Setting an Innovative Precedent for Craniofacial Reconstruction

How do you create a jaw? Plastic surgeon and Chair of the Department of Pediatric Plastic and Reconstructive Surgery, Phuong Nguyen, MD, FACS, FAAP, grappled with that question for a patient with a unique diagnosis. A lot of thought went into the treatment for 19-year-old Ashley, and through a bold plan and strategic collaboration, the team provided an effective, first-of-its-kind solution.

“How do we create something that nature never gave her?” Dr. Nguyen asked. Throughout his extensive career, Dr. Nguyen has traveled around the world performing surgery to repair and reconstruct facial and cranial differences caused by various conditions, but the combination of surgeries he planned for Ashley was a first.

Ashley was diagnosed with Goldenhar syndrome, left-sided hemifacial microsomia, microtia and Tessier 7 cleft, among other things. Though clefts of the orofacial region are among the most common congenital facial defects, the occurrence of lateral facial clefts (Tessier 7 cleft) in conditions such as Goldenhar syndrome is very rare.

The extent of her facial difference made it difficult to breathe and eat. To achieve the best possible outcome, Dr. Nguyen developed an ambitious plan and brought together numerous specialists to help him with the 16-hour surgery.

“The concept of a ‘jaw in a day’ means that we’re able to do all this in one fell swoop as opposed to multiple staged operations,” says Nguyen. “This means a lot of challenges for both the patient and logistically. When we concocted this plan, we knew we needed to have all the components together for this to be a success. The surgeons are just one part.”

Dr. Nguyen focused on the overall jaw and free fibula surgery. Plastic surgeon Jason Yu, MD, is trained in oral surgery and microsurgery and helped with the free flap harvest and the dental implants. Kristen Lowe, DDS, who works in craniofacial orthodontics, helped with the teeth and occlusion. The final piece, Dr. Nguyen says, was finding a prosthodontist who would volunteer his services. Anthony Chi, DMD, rose to the challenge, bringing with him experience working in similar cases, usually for adults being treated for cancer or tumors.

“The novel aspect of this,” says Dr. Nguyen, “is that, to my knowledge, it’s never been done in a kid before for a congenital disease. We may do 20 to 30 jaw surgeries a year, maybe one or two free fibula flap surgeries and a handful of dental implants — but to do all of them at the same time was a first.”

Dr. Nguyen weighed his options of using the patient’s own tissue versus prosthetics. He decided to use mostly the patient’s own tissue — the free fibula uses part of the fibula in the leg to create the jaw. Prosthetics usually need to be replaced at some later point, and because the patient is young, the team wanted to avoid this. The patient’s own tissue would grow with her and also allow doctors to vascularize the area by connecting it to tiny vessels in the patient’s face (facial artery and vein) using a microscope.

The ambitious surgery was very successful. Just weeks later, Ashley was already able to breathe and eat better. While the team is still doing some fine-tuning and dental rehabilitation, Ashley is already feeling the difference.

Dr. Nguyen went to great lengths to assemble a team of experts to make this surgery possible. Still, he wanted this to be Ashley’s decision. Having already been through many surgeries, she knew how challenging it could be. With complete trust in her medical team, Ashley said yes without hesitation, and it certainly paid off.