Tailored Anaesthesia in Paediatric Beckwith-Wiedemann Syndrome: A Case Report

Beckwith-Wiedemann Syndrome (BWS) is a complex overgrowth disorder characterised primarily by macroglossia, omphalocele, and gigantism, along with a range of anatomical and metabolic abnormalities such as neonatal hypoglycaemia, embryonal tumours, visceromegaly, naevus flammeus, and anal dimple. Airway management in paediatric patients poses a significant challenge for anaesthesiologists. Anaesthetic management in patients with BWS is demanding because macroglossia may cause difficult mask ventilation, intubation, and extubation. These patients often require repeated anaesthetic exposure for the correction of macroglossia and associated abnormalities, and their perioperative management can be taxing for anaesthesiologists. Therefore, the anaesthetic management of children with BWS is particularly challenging due to the combination of age-related physiological limitations and disease-specific abnormalities. Here, the authors present the case of a five-month-old female infant diagnosed with BWS, with macroglossia, naevus flammeus, and an anal dimple, who underwent surgical reduction of macroglossia. The patient also demonstrated additional systemic findings including non-obstructing hydrocephalus, syringohydromyelia, hepatosplenomegaly, and enlarged kidneys on imaging, further increasing the complexity of anaesthetic care. Given the likelihood of a difficult airway, comprehensive preoperative evaluation, meticulous preparation, and well-structured backup strategies for airway management, endotracheal intubation, and intraoperative haemodynamic management are critical to navigating the anaesthetic complexities in patients with BWS. In addition, vigilant postoperative monitoring is essential due to the risk of airway obstruction and hypoglycaemia. The present case underscores the importance of multidisciplinary coordination and individualised anaesthetic planning to ensure safe perioperative outcomes.