Revolutionary surgery improves airway and jaw position in children with Treacher Collins syndrome

A revolutionary surgery developed by Dr. Richard Hopper, surgical director of Seattle Children's Craniofacial Center, called subcranial rotation distraction, is changing the lives of children who are tracheostomy dependent. Seattle Children's Craniofacial Center is the first in the world to use subcranial rotation distraction to improve the airway and jaw position in children with Treacher Collins syndrome, a rare genetic condition that affects the development of a baby's facial skeleton, skin and face muscles before birth. After inventing the new technique, Hopper is now sharing it with craniofacial surgeons across the world so they too can offer it to their patients.

"This surgery will be a paradigm shift for how we treat children with severely restricted airways," said Hopper. "For children with a tracheostomy, the burden on a family is immeasurable. This new technique allows children to be freed from the limitations of their tracheostomy, enabling them to do all the activities kids should be able to do."

Treacher Collins occurs in about one of 50,000 newborns, and while the severity of the condition varies, babies born with the condition often have problems breathing, chewing, swallowing, hearing and speaking.

The first-of-its-kind procedure combines two procedures, surgery and distraction osteogenesis, to correct and rotate the position of the jaw to open up a child's airway.

"It's unlike anything else we do as surgeons," said Hopper. "During surgery the entire face is separated from the skull base and the two jaws are locked together. We create a metal hinge at the top of the nose that allows the distraction devices to rotate the entire face and unlock the breathing space behind the jaws and nose. The airway is like a narrow tunnel. Treatments in the past have only been able to remove single speedbumps within the tunnel. Subcranial rotation distraction essentially takes the whole roof of the tunnel and opens it. The airway results are something we hadn't seen before, and we've also found the surgery results in positive cosmetic differences as well."

Hannah Schow, 11, was the first patient with Treacher Collins syndrome to undergo this procedure. She lived with a tracheostomy since she was 2 weeks old, unable to breathe without a tracheostomy. When standard treatments were unsuccessful in opening her airway, the family thought they had run out of options. Fortunately, after traveling from Idaho to Seattle Children, the family learned she was a great candidate for Hopper's subcranial rotation distraction. Her first and most complex surgery was performed at Seattle Children's in August 2015.

This week, Schow had her tracheostomy removed and can now breathe for the first time without it.

"Getting her tracheostomy removed had been our hope since she was 2 weeks old," said Hannah's mother, Jennifer Schow. "Living with a tracheostomy is all she's ever known. Without it she can swim under water, go to sleepovers or play softball for the first time. It's a dream come true."

Hopper adds, "Hannah is a pioneer. She's paved the road for future patients that will benefit from this procedure. The benefits have opened up a new life for her, and I am so pleased she can now do all the things that she loves."