Macroglossia definition

Macroglossia is the medical term for when a child is born with an enlarged tongue. It is one of the most common features of Beckwith-Wiedemann Syndrome (BWS), affecting around 90 percent of children with the congenital disorder. If left untreated, macroglossia can lead to dental-skeletal deformities including open bite, crossbite, and jaw problems.

Macroglossia causes, symptoms, and diagnosis

Children with macroglossia have tongues that are disproportionately large for their mouths. In some cases, the tongue may protrude from the mouth and interfere with the child’s tooth placement, ability to eat, and/or speech development. Macroglossia diagnosis focuses on determining its underlying cause. The doctor will assess your child’s symptoms and family history to assess whether the enlarged tongue is a result of BWS or another abnormality. 

Treatment options

In some cases, the symptoms may not be severe enough to require surgery. However, our surgical program for the treatment of macroglossia is highly respected as one of the few teams in the world specializing in the W-cut procedure (anterior lingual tongue reduction).

If your child’s macroglossia is caused by Beckwith-Wiedemann, it is important to manage the full set of symptoms associated with the disorder. Children with BWS need to be screened regularly for malignancy as they are at an increased risk of cancers like Wilms' tumor (nephroblastoma), pancreatoblastoma, and hepatoblastoma. Screening protocols may vary, but we usually recommend that children under the age of eight have an abdominal ultrasound every three months. We also suggest annual blood tests for BWS patients to measure alpha-fetoprotein (AFP) until they are at least four years of age. The risk typically dissipates after childhood. Ongoing screening protocol includes:
  • AFP blood test every six weeks from birth to four years old.
  • Kidney and liver ultrasound every three months from birth to eight years old.

With ongoing management, children with macroglossia and BWS can live full, happy lives. 

Reviewed by: Chad Perlyn, MD

This page was last updated on: 6/21/2019 2:16:57 AM

Upcoming Events

Beckwith-Wiedemann Syndrome (BWS) Family Conference

Date: Friday, July 10, 2020

Nicklaus Children’s Hospital invites you to attend a conference designed to provide individuals with Beckwith-Wiedemann Syndrome (BWS) and their family’s up-to-date information about the possible aspects of BWS and their management.  Learn more.

Register Online