Seattle Children’s Craniofacial Center is the first to offer children with Apert syndrome a revolutionary procedure to normalize their facial proportions.
Dr. Richard Hopper and his colleagues in the Craniofacial Center have devised a single surgery to normalize the faces of children with Apert syndrome, a rare form of craniosynostosis characterized by a concave face and webbed fingers.
These children typically endure multiple surgeries, beginning in the first year of life and continuing into early adulthood, to relieve pressure on the brain, release their fingers, and reposition the midface to help with breathing, eating and sleeping.
Until now, none of these procedures addressed the central stigma associated with Apert: a sunken, spoon-shaped midface.
“As humans we instinctively scan a person’s face when we meet them,” says Hopper. “If the proportions and balance of the face look right, we’re more likely to interact. When someone meets a child with Apert syndrome for the first time, it can be hard for the person to get past the fact that the child’s face just looks wrong.”
Hopper was determined to give his patients the chance to interact with the world without their faces getting in the way.
Reassembling the midface
The traditional treatment to bring the midface forward is a subcranial Le Fort III distraction, which moves the bones in a block. While this improves the position of the bite and eye sockets and opens the airway, it doesn’t normalize the facial proportions.
Working with Dr. Hitesh Kapadia, a craniofacial surgical orthodontist, Hopper developed a novel procedure to move the bones of the face different amounts, in different directions. “Part of the face needs to move down and part of it needs to move up. Essentially, you have to unlock the compressed jigsaw puzzle of the midface and then reassemble it,” says Hopper.
Hopper and Kapadia combine low-dose CT scans, Dolphin imaging software and standardized growth predictions to precisely plan the direction and magnitude of the movement of the facial bones. Taking into account the age of the child at the time of surgery, Kapadia predicts where the teeth need to meet and how that affects the cheekbones, nose and the soft tissues of the face.
During the surgery, Hopper and his team separate the bones of the face from the skull so that the entire face is mobile. The cheekbones and eye sockets are then released from the middle of the face and repositioned into a more normal position using small plates and screws. The forehead is smoothed and advanced with a custom implant designed specifically for the child.
A distractor is then attached to the central part of the face that is still mobile. For the next two to three weeks, while the child is at home, a parent or caregiver turns the distractor 1 to 2 millimeters a day to continually move the bones. This can move the upper jaw up to an inch or more. New bone then grows to fill in the gaps and the distractor stays on for about six more weeks to allow for healing. Once everything is in the right position, the distractor is removed.
In 2013, Hopper, Kapadia and medical student Trent Morton published a comparison of the new procedure with the more typical Le Fort III treatment in a paper titled “Normalizing Facial Ratios in Apert Syndrome Patients with Le Fort II Midface Distraction and Simultaneous Zygomatic Repositioning” in the official journal of the American Society of Plastic Surgeons. Their retrospective study, based on analysis of patients’ CT scans, demonstrates that the new procedure is more effective than the Le Fort III in normalizing facial ratios.
The paper received the Marchac award from the International Society of Craniofacial Surgery and the Kawamoto award from the American Society of Craniofacial Surgery.
To date, Children’s has treated nine patients with this approach, with excellent results. Surgeons in Birmingham, England, Dallas and Tokyo have reported adopting this procedure.
“It’s been a joy to be able to offer kids this new technique that has such a good outcome,” said Hopper. “There aren’t a lot of other centers that are able to focus on revolutionizing the way we treat Apert syndrome, and we look forward to sharing the knowledge and offering it to other kids in the future.”