In January 2010, at the age of 2, Dominic had tongue reduction surgery on the anterior portion of his tongue. This was performed by Dr Andrew Heggie, Plastic and Maxillofacial Surgeon, and head of the Oral and Maxillofacial Department at RCH Melbourne. Dr Heggie has performed a number of tongue reduction surgeries for children with BWS in Australia.
The decision to go ahead with tongue reduction surgery, is often a very difficult one for parents of children with BWS. Every child with BWS and every case of Macroglossia (an enlarged tongue) is different. In Dominic’s case, there were a number of things we had to consider. Both the back (posterior) and front (anterior) portions of his tongue were enlarged. The back of his tongue causes problems with his breathing, because it blocks his upper airway. This is why he needs a trache. Surgery on this part of the tongue is both difficult and dangerous and comes with an increased risk of complications, so it is not an option. The front of Dominic’s tongue, was causing him to have problems with feeding, jaw alignment, teeth splaying and dental hygiene. An enlarged anterior, or protruding tongue, can also have an affect on the way speech is developed. This wasn’t a concern for us at the time, since having a trache by-passes the vocal chords. Dominic’s speech and language development had already been affected.
During the decision making process, we consulted with a number of specialists. These included Dominic’s Paediatrician, two Oral/Maxillofacial Surgeons, a Respiratory Physician, a Speech Pathologist, a Speech Therapist, an Orthodontist and a Dentist. Each had their opinions for and against, and we were left to ponder each one. The advice that really helped us make up our minds though, came from the parents of children who had already been through it. A number of them shared their experiences and photos with us, through the BWSChat and Beckwith-Wiedemann Groups. Despite being told that surgery on the anterior portion of Dominic’s tongue wouldn’t improve his breathing issues, we decided to go ahead. We learned that the surgery would improve his jaw alignment, teeth and feeding. We also learned that a tongue reduction early on, could possibly reduce the need for other, more invasive surgeries later.
The team at RCH, planned to do a bronchoscope while Dominic was under anesthetic. It had been 18 months since his last scope, so we were eager to know if his airway had improved. Dr Jo Harrison did the scope and met with me immediately afterwards to explain her findings. She found that Dominic had large hypertrophied adenoids that would need to be removed. Meaning one of his adenoids was larger than the other, a common characteristic in children with BWS. Dominic also had a mild subglottic stenosis, reducing his airway by only 20%. She explained that the most likely cause of this was repeated intubations (the insertion of breathing tubes to assist breathing through ventilation) as a newborn. This process can sometimes damage newly formed or under developed airways. She mentioned that it wasn’t too severe and wouldn’t be a problem, if the trache was removed. Good news! She also found he had a small amount of granuloma tissue in his trachea. This is scar tissue that forms, and is the body’s natural reaction to having a plastic tube in the airway. This would also need to be surgically removed, with both procedures able to be done during the one surgery by an ENT surgeon. Phew! Of course the large posterior tongue was still the big problem and I felt myself sink as she explained that even if the lesser airway issues were addressed, it was the tongue that would prevent the trache from coming out.
When Dr Heggie came to see me after surgery, he delivered news that was a little more positive. He discovered while in theatre, that Dominic was tongue tied. By releasing the tie from underneath his tongue, Dr Heggie was able to pull the tongue forward and out of Dominic’s airway. What he had originally thought was a mild to moderate sized tongue, turned out to be a moderately large tongue. While he still couldn’t guarantee that this would improve Dominic’s airway, he expressed that he was interested to see if it would help at all. And so were we.
The healing process was mostly what we expected. Dominic had a small bleed post-op that looked worse than it actually was. He also had two breath holding episodes which were in response to pain. He was smiling again the day after surgery and up and about on day 3. By day 4 he was drinking and eating yoghurt, albeit gingerly. We had thrown away his bottle and dummy and switched to a sippy cup with the valve removed so it was free flowing. He quickly learned to control the flow by tipping the cup up. He dribbled profusely and there was no point dressing him because of this. We decided to use tubi-grip on his arms and legs, to help keep him warm and we changed his bib regularly, as it would be soaked through within an hour. We went through a lot of washers and bibs. The feeding tube came out and we were discharged on Day 5.
The next few days at home Dom ate less but drank more milk, eventually moving from the sippy cup to sports bottles for drinking. He started eating properly again about a fortnight after surgery. You could still see the stitches for a few more weeks after this and despite a small cyst where one of the stitches had been, the healing process was uneventful. 2 months post-op and Dominic started eating pieces of banana and savoury biscuits. He can now chew his food and eats a range of different textures, showing a preference for things that crunch. He is a slow eater but the progress he has made since the surgery is remarkable. Beforehand, all his food had to be pureed, or it would cause him to gag and vomit. His oral hygiene has also improved dramatically, since we can now brush his teeth without the toothbrush making him gag. I find him stealing his toothbrush every time we’re in the bathroom and he has no problem with me finishing the job for him. His Dentist reported that now he doesn’t have the force of his tongue pushing against his teeth, they should start to move back into place. His face shape is starting to change too. The most amazing change of all though, is the change to his breathing. Dominic is now able to breathe around the trache and has started vocalising. He has just moved from a modified portex speaking valve to an unmodified passy muir speaking valve. He is up to wearing it for an hour, before the secretions become too difficult for him to manage. We are trying to extend this to longer periods in the lead up to his next surgery and decanulation trial, to give his upper airway a good work-out.
It has always been maintained that while it may be possible to fix Dominic’s daytime airway, it is highly likely that he will need airway support overnight. This would be in the form of CPAP (continuous positive airway pressure), delivered through a mask that will be worn over his nose when he sleeps. Whether we can remove the trache will depend on whether or not his large posterior tongue still blocks his airway during the day, and if not, whether he will tolerate sleeping with CPAP at night. We are hopeful, but we also understand that having a stable airway is more important than anything else. It’s about what’s best for Dominic. Only time will tell.
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