observed with growth. The patient underwent a tongue reduction following diagnosis of macroglossia. However, immediately after surgery, obstruction of the upper airway due to swelling of the tongue was noted and it was decided to re-intubate the patient. As swelling improved on the second day of surgery, extubation was performed and a nasal airway was inserted. The nasal airway was extubated on the third day of surgery. No particular respiratory problems were observed. Oral ingestion was initiated on the fourth day. In cases of tongue reduction, perioperative management is important considering the possibility of re-intubation for upper airway obstruction.