Beckwith-Wiedemann syndrome is characterized by an overgrowth syndrome with numerous signs and symptoms depending on the affected individual: ompalocele, other abdominal wall defects, macroglossia
, visceromegaly, or hypoglycemia.
, aglossia) and its associated anatomical structures (e.g.
He had macroglossia
. The patient was pre-oxygenated via a face mask.
Some of the relevant anatomic characteristics include the maxillofacial and airway morphology, with patients usually presenting with broad head, prominent forehead, short maxilla, large mandible, macroglossia
, limited neck extension, and pharyngeal, laryngeal, and/or tracheal stenosis, (4,7) which in combination may lead to difficult airway management during general anesthesia.
DS is accompanied by physical functional problems that are highly specific for the orofacial region.1 These functional problems include speech, swallowing and chewing difficulties, and there also appears to be an increase in facial, skeletal and orthodontic problems.1,2 Among the soft tissue characteristics of oral cavity, there exists relative macroglossia
since there is a protrusive tongue with deep fissures and a smaller oral cavity due to the deformity in the midface.1,3,4
Individuals with neck circumference more than 40 cm, narrow nasal or pharyngeal airway, enlarged adenoids and tonsils, deviated nasal septum, macroglossia
and micrognathia are susceptible to obstructive sleep apnoea.
Angular cheilitis with secondary infections often occurs also in patients with macroglossia
(congenital hypothyroidism and Down syndrome).
He showed the distinctive features of a short neck, jaw deformation, and macroglossia
, and he had slight cervical motor restriction and a Mallampati classification of d.
Approximately 20 minutes after ED arrival, the patient deteriorated with respiratory failure and required intubation due to severe macroglossia
and lip swelling with facial fullness.
This condition consists of dysostosis multiplex, especially of the spine, growth retardation-associated muscular atrophy, hepatosplenomegaly, cardiac involvement (thickening of the heart valves), macroglossia
, and hearing loss [1, 3, 9].
The clinical features of Beckwith-Wiedemann syndrome (BWS) include hemihypertrophy and/or macroglossia
. Hypoglycemia is reported in 30-50% of the babies with BWS .
Notable dysmorphic features included transverse palmar crease, macroglossia
, a flat nasal bridge, low set ears, increased skin over back of neck, a short neck, and upslanting palpebral fissures.