Extraordinary Knee

Surgery delivers hope for future in sports

An ankle becomes a knee for child with sarcoma

Childhood is typically filled with adventure and joy. Kids run and play with reckless abandon at recess and fill their minds with new concepts in the classroom. For 9-year-old Jorge Portugal-Herrera, a Green Bay Packers fanatic and aspiring football player, all that stopped when he was diagnosed with cancer.

“It began with knee pain in December 2015,” says Jorge’s mother, Alma Portugal. “Every month, there were emergencies because of the pain, but doctors couldn’t find anything wrong. We were told it was inflammation and to go home and ice his knee.”

Thanks to a multidisciplinary team of sarcoma experts at UC Davis Health, Jorge received comprehensive care to treat the cancer and save his leg. That involved an extraordinary procedure to replace his diseased knee with his own healthy ankle. UC Davis Health is the only Sacramento-area health system to offer the complex surgery, called a rotationplasty.

The Portugal-Herrera family, originally from Michoacán, Mexico, lives in Acampo, a 600-acre town near Lodi, California. Jorge Herrera, Sr. is a farmworker in the area, and Alma pruned grape vines before becoming Jorge’s full-time caregiver. Their home also includes 11-year-old Gaby, 18-year-old Sarai and her 18-month-old son, Ernan. The eldest, 23-year-old U.S. Marine Francisco, stays in touch with his family via FaceTime.

From unexplained pain to a sarcoma diagnosis and treatment plan

Jorge endured six months of intense knee pain and missed a lot of school before his doctor discovered something new on an X-ray. Physicians in Lodi quickly referred the family to UC Davis Health, where specialists diagnosed Jorge with Ewing’s sarcoma and devised a treatment plan.

“We worked as a multidisciplinary team,” says pediatric oncologist Marcio Malogolowkin. “Steven Thorpe, his orthopaedic oncology surgeon, and I were involved from the very beginning, from patient referral to evaluation to confirmation of diagnosis and start of therapy.”

Collaboration is fundamental to the mission of the Cancer Center’s Pediatric and Adolescent and Young Adult Sarcoma Clinic, where families get care from oncologists, surgeons, nurse navigators, physical therapists and child life specialists.

“The best way to provide care to our patients in a timely manner is this kind of approach,” says Malogolowkin. “Families leave the clinic with a full assessment and agreement among the team. They have an individualized plan, and we go through the process with them so they don’t feel alone.”

Jorge’s tumor was in his left distal femur and proximal tibia — or inside his left knee — and surrounding soft tissues. To help shrink the mass before surgery he would receive several weeks of chemotherapy. The next phase of treatment would be far more complex.

The doctors presented three options to the Portugal-Herrera family — all were daunting: amputation of the leg from above the knee; limb-sparing surgery with a custom-implanted, expandable metal prosthesis; and rotationplasty, using Jorge’s ankle joint to make a new knee.

Thorpe explained that amputation above the knee would drastically limit Jorge’s mobility. An implanted prosthesis would require multiple surgeries to accommodate his growing body and would be a difficult procedure because the cancer involved both sides of his knee. Rotationplasty, although cosmetically challenging, would give Jorge a new knee joint after tumor removal and better mobility for an active life without the need for additional surgeries.

Convincing him and his parents, however, was another matter. After the operation, Jorge’s foot would extend out backwards from where his knee would normally be — an anomaly that keeps many people from choosing to do it.

“It was not an easy decision,” says Portugal-Herrera. “It gave us a lot of anguish, but in the end it was the best option for him. The doctors told him he had to know everything because his body was going to change.”

Preparing then 7-year-old Jorge was critical. Part of that included watching a video, which showed Jorge what his new leg would look like. Throughout, Jorge was quiet, but seemed to understand.

A long day of surgery and waiting

At 6 a.m. on Nov. 29, 2016, hospital staff admitted Jorge, and the Portugal-Herrera family waited anxiously for the life-saving surgery to end.

In the operating room, Thorpe worked to remove the tumor which involved resecting the knee, including the top of the tibia and the bottom of the femur. Separation and isolation of the blood vessels and nerves from the tumor resection required six hands — those of Thorpe and his assisting surgeons to safeguard against damage or clotting.

“What takes a meticulous amount of time is that the blood vessels and nerves extending from the thigh to supply the leg and foot have to be dissected away from the tumor, bone, and surrounding soft tissue you are taking out,” says Thorpe. “If a vessel clots off, the rotationplasty can fail. If you stretch a nerve too much, you can cause injury to it.”

Four hours later, the team quickly set to work on the next phase of surgery: reconstructing the leg. First, the team rotated the leg 180 degrees so that the preserved shin bone and femur were aligned and could be affixed.

“This can be difficult because of the difference in the anatomy of the two bones,” says Thorpe. “You have to get the rotation just right so it can heal in appropriate limb rotation so that the eventual prosthetic and leg can function.”

The surgeons next stitched the quadriceps muscles in the thigh to the calf muscle to create knee extension, and the hamstrings to the long muscles on the front of the shin to create knee flexion. To protect the spared nerves and vessels, now much longer than needed for the length of the shorter leg, the surgeons carefully coiled them and tucked them into space to protect them from damage or potential clots.

When reconstruction was complete, Thorpe carefully closed the skin of the calf to the thigh, completing the operation. All told, with anesthesia and surgery Jorge was in the operating room nearly 12 hours.

Post-op recovery another long ordeal

Jorge could feel his foot immediately and was soon wiggling his toes. He was able to move his new knee within two months of the surgery. But the healing took time. He needed postoperative chemotherapy which, while important to curing his cancer, can impair healing.Jorge also fell and broke his ankle (new knee), which in turn delayed his getting a prosthesis.

Happily, his family, friends and school personnel were there throughout to help him pull through. While he was hospitalized, for example, his mother performed “surgery” on his stuffed toys, including several Teddy bears his brother had given him so that now all of them had the same procedure Jorge had.

It was a year before Jorge finally had the prosthesis, and he experienced another setback when he broke the ankle (new knee) again trying to kick a soccer ball. Today, however, he easily pulls the prosthesis on and off and can climb, run and dive for the football, albeit with some happy tumbles along the way.

The Portugal-Herrera family is pleased with the collective decision to reconstruct Jorge’s leg, which they believe will give him the best chance to be a regular kid with few physical limitations.

“I told his family, ‘I hope he’s so physically active he wears the prosthesis out,’” says Thorpe. “I love taking care of kids with sarcoma like Jorge. Their resilience always amazes me.”

In June 2018, Jorge celebrated one year cancer-free with his family and friends with a party and a “Krabby Patty” cake inspired by SpongeBob SquarePants. He still works to get caught up with his studies, and still struggles from time to time getting accustomed to his new normal at home and at  chool.

But when he’s strong enough, he hopes to acquire a state-of-the-art prosthesis like the ones disabled athletes use, and lights up when he imagines it.

“What motivates us to keep moving forward is that he always smiles,” says Portugal-Herrera. “Even through things that are really hard, he smiles.”