New York City, NY  (November 13, 2006)  --  After a successful eight-hour operation at The Children’s Hospital at Montefiore (CHAM) last week to straighten and strengthen his tumor-ridden, painfully stretched cervical spine, six-year-old Aidan Fraser spent a quiet weekend playing with his Power Rangers in bed.  According to his visibly smiling physician, Adam Levy, MD, director of pediatric neuro-oncology at CHAM,  “Aidan and his mother are both doing well." 

Aidan will have to endure another lengthy surgical procedure this week to complete the procedure on his spine, which a team of five surgeons are performing to prevent the boy from becoming permanently paralyzed and jeopardizing his ability to breathe. The surgeons and his doctor all described his long-term chances of living to adulthood as “good,” despite having a very aggressive form of neurofibromatosis (NF1), the tumor that has caused his spinal problems.

The surgical team of senior specialists, headed by Rick Abbott, MD, a pediatric neurosurgeon at CHAM, and the boy's long-time specialist, Adam Levy, MD, described in detail the first stage of the operation at a news conference last week.

Navigating Through The Tumor

“My job was 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 accomplished 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 used a special computer guidance system to assure he avoided both blood vessels and nerves, a technological assist that reduced the time of the procedure by several hours.

Once at the spine area, he turned the operation over to John Houten, MD, director of Neurosurgical Spine Service at CHAM.   To reduce pressure on Aidan's spinal cord, Dr. Houten 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

David A. Staffenberg, MD, chief, pediatric plastic and reconstructive surgery at CHAM, then operated through a tiny incision in the boy’s left rib cage to remove two 10-centimeter lengths of rib that were to serve as scaffolding to strengthen the front and back of Aidan’s weakened spinal column.  Dr. Staffenberg then 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 will remain vital until used in next week’s 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.

Surgery Was To Correct Spine, Not Remove the 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, this surgery is 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.

This week, Aidan will undergo an additional 8-10 hours of surgery during which the team, led by Dr. Abbott, will attempt to provide stability to the back portion of the cervical spine.  They will enter through the back of the neck and insert two rib bones between the back of Aidan's skull and back of the spinal column, providing something very much akin to a "three-legged stool" to better support Aidan’s spine.

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.

One reporter at the news conference asked if it were unusual for a team of senior surgeons to work together this way on a single case.  Dr. Abbott replied:  “There is an incredible team spirit at CHAM.  It is a unique environment to work in and a privilege to work here. When Aidan was admitted a week ago, 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 part, passing along the baton as the procedure unfolded.  That does not happen at most institutions.”

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