Macroglossia

Also known as: enlargement of the tongue

What is Macroglossia?

Macroglossia, or enlargement of the tongue, is one of the hallmark features of BWS. It affects approximately 90 percent of children with BWS. If left untreated, macroglossia can lead to dental-skeletal deformities including open bite, cross bite, and jaw problems. Other features of BWS include ear pits or creases, nevus flammeus (port-wine stain) on the nasal bridge or neck, abdominal wall hernias or anomalies, and hemi-hypertrohpy (enlargement of one side of the body).


Surgical Treatment for Macroglossia

The surgical program for the treatment of macroglossia (enlarged tongue) at Nicklaus Children’s Hospital  is highly respected and is one of the few hospitals in the world with a team specializing in what is often called the W-cut procedure, also known as an anterior lingual tongue reduction.

Ongoing Care Management

Children with BWS need to be screened regularly for malignancy as they are at an increased risk of cancers, including Wilms' tumor (nephroblastoma), pancreatoblastoma and hepatoblastoma.  Screening protocols may vary, but on average, children should have an abdominal ultrasound every 3 months until at least eight years of age and a frequent blood tests every year to measure alpha-fetoprotein (AFP) until at least four years of age. This risk typically dissipates after childhood.
Ongoing screening protocol includes:
  • AFP blood test every 6 weeks from birth to 4 years old
  • Kidney and Liver ultrasound every 3 months from birth to 8 years

Reviewed by: Chad Perlyn, MD

This page was last updated on: 8/31/2017 4:13:21 PM

Video screen capture of Tongue Reduction Surgery for BWS
Dr. Chad Perlyn, pediatric plastic surgeon, talks about the different types of tongue reduction operations for BWS.

From the Newsdesk

Harper's Success Story
08/31/2017 — When Harper was diagnosed with Beckwith Wiedemann Syndrome shortly after birth, her family knew they wanted the best team possible for her tongue reduction surgery. Harper now leads a limitless life thanks to Dr. Chad Perlyn, an expert in treating macroglossia, and the Craniofacial Center at Nicklaus Children’s Hospital.
Harper's Success Story
08/31/2017 — When Harper was diagnosed with Beckwith Wiedemann Syndrome shortly after birth, her family knew they wanted the best team possible for her tongue reduction surgery. Harper now leads a limitless life thanks to Dr. Chad Perlyn, an expert in treating macroglossia, and the Craniofacial Center at Nicklaus Children’s Hospital.