Newswise — Tony Pfeifer's parents became increasingly perplexed as the 8-year-old from Santa Clarita, CA, became increasingly forgetful and uncoordinated. He and his younger brother had always been healthy, active and sharp.
"We kept being assured that he was OK. I knew he wasn't OK. He had always been real animated, and he got to the point that he was so spaced and so almost like catatonic at times, he couldn't remember anything. He had been able to ride a bicycle without training wheels at the age of 2 1â„2, and he could not ride his bicycle anymore, couldn't walk up a hill without feeling like he was going to fall down. We knew something was really, really wrong," recalls Rob Pfeifer, Tony's dad.
Rob says he and his wife, Cathy, noticed subtle differences in energy, balance and personality as early as 2001, a few months after Tony's 8th birthday in August. Doctors attributed those early symptoms to natural changes caused by growth and development. But when school started in the fall of 2002 and one of Tony's new teachers asked if he had a learning disability, the Pfeifers immediately arranged for their son to have an MRI. Their anxiety turned to panic when they got the results.
"They called us " from him being fine " and told us that we needed to see a neurosurgeon the following morning, that there was a problem, that he had hydrocephalus "¦ I knew something was wrong but I didn't think he needed to have brain surgery," says Rob, a music composer in the entertainment industry. In fact, based on the results of the MRI, doctors recommended immediate surgery.
Rob says he and Cathy quickly researched the names of top specialists in the area and decided to contact Moise Danielpour, M.D., Director of Pediatric Neurosurgery at Cedars-Sinai's Maxine Dunitz Neurosurgical Institute. A second MRI was performed at Cedars-Sinai, confirming to Dr. Danielpour and his colleagues that Tony had a slow-growing, low-grade tumor blocking the normal flow of spinal fluid, creating a condition called aqueductal stenosis.
Normally, the brain is bathed in spinal fluid that circulates down through the middle of the brain through a narrow passageway called the aqueduct to the back of the brain. It then flows up to the top of the brain where it is reabsorbed.
"The roof and the sides of the aqueduct are areas where these low-grade tumors or growths may form and obstruct the very fine pathway of spinal fluid flow. They are very slow-growing tumors and sometimes they stop for decades. They may have a transient growth potential during puberty or growth spurts but they often stop growing after that," says Dr. Danielpour. "Because of their location, attempts to remove them can cause very significant deficits. In most cases, the best approach is to leave the tumor intact while resolving the issue of fluid buildup."
Although the most common approach has been to surgically insert a shunt to drain off excess fluid, neurosurgical specialists may employ a technique that allows the spinal fluid to bypass the blocked area and be reabsorbed naturally. Minimally invasive ventriculostomy often reduces hospitalization and recovery time as well as risk of complications.
"If patients are not in an area where there is experience and knowledge in this procedure, they would probably have a shunt, which has the risk of malfunctioning, becoming obstructed or becoming infected. We performed Tony's surgery in a minimally invasive manner with a 1.2 mm endoscope. The scope is passed through the brain into the ventricles, or fluid spaces. An opening is created between the floor of the third ventricle and the prepontine cistern, the spinal fluid lakes that are just in front of the brainstem. This allows the fluid to go through a different pathway to be reabsorbed," says Dr. Danielpour, who has performed many similar procedures with excellent success rates.
He says that in cases of aqueductal stenosis, symptoms caused by the tumor may be compounded by pressure from the fluid buildup. "Some problems, can be caused by the location of the tumor, and Tony may continue to have those. We've taken away the issue of the pressure and the stretching of the nerve fibers by the presence of hydrocephalus, and he has improved dramatically. He wasn't able to stand, and now he's running again. He has gotten much better and I think he's going to continue to improve. Nobody can say what deficits he'll be left with, but with kids, there's a great deal of plasticity and the brain has an ability to compensate."
Expecting their son to emerge from surgery with his head wrapped in bandages, the Pfeifers were pleasantly surprised to see a little bandage covering a small hole where the instruments had been inserted. "He woke up and he was coherent," Rob recalls of the operation that was performed on Sept. 23, 2002. "Within a month, he was back at school and he has done really well."
Rob says Tony is enrolled in a school that gives him extra help to deal with his remaining learning disabilities. He continues to have MRIs every six months to a year to evaluate his brain tumor, and is seen periodically by Cedars-Sinai physicians in a variety of disciplines. Institute specialists often collaborate with pediatric, surgical and other specialists at Cedars-Sinai to meet all of a patient's needs.
"We can call anybody and they all talk to each other. We feel really comfortable," says Rob. "I'm extremely thankful for Dr. Danielpour and everybody over there."
"We take a thorough, compassionate, ongoing approach with all of our patients. They're not just a procedure. We have multiple specialists " the oncologist, the neurosurgeon, the pediatrician " and we all work as an integrated team with a lot of thought and effort. These patients will be taken care of in the long term," says Dr. Danielpour.
Rob says Tony is happy, adjusting well, and enjoying the golden retriever puppy he received for his 10th birthday three months ago. "I gotta tell you, this kid looks and feels great right now. I'll take that as long as I can get it."
Cedars-Sinai is one of the largest nonprofit academic medical centers in the Western United States. For the fifth straight two-year period, it has been named Southern California's gold standard in health care in an independent survey. Cedars-Sinai is internationally renowned for its diagnostic and treatment capabilities and its broad spectrum of programs and services, as well as breakthroughs in biomedical research and superlative medical education. It ranks among the top 10 non-university hospitals in the nation for its research activities.
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