Aidan Fraser, a six-year-old boy from Brooklyn whose spinal column was already weak because of a rare neck tumor, fell accidentally three weeks ago, resulting in paralysis of his arms and excruciating pain throughout his body. To prevent permanent paralysis and preserve his ability to breathe on his own, Aidan is undergoing lengthy and delicate surgery in a rare porcedure to stabilize his cervical spine at the Children’s Hospital at Montefiore (CHAM). Doctors began surgery Wednesday morning and while everything went well, decided late in the day to schedule a second surgery next week during which they will complete the procedure.
Aidan’s story is a complex one. His father, Richard Fraser, worked at the World Trade Center and died in the 9/11 terrorist attack. Prior to his father’s death, the then-toddler had his first bout of paralysis, for which he had corrective surgery at another hospital, but the tumor kept growing. For the last few years, Aidan has been under the care of Dr. Adam Levy, a physician at CHAM who is trying to prevent the aggressive tumor from growing.
Aidan lives in Brooklyn with his mother, Suzanne Fraser. “My hope is that Aidan lives and comes out of this surgery happy and healthy and able to run around.”
Rick Abbott, MD, is the pediatric neurosurgeon at CHAM leading a team of specialists – including two neurosurgeons, a head and neck surgeon and a plastic surgeon – in trying to make a mother's hope a reality. “To straighten and strengthen the cervical spine in this case is a very unusual, technically tricky operation,” he said. “First, we have to navigate through the huge tumor which has encased Aidan's spine. Then, using microsurgical instruments, we will remove the front sections of the distorted 3rd and 4th cervical vertebrae and replace them with a rib bone graft (which will be removed in the same procedure) to straighten his spinal column. The team will then fuse the back sections of the weakened vertebrae.” Because of the delicate and complex nature of the surgery it could take more than 12 hours.
Aidan has neurofibromatosis (NF) type 1. Related to this syndrome, he developed a very rare, large tumor, called a plexiform neurofibroma, which over the years has grown into his spinal column and caused several vertebrae in his neck to become distorted in the shape of a reversed ‘c’. As a result, his spinal cord has been stretched and his spinal column has weakened. The NF tumor now encircles his cervical spine and has grown into his left shoulder and chest.
Physician, Aidan’s Father Who Died in 9/11, Were Soccer Teammates
Aidan has a history of near brushes with paralysis, all caused by plexiform neurofibroma, which affects about 25 percent of children who have neurofibromatosis type 1.
The first bout with paralysis occurred five years ago when, as a toddler, his family discovered he could not walk. Emergency surgery allowed him to walk again, but it required removing part of his spinal column, thus weakening it. Aiden was too young to have his vertebrae fused. Just months after his surgery, Aidan's father died in the 9/11 World Trade Center attack.
Before his father died, however, Aidan was chosen by the Children's Tumor Foundation to be featured in a CTF brochure. Adam Levy, MD, a pediatric hematologist and oncologist at CHAM who treats children with tumors, saw the brochure and recognized the father as a former Jericho high school soccer teammate. “Seeing the boy and the father I went to school with registered with me. I made a connection. I thought if I ever did care for Aidan as his physician, it would have special meaning,” said Dr. Levy.
A year later, Aidan was by chance referred to Dr. Levy for consultation and the two immediately developed a personal and professional bond. Over the next few years, Dr. Levy, with Aidan’s mother, helped the boy enroll in a series of national clinical trials to reduce the growing NF tumor. Aidan was on a clinical trial using interferon, which may have stopped the tumor's growth, when he had his fall three weeks ago. He is no longer taking the drug because it can affect wound healing.
After the Fall
After his fall, Aidan was admitted to the Children’s Hospital at Montefiore on an emergency basis by Dr. Levy because his arms were paralyzed and his entire body in pain. Under Dr. Abbott’s direction, Aidan was placed in traction, which restored movement to his arms and reduced the pain. Aidan has been at home, with a stabilizing halo device around his head, awaiting surgery. He was brought to CHAM on November 7, prior to his first surgery.
Neurofibromatosis
According to the Children’s Tumor Foundation, neurofibromatosis (NF1, NF2 and Schwannomatosis) are 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.
When a parent has NF, there is a 50 percent chance of passing on NF with each pregnancy. When there is no family history of NF, the odds are 1 in 4,000 births for NF1 and 1 in 40,000 for NF2.
© 2009 Montefiore Medical Center