When 7-year-old Naomi Adrian took a spill on the school playground earlier this year and a schoolmate fell on top of her left leg, she got up with a slight limp.
After what appeared to be a bruise kept growing, and as Naomi continued to walk awkwardly, her mother, Maria Nino, took her to the doctor.
A subsequent visit to a specialist revealed a tumor – an osteosarcoma, a type of bone cancer — on Naomi’s left distal femur, the area of the leg just above the knee joint.
When a doctor who was supposed to remove the tumor at a hospital near their home was unable to see Naomi on the day of the planned surgery, Maria rushed her to CHOC.
This began a medical journey for Naomi that, among other lessons, would give her and Maria a firsthand look at CHOC’s commitment to outside-the-box thinking when it comes to patient care, including investigational therapies, diagnostics and cutting-edge devices — as well as the benefits of CHOC’s partnership with UC Irvine.
Naomi’s lead physician at CHOC, Dr. Amir Misaghi, an orthopaedic surgeon with specialties in pediatric orthopaedics and musculoskeletal oncology, was able to secure a cutting-edge device — that doesn’t have U.S. Food & Drug Administration approval yet for use in pediatric patients — to help him successfully remove the tumor and reconstruct Naomi’s leg. He had used the cutting-edge device, Onkos Surgical cutting guides, before, but this would be the first time using it on a pediatric patient. These guides help surgeons make extremely precise cuts to remove tumors and surrounding bone.
When creating a personalized treatment plan for Naomi, Dr. Misaghi ruled out several surgical options that he deemed less promising and more burdensome on Naomi’s growing body. These included a rotationplasty, which would have resulted in the loss of her knee joint and a large portion of her leg, and required a prosthesis. He also ruled out another technique that likewise would have meant the loss of Naomi’s knee joint and a prosthesis that would have required several more surgeries as Naomi grew.
Dr. Misaghi then applied for a one-time compassionate use trial request with the FDA, which was approved.
In the surgery, Dr. Misaghi, assisted by CHOC orthopaedic surgeon Dr. Carl Weinert, used the Onkos 3D resection guides which had been customized to Naomi’s leg and the growing tumor on it, and removed the baseball-sized tumor and surrounding bone. This allowed them to spare Naomi’s knee joint and the part of her bone that grows through adolescence.
Dr. Raj Vyas, chair of plastic surgery at CHOC and vice chair of plastic surgery at UCI School of Medicine, then began to reconstruct Naomi’s femur. To do this, he dissected free a segment of Naomi’s fibula bone – which runs from the knee to the outside of the ankle, parallel to the tibia or shin bone — along with the artery and vein that supply blood to that segment of bone.
The fibula bone can be mostly removed without affecting someone’s ability to bear weight.
Drs. Misaghi and Vyas then worked together to hollow out a humerus bone – which runs from the shoulder to the elbow — and insert Naomi’s fibula bone into its core. The humerus bone was custom selected from a cadaver to match the size of Naomi’s femur and provide structural support to her leg. Dr. Vyas then connected the artery and vein supplying the fibula bone to a nearby artery and vein in Naomi’s leg using microsurgical techniques. This allowed the vascularized fibula to “bring back to life” the cadaver’s humerus bone so that it can continue to grow with Naomi.
“This was pushing the envelope as far as reconstructive surgeries go for this type of thing,” Dr. Misaghi says.
Dr. Vyas spent 10 hours working on Naomi’s leg.
“In kids, it’s especially an advantage to use their own tissue if possible. If we didn’t have the ability to do this miscrosurgery, then we would have just done an inferior operation,” Dr. Vyas says. “Being able to work together and plan to do this at a children’s hospital with Dr. Misaghi’s expertise and our expertise at UCI Health, we were able to provide Naomi with a state-of-the-art reconstruction.”
He added, “A while ago, maybe at some hospitals they would have just performed an amputation. Because we have Dr. Misaghi, who can do a limb salvage using a cadaver graft, we were able to really perform the best operation possible.”
A very active child, Naomi loved to participate in ballet and gymnastics before her diagnosis.
“Hopefully we can get Naomi back dancing,” Dr. Misaghi says. “That would be the goal.”
Trips to the park
These days, as Naomi continues to undergo chemotherapy, the now-8-year-old enjoys trips to the park where her older sister, Itzel, pushes her in her wheelchair.
Naomi, whose family has since relocated to the Fresno area, also loves arts and crafts, her dolls, and playing with her twin sister, Natalie.
“I felt sad, but I knew that someone was always with me,” Naomi says of her cancer diagnosis and journey. “Thanks to Dr. Misaghi, they saved my leg and I’m feeling happier now.”
So far, Naomi is progressing very well, Dr. Misaghi says, who keeps in regular contact with her. A year out from surgery, Naomi will be back at CHOC for a second surgery to remove screws that were placed near her growth plate.
“It remains to be seen how her growth plate responds,” Dr. Misaghi says. “But the survival part of her prognosis is very good, since she had clear margins and she’s resumed chemotherapy, and so the function of her leg prognosis is very good. She has a normal knee joint. And hopefully when everything heals, she’ll have some metal plates remaining but be able to grow normally with her own growth plate.”
Naomi says her leg feels “good” but that she can’t walk yet – maybe in a year, though.
Itzel says Naomi has been a champ through the entire process.
“She never complained about pain even after the surgery,” Itzel says.
Itzel and her mother are grateful for the excellent care Naomi received at CHOC.
“We knew that it would be very hard for Naomi to accept losing her leg,” Itzel says. “She’s so young and she’s such a girly girl; she’s always worrying about how she looks and everything. We were happy that there was a way that that could save not only her life, but her leg.”
Mom Maria echoes that feeling of gratitude.
“We are extremely grateful to God for listening to our prayers and for the amazing doctors, nurses and staff at CHOC. I want to especially thank Dr. Misaghi and Dr. Vyas as they made sure Naomi got the care she needed. They are a great team,” she says.
Naomi already knows what she wants to be when she grows up.
“I want to be a surgeon so I can save other people’s hands and legs,” she says.