Beckwith-Wiedemann Syndrome (BWS) is an overgrowth disorder.

The most common features are a large tongue (macroglossia), an abdominal wall defect and increased growth, but there are many others. Macroglossia in BWS is often symptomless and may not need treatment. Children with large tongues may require therapy or tongue reduction surgery.

In 2012, David Dunaway and his colleagues at Great Ormond Street Hospital set up a national service for children with BWS and macroglossia to provide assessment and treatment. In his private practice, David provides this same multidisciplinary service.

Frequently Asked Questions

This depends on the size and shape of the tongue;

Changes to appearance;

  • The tongue may protrude out of the mouth for some, most of the time or all of the time
  • It rests inside the lower lip causing the lip to protrude and appear “floppy”
  • It may cause increased spacing between the teeth (‘splaying of the teeth’)
  • It may cause an anterior open bite (gap between the upper front teeth and the lower teeth)
  • It may cause the lower jaw to protrude further than the top jaw
  • Some children sleep with their mouth open causing the tongue to become dry or cracked
  • Very rarely, children have difficulties with breathing caused by thickening at the base of the tongue as this can block the upper airway

Respiratory/upper airway difficulties;

  • Feeding difficulties
  • Infants and children may experience a variety of feeding difficulties.
  • Speech difficulties
  • Surprisingly, macroglossia has little impact on the development of speech unless it is extremely large.
  • Others effects
  • Increased dribbling/drooling may occur

It is very important to manage the problem within a multidisciplinary team. The first phase of treatment involves a detailed assessment of the macroglossia and problems associated with it. In addition to being assessed my Professor Dunaway, your child will need to see a specialist Speech and Language Therapist and Orthodontist. Some children will benefit from a tongue reduction. Whether or not a tongue reduction is required, it is recommended that children have regular orthodontic checks and speech therapy assessments to monitor the development of their dentition, speech, feeding, oro-motor skills and facial growth until facial growth is complete in the mid teens.