Anthony Camnetar remembers the first time he was told that he had von Hippel-Lindau syndrome, a rare genetic disorder. He was 12 years old and his mother, who had just picked him up from school, broke the news through tears realizing the health challenges her son would face for the rest of his life.
While he didn't fully understand it at the time, Anthony -- now 23, married, and a father -- would later grasp the significance of that day years later when it came time to remove the dozen tumors that had sprung up throughout his body. He would start down a path that would result in multiple surgeries at different hospitals and long and frustrating periods of recovery.
His journey would ultimately lead him to University of Rochester Medical Center (URMC) urologist Ahmed Ghazi, M.D., who would build an exact replica of Anthony's kidney -- complete with tumors -- allowing him to practice the complex surgery Anthony required long before he went under the knife.
Ahmed Ghazi, M.D., holds a lifelike model of Anthony Camnetar's kidney
A Race Against the Clock
The gene responsible for suppressing tumors is flawed in von Hippel-Lindau syndrome, resulting in uncontrolled cell grow and division. This, in turn, can give rise to tumors in multiple organs which typically begin to appear in early adulthood. A few years ago, Anthony underwent surgery to remove a tumor from his brain. Physicians also spotted multiple tumors in both of his kidneys and his pancreas.
Operating on Anthony's kidneys posed a significant challenge. During this procedure, called a partial nephrectomy, the surgeons attempt to remove the tumors while preserving as much of the healthy kidney tissue as possible in order to avoid the need for dialysis or a kidney transplant. The kidneys are highly prone to bleeding -- despite their small size, they received 20 percent of the heart's blood output -- so surgeons will typically clamp the vessel that supplies blood to the organ before resecting the tumors. However, this makes the procedure a race against the clock as blood flow must be restored within 30 minutes or the kidneys become irreversibly damaged.