[Perioperative management of tongue reduction surgery for macroglossia associated with Beckwith-Wiedemann syndrome -A retrospective evaluation of 14 patients--].

BACKGROUND

Macroglossia is the commonest symptom of Beckwith-Wiedemann syndrome (BWS) and sometimes requires surgical tongue reduction for cosmetic, feeding, drooling and speech problems.

METHODS

We retrospectively reviewed the perioperative course of 14 BWS patients. The subjects were children who underwent tongue reduction surgery or glossopexy between 1994 and 2008 at Osaka Medical Center for Maternal & Child Health.

RESULTS

The median age was 18 months, and the median weight was 12.2 kg at the time of surgery. One patient had the trachea intubated and another had tracheostomy to keep airway patency. Other 12 patients had no artificial airway and were premedicated with midazolam or diazepam and had the trachea intubated after induction with sevoflurane and nitrous oxide in oxygen. Only one patient developed difficult mask ventilation for which a nasal airway was applied. No patients demonstrated difficult intubation. All without preoperative artificial airway were extubated in the OR after the surgery. One patient demonstrated hypoglycemia. Airway compromise in the two patients who was intubated or had tracheostomy prior to surgery was not alleviated by the surgery.

CONCLUSIONS

Airway disorder was not alleviated by tongue reduction surgery or glossopexy.