Perioperative anesthetic management of reductive glossoplasty in a patient with Beckwith-Wiedemann syndrome. / Manejo anestésico perioperatorio de glosoplastia reductora en paciente con síndrome de Beckwith-Wiedemann.

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>96% and good glycaemic control. Oral intake was started 4hours after surgery, and she was discharged to the ward at 24hours. CONCLUSION: BWS patients require a multimodal approach that includes detailed preoperative planning and knowledge of potential airway-related and systemic complications.