Emmett Paves the Way for Other Patients

Emmett’s days center around superheroes and LEGOS. He likes to think about which book he’ll read at night and which Minecraft creation he can build next.

After all, he’s 6 years old.

What Emmett doesn’t think about is how the medical care he received at Shriners Children’s St. Louis is becoming a model for future patients just like him.

Emmett was born with proximal femoral focal deficiency (PFFD). His parents wouldn’t find a correct diagnosis and treatment plan for years, until they traveled from their home in Springfield, Illinois, to the Gateway City. Eric Gordon, M.D., Shriners Children’s St. Louis orthopedic surgeon and Washington University professor, shared his expertise, guiding the family to solutions.

PFFD is a birth defect that leaves one leg shorter than the other. For most children, mobility becomes an issue. For Emmett, that happened at about his sixth birthday.

“At this point, he was having problems walking around. You can put a lift on the bottom of a child’s shoe, but if it gets more than about 2 to 2.5 inches, it becomes a problem. They start rolling off of it, they can’t do activities they want to. So, we really wanted to go ahead and do a lengthening for Emmett,” explained Gordon.

The only issue: Emmett was 6 years old, not 8, which is the typical baseline age for the use of an Ellipse Nail for limb lengthening. Simply put, his femur wasn’t long enough or mature enough to support the tool.

So, Emmett’s case led Gordon to perform a first for Shriners Children’s St. Louis and the Center for Advanced Pediatric Limb Reconstruction and Lengthening: an internal rod lengthening where the Ellipse Nail is on the outside of the bone – instead of inside the center of the bone. Only a handful of hospitals in the world offer such a procedure.

“The alternative is to lengthen the kids with an external fixator. Especially for the thigh bone, no one likes to have an external fixator up there. It’s uncomfortable for the kids. It’s awkward to get around and severely limits their motion. It’s a real struggle. So, a lot of the kids who are in this situation – who would have used an external fixator in the past, we can use this technique with the rod placed on the side of the bone but completely inside the thigh. We plan on using it more,” said Gordon.

“He had the nail placed in September. We used a magnet to lengthen – to give him 5 centimeters in his femur. That process finished in October. The nail will stay in until the bone fills back in,” said Daphne, Emmett’s mother.

Before surgery, Emmett’s team at Shriners Children’s St. Louis calculated a 2.5 inch difference between his left leg and his right. Gordon said the lengthening gave him 2 inches back.

The issue with limb lengthening in a child: Their bodies aren’t finished growing yet. Thus, the length discrepancy returns. In Emmett's case, however, the use of several lengthening treatment plans will allow the left leg to stay at a manageable difference and allow him to continue to be active.

For the time being, Emmett’s lift is shorter and he’s able to be more active.

Daphne said Emmett’s long-term plan includes two more lengthening treatments and a potential third surgery to stunt the growth plate in his right leg to halt growth. Gordon believes that by the time he needs his second lengthening, Emmett’s femur will be long enough that he’ll be able to insert the nail into the center of the bone as he normally does.

Daphne is hopeful that one day Emmett will be able to wear flip flops or even roller skates. His Shriners Children’s St. Louis care team hopes so too!

“He can walk flat-footed at times. It’s strange that the lifts in his shoes now, are all too high. They need to be shortened,” Daphne said as she smiled.

“Shriners has given us a lots of confidence. They have really helped him,” she said.