Anesthetic management of two cases of Beckwith-Wiedemann syndrome

Abstract

Two cases of children with Beckwith-Wiedemann syndrome are presented. This syndrome consists of various abnormalities, including macroglossia, visceromegaly, omphalocele, and gigantism. These abnormalities frequently require operative correction during the neonatal period. We anesthesiologists should make plans for difficult airway managements in patients with this syndrome. Our two patients also showed larger sized tracheas than those estimated by their age and height. A cuffed tube, though still controversial, has recently been used in children. We recommend using a cuffed tube in patients with this syndrome, because the appropriate tracheal size may not be predictable, tracheal intubation might be difficult, and risks incurred during changing of a tracheal tube should be avoided.

Anesthetic management of tongue reduction in a case of Beckwith-Wiedemann syndrome

“…[ 7 ] We used an uncuffed endotracheal tube and packed the pharynx with saline soaked gauze, although cuffed tubes have also been used for such cases. [ 8 ] Tracheal intubation is preferred under sedation with inhalational agents while the patient is breathing spontaneously as in our case. Failure to negotiate size 3 ETT could have been due to a hypertrophied subglottis as in this hypertrophy disorder or a narrow subglottis in accordance with the age of prematurity of this child.…”

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 ].…”

Successful perioperative airway management in a patient with angiomatous macroglossia for laser ablation under general anesthesia

“…Macroglossia is predominantly observed in pediatric patients and is not very frequent in adult patients. In patients with Beckwith-Wiedemann syndrome, which is a genetic disorder, macroglossia arises from hyperplasia of the tongue tissues and hypertrophy of the tongue musculature (Horn et al 2001;Kimura et al 2008). In adults, hypothyroidism and hyperpituitarism may cause macroglossia due to hyperplasia of the tongue tissues.…”

“…Assessment or evaluation of structures and functions controlled or affected by the tongue is absolutely necessary for successful anesthetic management (Kimura et al 2008;Perkins 2009). The intrinsic tongue musculature and the size of the infiltrated tissues should be assessed by computed tomography and, preferentially, MRI.…”