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: 1/11/2018 1:59:07 PM
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 and register
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.
From the Newsdesk
Children who receive care at the Craniofacial Center at Nicklaus Children’s, along with their families, enjoyed an afternoon of red carpet glamour and photo booth fun on April 28.
A look into the Fetal Care Center at Nicklaus Children's Hospital. For more information and to contact a nurse navigator visit nicklauschhildrens.org/FetalCare