Kindergartner Kai Fujimori was about 1 1/2 when his mom first had a name for the "bump" on his shoulder.
Then living in Miami, a doctor told his mother, Chiemi Otani, that Kai had something called Sprengel’s deformity. It’s a rare condition that happens in the womb when the scapula (shoulder bone) fails to migrate into the correct position. Instead, it gets stuck near the neck. Usually, it’s only on one side. In Kai’s case, the right side didn’t go into its proper place.
Sometimes this malformation can come with a host of problems that can affect organs such as the kidney and heart, as well as problems with the vertebrae. In Kai’s case, he didn’t experience that.
Kai was not able to lift his right arm above 90 degrees or overhead, and he looked different from other kids, even with a T-shirt on.
There were things that Kai had trouble doing, such as swimming or gymnastics, and when he ran, his gait was different.
Each start of the school year, his mom would explain to the preschool teacher about Kai’s "little bumpy shoulder." This year, she didn’t have to.
When Kai and his family moved to Austin in March 2019, they met with Dr. Hilton Gottschalk, a pediatric orthopedist at Dell Children’s Medical Center of Central Texas who specializes in hands and upper extremities.
Gottschalk says moderate cases of Sprengel’s don’t need surgery and can be helped with physical therapy, but some, like Kai’s, are more severe and noticeable and require surgery.
At first, Kai had therapy to see if he could gain any range of motion without surgery.
"He hit a plateau because of the severity of it," Gottschalk says.
Gottschalk was thinking about Kai being able to be more functional in his daily living, such as dressing himself, and he also was thinking about doing the surgery before Kai began kindergarten because of his peers.
"Anytime you do a big procedure like this, it definitely crosses your mind, what are the risks and the benefits?" Gottschalk says. "The persistence by Mom and watching him not be able to use that hand, that arm, and the cosmetic appearance is the reason why you show up to work every morning."
Gottschalk turned to colleague Brian Kaufman, a pediatric orthopedic surgeon at Dell Children’s who specializes in spine surgeries, to lead the procedure team.
"We certainly could have left it," Kaufman says.
This age of late preschool, early elementary school is ideal for this surgery because physiologically it’s easier to do the post-surgery physical therapy and for things to stay where the surgeon puts them. Older kids have tougher muscles, which makes the surgery more difficult, Kaufman says.
"As he gets older and as his muscles develop more, that scapula becomes fixed," Kaufman says. It becomes harder to relocate the scapula and harder for Kai to accommodate the new way his body works.
In the past, kids who had a Sprengel’s deformity in Austin either weren’t operated on because it wasn’t as severe or they would have the surgery done elsewhere because of lack of expertise here, Kaufman says.
"This city has really benefited from being able to bring in specialists and subspecialists," Gottschalk says. Ten to 15 years ago, patients would have been sent to children’s hospitals in Dallas or Houston, he says.
Because it’s a rare condition, Kaufman had not performed a surgery like this one, nor had it been done at Dell Children’s before, but Kaufman understood how to reshape spines and the muscles around them in cases of spina bifida and other spine conditions, for which he’s done hundreds of surgeries, he said.
Kaufman did research and talked to experts in the field to develop the plan.
On Aug. 14, Kaufman made a large incision in Kai’s back. Then he slowly detached or loosened all the muscles from the spine and the shoulder blade. In Sprengel’s, the shoulder blade is enlarged, and Kaufman removed the top corner of it. Then he took the muscles attached to the shoulder and guided the shoulder down the back into the place where it should have been. Kaufman likens it to the way a dock worker will use ropes to pull a boat down a dock to bring it in closer.
Kaufman had to identify which muscles were too tight and then loosen them by cutting them and reattaching them so that they would be functionally longer and allow the shoulder blade to be in its proper place.
After three hours of surgery, Kai’s shoulder and back looks like most other boys’ his age, except for the long scar that will fade with time. Kai now has a neck that you can see from the back as well as from the front.
Kai wore a sling to keep the shoulder in place while the muscles healed for a few weeks. He will do physical therapy to work on using those muscles in a different way, which means he should have better range of motion for his right arm. The goal, Gottschalk says, is for Kai to gain 30 degrees to 50 degrees from where he has been able to lift his arm.
Kai should not need additional surgeries, Gottschalk says. "It’s one big surgery and you’re done."
"I’m really excited for Kai," Kaufman says. "... It’s hard to be the kid in school that looks different than anyone else. To be able to look like other kids, to use the right arm, to not worry about how he looks in a T-shirt or a bathing suit, I feel great about the opportunity we are going to give him."
For his mom, "I hope with his shoulder he can move his arm as much as possible and do anything he wants and doesn’t have any limitations," she says. "That’s my hope."