What is it?
Beckwith Wiedemann Syndrome is a condition of overgrowth that affects different parts of the body. Babies are generally larger than other children the same age, and one whole side of the body may be bigger than the other. They may have hernias, omphaloceles, endocrine problems, and are at an increased risk for developing different cancers. Because of this, monitoring all of these different problems and keeping your child healthy will require multiple different specialists. As part of the syndrome, your child may also have an unusually large tongue called “macroglossia”. If the tongue is causing problems, you may be referred to see a craniofacial surgeon for something called a “tongue reduction”. Often the tongue is so big that it always hangs out of the mouth, and your child may not be able to close their lips. This can cause problems with eating, speaking, drooling, and jaw growth. There are two main reasons to perform a tongue reduction. This first is for appearance. Children with Beckwith Wiedemann Syndrome tend to have normal intelligence and lifespans, and will likely be socially stigmatized if they are unable to control their tongue and keep it in their mouth. The second reason is to control jaw growth. When the tongue is very large, it tends to push on the teeth and lower jaw and make them larger as well. This leads to an “underbite”, or even an “open bite” where the teeth do not come together in the middle. The data that we have available seems to show that, if a tongue reduction is performed before the age of two, it tends to prevent the formation of this abnormal bite, and the children usually have normal jaw growth. Overall, the surgery does not seem to specifically affect drooling, breathing or speaking. Even though we are removing part of the tongue, taste is not negatively affected. Generally, we perform tongue reduction surgery between 3-6 months of age, and ideally before 2 years.
What happens during surgery and recovery?
During surgery, parts of the front and sides of the tongue are removed in a W shape. This makes the tongue shorter and less wide. The incisions are then closed with absorbable sutures that will fall out in several weeks. There will be a suture line in the middle of the tongue on the top and bottom, and along both sides. Usually there is very little bleeding with surgery, but the tongue does tend to become quite swollen. Because of this, we have your child spend a night in the ICU to monitor for any breathing problems, and we also give a few doses of steroids. After tongue reduction surgery, children are more susceptible to fungal infections of the tongue and mouth, so we will have you paint a special antifungal medicine on the tongue. You should expect to stay in the hospital for 2-3 days. As a result of pain and swelling, it may take your child a day or two before they are interested in eating normally. You will be able to go home once your child is eating and pain is controlled. Most children heal very well after the surgery, and have normal motion and use of the tongue.
Beckwith Wiedemann Syndrome is a condition of overgrowth that affects different parts of the body. Babies are generally larger than other children the same age, and one whole side of the body may be bigger than the other. They may have hernias, omphaloceles, endocrine problems, and are at an increased risk for developing different cancers. Because of this, monitoring all of these different problems and keeping your child healthy will require multiple different specialists. As part of the syndrome, your child may also have an unusually large tongue called “macroglossia”. If the tongue is causing problems, you may be referred to see a craniofacial surgeon for something called a “tongue reduction”. Often the tongue is so big that it always hangs out of the mouth, and your child may not be able to close their lips. This can cause problems with eating, speaking, drooling, and jaw growth. There are two main reasons to perform a tongue reduction. This first is for appearance. Children with Beckwith Wiedemann Syndrome tend to have normal intelligence and lifespans, and will likely be socially stigmatized if they are unable to control their tongue and keep it in their mouth. The second reason is to control jaw growth. When the tongue is very large, it tends to push on the teeth and lower jaw and make them larger as well. This leads to an “underbite”, or even an “open bite” where the teeth do not come together in the middle. The data that we have available seems to show that, if a tongue reduction is performed before the age of two, it tends to prevent the formation of this abnormal bite, and the children usually have normal jaw growth. Overall, the surgery does not seem to specifically affect drooling, breathing or speaking. Even though we are removing part of the tongue, taste is not negatively affected. Generally, we perform tongue reduction surgery between 3-6 months of age, and ideally before 2 years.
What happens during surgery and recovery?
During surgery, parts of the front and sides of the tongue are removed in a W shape. This makes the tongue shorter and less wide. The incisions are then closed with absorbable sutures that will fall out in several weeks. There will be a suture line in the middle of the tongue on the top and bottom, and along both sides. Usually there is very little bleeding with surgery, but the tongue does tend to become quite swollen. Because of this, we have your child spend a night in the ICU to monitor for any breathing problems, and we also give a few doses of steroids. After tongue reduction surgery, children are more susceptible to fungal infections of the tongue and mouth, so we will have you paint a special antifungal medicine on the tongue. You should expect to stay in the hospital for 2-3 days. As a result of pain and swelling, it may take your child a day or two before they are interested in eating normally. You will be able to go home once your child is eating and pain is controlled. Most children heal very well after the surgery, and have normal motion and use of the tongue.
