Montefiore News Releases
New York City, NY (November 16, 2006) -- Six-year-old Aidan Fraser continues to do "very well" after enduring a marathon surgery of almost seven hours at The Children's Hospital at Montefiore (CHAM). The team of pediatric surgical specialists recently completed the second part of a rare, high-risk procedure to stabilize the boy's twisted, tumor-ridden spine, preventing permanent paralysis of his limbs and preserving his ability to breathe on his own.
The CHAM surgeons and his doctor all described his long-term chances as “good,” despite his having a very aggressive form of neurofibromatosis (NF1), the tumor that has caused his spinal problems.
"Aidan's surgery went spectacularly," said Rick Abbott, MD, pediatric neurosurgeon at CHAM, who led the multidisciplinary team that included two pediatric neurosurgeons, a pediatric plastic and reconstructive surgeon, a surgeon with expertise in the spinal column and cord and a head-and-neck surgeon.
"It was a very positive day. Aidan's strength and mobility is already returning to his arms and legs which is a great sign," said Dr. Abbott. "Our team will be watching closely for any remaining issues that need to be resolved after Aidan has a chance to recover more fully from these difficult and complicated procedures."
The surgical team of senior specialists, headed by Dr. Abbott, and the boy's long-time pediatric oncologist, Adam Levy, MD, described in detail the rare and high-risk procedures at a news conference.
Navigating Through The Tumor
“My job is to help other surgeons gain access to the spine,” said Richard Smith, MD, vice-chairman of the Department of Oto-rhinolaryngology – Head and Neck Surgery at Montefiore Medical Center. Dr. Smith does this by carefully making an incision in the front area of the neck and, with microsurgical instruments, dividing the grapefruit-sized tumor in two, then carefully navigating through it to avoid the carotid and vertebral arteries. Dr. Smith uses a special computer guidance system to make certain he avoids both blood vessels and nerves, a technological assist that reduces the time of the procedure by several hours.
Once at the spine area, John Houten, MD, director of Neurosurgical Spine Service at CHAM, used a tiny drill to cut away the front portions of the 3rd, 4th and 5th vertebrae, which the large tumor had distorted into a “c” shape. In addition to weakening his spinal column, the distortion had stretched Aidan’s spinal cord, causing him pain.
Several weeks ago, Aidan fell accidentally and bruised his already stretched spinal cord, causing paralysis in his arms and excruciating pain throughout his body. “Our first goal was to decompress the spinal cord and reduce his pain, which we did successfully by drilling out the bones,” said Dr. Houten. “The second goal was to bring structural stability to the spinal column.”
Rib Serves As Scaffolding for Spine
In the first surgery, David A. Staffenberg, MD, chief, pediatric plastic and reconstructive surgery at CHAM, operated through a tiny incision in the boy’s left rib cage to remove two 10-centimeter lengths of rib that served as 'scaffolding' to strengthen the front and back of Aidan’s weakened spinal column. Dr. Staffenberg inserted one of the rib-bone grafts into the weakened area of the spine. He used the graft to form a bridge from the second to the seventh vertebrae. The rib should fuse with Aidan's spinal column over the next few months and provide additional strength.
“We used bone graft because it comes from the boy’s own body and will not only fuse with his spinal column, but grow as the boy grows,” said Dr. Staffenberg. The excess portion of rib not used in the first surgery was reinserted by Dr. Staffenberg into Aidan’s rib cage, where it remained vital until the second operation. “The incredible thing about this procedure, this way of providing scaffolding for the weakened vertebrae, is that the portion of the rib that was removed and inserted in his spine will grow back again over time,” said Dr. Staffenberg.
In the second operation, the team entered through the back of Aidan's neck and inserted bone between the back of Aidan's skull and back of the spinal column, creating something resembling a "three-legged stool" to better support Aidan’s spine.
Surgery Corrects Spine, Not Meant to Remove Tumor
Drs. Abbott and Levy confirmed that Aidan’s prospects for the long-term were good, cautioning that this is a very aggressive form of NF, called a plexiform neurofibroma. “I have never seen it this bad,” said Dr. Abbott. “Remember, these surgeries are about removing pressure on the boy’s spinal cord and stabilizing his spinal column, not about removing or treating the tumor,” he said.
Dr. Levy has had Aidan on a variety of drugs and chemotherapy over the past few years, and the tumor stopped growing 18 months ago after Aidan began treatment with the drug Interferon. “Aidan has an incredible spirit. He is a happy, fun kid and a great inspiration – all of which help his long-term prospects immensely,” said Dr. Levy.
Neurofibromatosis (NF1, NF2 and Schwannomatosis) is a set of distinct genetic disorders. Individuals with NF are prone to the development of benign tumors that grow on the nerves anywhere on or in the body. NF1 can also affect other tissues, including the bones, skin, blood vessels and eyes.
Aidan will continue to wear a 'halo' brace around his head for several months as the bones fuse. Doctors said it could take up to three years for the bones to completely fuse and heal, and during this time Aidan will be constantly monitored for any problems that may arise because of the tumor.
“There is an incredible team spirit at CHAM," said Dr. Abbott. "When Aidan was admitted, I knew I could not help this boy alone. Within a few days we were able to assemble two neurosurgeons, a head and neck surgeon and a plastic surgeon as a team – all specialists who did their own parts, passing along the baton as the procedures unfolded. The Children's Hospital at Montefiore is a unique environment to work in and it is a privilege to work here."