Anaesthesia and the Beckwith‐Wiedemann syndrome

Abstract

Infants with Beckwith-Wiedemann syndrome usually present different abnormalities which may require surgical correction. Anaesthetic management may be complicated by abnormal airway anatomy, congenital heart disease and severe hypoglycaemia. Careful preoperative evaluation, perioperative monitoring and suitable choice of anaesthetic technique are required for a successful outcome. We report the perioperative management of a patient with Beckwith-Wiedemann syndrome presenting for omphalocoele surgery on his first day of life and for bilateral inguinal hernia repair four months later.

Perioperative airway management of a patient with Beckwith-Wiedemann syndrome

“…Beckwith-Wiedemann syndrome is caused by mutations in the genes; the prevalence of this condition is 1 per 13,700-15,000 births, with equal sex distribution [ 3 4 5 6 ]. Clinical features of the syndrome include macroglossia, omphalocele, umbilical hernia, and neonatal hypoglycemia [ 4 5 6 7 ]. Macroglossia is especially associated with Beckwith-Wiedemann syndrome in 95% of the patients [ 8 ].…”

“…It was first described by Beckwith in 1963 and later substantiated by Wiedemann in 1964 [ 1 2 3 ]. Macroglossia is a condition observed in patients with this syndrome, and is associated with difficult perioperative anesthetic management [ 4 5 6 ]. It has been reported that perioperative anesthetic management might be complicated by anatomical airway abnormalities [ 1 2 3 4 5 6 ].…”

“…Macroglossia is a condition observed in patients with this syndrome, and is associated with difficult perioperative anesthetic management [ 4 5 6 ]. It has been reported that perioperative anesthetic management might be complicated by anatomical airway abnormalities [ 1 2 3 4 5 6 ]. Here, we report the perioperative airway management of a 14-month-old girl with Beckwith-Wiedemann syndrome who underwent a procedure for the reduction of macroglossia.…”

Ruptured Arteriovenous Malformation in a Boy with Beckwith-Wiedemann Syndrome

“…Abnormal airway anatomy, congenital heart disease and severe hypoglycemia [19, 20]may complicate anesthetic procedures. Craniofacial anomalies, as observed in the present case, often aggravate endotracheal intubation.…”

Successful airway control with the laryngeal mask in an infant with Beckwith–Wiedemann syndrome and hepatoblastoma for central line catheterization

“…The Beckwith–Wiedemann syndrome (BWS) has as its essential features omphalocele, macroglossia, hypoglycaemia, inguinal hernia with gigantism, organomegaly, renal medullary dysplasia, cardiac defects and embryonal tumours occurring less frequently ( 1). Anaesthetic management in children with the BWS ( 2) may be complicated by a potentially difficult airway ( 3–9). The laryngeal mask airway (LMA) may afford airway patency when we are faced with abnormal anatomy and troublesome intubation.…”