July 9, 2014
When plastic reconstructive surgeon Amir Dorafshar first examined his 11-yearold patient’s right thigh, he knew right away he had never seen a leg mass this big in a child—so big the patient could hardly walk and had to deal with classmates’ taunts about having a balloon in her pants. Indeed, Dorafshar had never seen a case in the surgical literature in which a tumor this size was removed from a pediatric patient’s limb. But that’s what he was being asked to do because no other surgeon the family had approached would consider surgery.
“No one wants to do these surgeries so these patients have nowhere to go,” says Dorafshar. “The reality is, people are being shipped from place to place with no hope. We want to say we’re the hope.”
In similar cases in which patients have some mobility, Dorafshar says he wouldn’t consider surgery—the risks are many and potentially severe. But this patient suffered the rare progressive disorder hemihypertrophy, in which one side of the body abnormally grows more than the other. In other words, the mass would get even larger and pose an even bigger risk to what little mobility she had remaining.
“Because this is disabling, I’m going to have to debulk it,” Dorafshar says.
The challenge of the surgery was partly due to the makeup of the mass—muscle, fat and soft tissue all intertwined and presenting a marbled, difficult-to-dissect structure. While Dorafshar wanted to preserve as much muscle—and leg function—as possible, he knew some of the entangled muscle would have to be removed.
“But we’ve gone through the thinking process and over the scans with our muscularskeletal radiologists, we’ve done all of our homework,” Dorafsar says. “Still, we won’t know exactly how much of the mass we can safely take out until we get into the OR.”
Another surgical risk was damage to fragile blood vessels already stretched thin by the mass—and to nerves that trigger thigh muscles and flex the knee. Nip these vessels or nerves and Dorafshar’s patient could have less function coming out of surgery than she did going in. To reduce that risk, Dorafshar asked pediatric orthopedic surgeon and nerve-sparing expert Paul Sponseller to join him in the OR. Sponseller is well experienced in operating around the sciatic nerve that stimulates hamstring muscles on the back of the thigh.
“The hardest part of this surgery is dissecting the muscle and preserving the blood and nerve supply,” says Dorafshar. “If damaged, a whole muscle group could die.”
There could be post-operative complications, as well, including problems with wound healing given the size of the wound. But Dorafshar felt certain he and the team would be successful in both the surgery and recovery. Part of that confidence, he says, comes from consultation with the multidisciplinary vascular anomalies group at Hopkins, which meets monthly to review such complex cases.
“We have the whole shebang here—the group and our deep experience in treating a constellation of vascular anomalies illustrate the beauty of Hopkins in treating these patients,” Dorafshar says. So, how did the surgery go?
“You could see that the mass was a monster, but we enhanced both the appearance and function of her leg,” says Dorafshar. “The patient said she felt lighter and two inches taller, and that she could finally put her legs together.”
And, of course, Dorafshar adds, she would no longer have to face classmates teasing her about having a balloon in her pants. The balloon was gone.