Perioperative anesthetic management of reductive glossoplasty in a patient with Beckwith-Wiedemann syndrome

Abstract

Abstract Introduction Postoperative management of patients with the congenital growth disorder Beckwith-Wiedemann syndrome (BWS) can be complicated. The main clinical manifestations of the syndrome are macroglossia — which may hamper airway management —, prematurity, hemihypertrophy, omphalocele, embryonal tumours and episodes of neonatal hypoglycaemia. Objective Our main objective is to describe the perioperative management and potential anaesthetic complications in paediatric patients with BWS undergoing glossectomy. Methods Case report and literature review. Results We describe the case of an 11-month-old patient diagnosed with BWS who underwent reduction glossoplasty. We performed a comprehensive preoperative evaluation, taking into account potential anaesthetic complications derived from both macroglossia and prematurity, and the risk of hypoglycaemia. The procedure was performed under general anaesthesia. Intubation — performed according to difficult airway management algorithms — was uneventful and the patient was successfully extubated in the operating room. The patient remained stable during the postoperative period, with good respiratory dynamics, SatO2\u202f>\u202f96% and good glycaemic control. Oral intake was started 4\u202fh after surgery, and she was discharged to the ward at 24\u202fh. Conclusion BWS patients require a multimodal approach that includes detailed preoperative planning and knowledge of potential airway-related and systemic complications.