Cutting-edge device approved for compassionate use saves leg, life of cancer patient
November 24, 2020 CHOC Children's
When 7-year-old Naomi Adrian took a spill on the school playground early 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 she 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 the Riverside girl's left distal femur, the area of the leg just above the knee joint.
When a Riverside doctor who was supposed to remove the tumor was unable to see her on the day of the planned surgery, Maria rushed Naomi to CHOC.
Thus began a medical journey that showcases CHOC's commitment to outside-the-box thinking when it comes to patient care, including investigational therapies, diagnostics, and devices — as well as the benefits of CHOC's partnership with UCI Health.
In Naomi's case, her lead physician at CHOC, Dr. Amir Misaghi, an orthopaedic surgeon with specialties in pediatric orthopaedics and musculoskeletal oncology, was able to secure a 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.
Dr. Amir Misaghi, pediatric orthopaedic surgeon at CHOC
"I've used it before," Dr. Misaghi said of Onkos Surgical cutting guides, "but this was the first time I've used it in a pediatric patient."
Ruling out other surgical options that he deemed less promising and more onerous on young Naomi's growing body, Dr. Misaghi, who came to CHOC in October 2019 from Children's Hospital of Philadelphia, used the Onkos device after his one-time compassionate use trial request was approved by the FDA.
In the surgery, in which Dr. Misaghi was assisted by orthopaedic surgeon Dr. Carl Weinert, the Onkos 3D resection guides, which were customized to Naomi's leg and the growing tumor on it, helped the surgeons make extremely precise cuts as they removed the baseball-sized tumor and surrounding bone while sparing Naomi's knee joint and the part of her bone that grows through adolescence.
Dr. Raj Vyas, chief 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 along with the artery and vein that supply that segment of bone.
The fibula bone runs on the outside of the leg from the knee joint to the ankle joint and can be mostly removed without affecting one's ability to bear weight.
Drs. Misaghi and Vyas then worked together to hollow out a cadaveric arm bone (humerus) and insert Naomi's fibula bone into its core. The cadaver humerus bone was custom selected to match the size of Naomi's femur and provide structural support. Dr. Vyas then connected the artery and vein supplying the fibula bone to a nearby artery and vein in the leg using microsurgical techniques. This allowed the vascularized fibula to "bring back to life" the cadaveric 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 said.
Naomi with her mom and Dr. Misaghi
Said Dr. Vyas of the 10 hours he worked 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. 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 to Naomi state-of-the-art reconstruction.
"A while ago," Dr. Vyas added, "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."
Alternative treatments that Dr. Misaghi ruled out for Naomi included a rotationplasty, which would have resulted in the loss of her knee joint and a large portion of her leg, thus requiring a prosthesis; and another technique that also would have meant the loss of her knee joint and a prosthesis that would have required several subsequent surgeries as Naomi grew.
A very active child, Naomi loved to participate in ballet and gymnastics before her diagnosis on March 3, 2020.
"Hopefully we can get her back dancing," Dr. Misaghi said. "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 sister, Itzel, or other relatives push her in her wheelchair.
Naomi, whose family since has relocated to the Fresno area, also loves arts and crafts, her dolls, and playing with her twin sister, Natalie.
Naomi, pre-diagnosis
Naomi and her twin sister, Natalie
"I felt sad (when diagnosed), but I knew that someone was always with me," Naomi says of her cancer journey. "Thanks to Dr. Misaghi (and others), they saved my leg and I'm feeling happier now."
So far, Naomi is progressing very well, said Dr. Misaghi, who keeps regular tabs on 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 said. "But the survival part of her prognosis is very good since she had clear margins and she's gotten back on the chemo, 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."
In a Zoom call, Naomi, wearing a white bow on her head, was asked how her leg felt.
"Good," she said.
Can you walk and do normal things?
"Not yet. Maybe in a year."
Naomi's oldest sibling, Itzel, 20, said Naomi has been a champ through the entire process.
"She never complained about pain even after the surgery," Itzel said.
Itzel and her mother are grateful for the excellent care Naomi received at CHOC.
"We knew that it would be very hard for her to accept losing her leg," Itzel said. "We were happy that there was a way that that could save not only her life, but her leg."
Said Maria: "We are extremely grateful to God for listening to our prayers and with 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. May God continue giving them the intelligence and determination to continue to save other kids' lives."
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 said.
For more information about the Hyundai Cancer Institute at CHOC, click here.
Dr. Amir MisaghiOrthopaedic Instituteorthopaedic oncologyorthopaedic surgeryorthopaedicsosteosarcoma
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