Specialists at the Federal Center of Neurosurgery in Novosibirsk
, Russia, conducted a unique operation on a patient with a rare and little-understood condition called Gorham’s vanishing bone disease. Surgical training before the difficult procedure was carried out on 3D printed mock-ups.
58-year-old Alexander Tolstikov did not immediately realize the seriousness of his ailment. It started with a barely noticeable dimple on the back of his head, which didn’t cause any discomfort. Over five years the depression grew in size to a point, where the occipital bone has almost disappeared, so the man could no longer keep his head up. Any movement caused severe neck pain, and Alexander found himself effectively immobilized.
Medical examination revealed massive osteolysis in form of Gorham’s vanishing bone disease. The disease is characterized by massive proliferation of blood vessels, gradually replacing bone tissue. This is an extremely rare condition: as surgeons explain, only about two hundred cases are known to this day, while the etiology of the disease remains a mystery. Moreover, there are no recorded cases of Gorham’s disease affecting the occipital bone. Faced with a unique case, surgeons set about developing a unique method of treatment.
“X-ray images showed absence of the occipital bone, which could have been an indication of a past surgery or an inflammatory process, such as meningitis, but neither was in this patient’s medical history. Furthermore, the second and third vertebrae of the cervical region had shifted. That’s why he could not hold his head up – it was simply lacking any support. The problem was that the deformity of the spine had to be corrected first through halo traction, so that the head would stay up, and the patient could walk and see normally. Under normal circumstances that would imply a strong connection between the vertebrae and the occipital bone, but in this case the bone was not there to begin with, therefore standard treatment was unsuitable,” explained neurosurgeon Dr. Vladimir Klimov.
Two months prior to surgery Alexander was fitted with a custom-made traction device, which he jokingly referred to as “antlers”. Meanwhile the medical team made preparations for a complicated surgical procedure: afflicted areas of tissue would have to be removed and then a number of titanium alloy rods would be implanted into the skull and vertebrae, securely connecting patient’s head to his spine. Surgeons decided against closing the bone gap for the time being, since there is no certainty that the decease will not recur after the treatment. The matter was complicated further by the need to exercise extreme caution due to the immediate proximity of brain and spinal cord tissue. Even a tiny mistake could easily lead to paralysis or even death. In order to minimize the risk, surgeons practiced the procedure extensively on special 3D printed mock-ups of patient’s cranium and spine, modeled on tomographic images.
The operation itself took eight hours to complete and is so far considered a success. Alexander is still recovering, but already walking and even smiling. Since the treatment is experimental, doctors will keep tabs on the man over the next eighteen months to make sure his condition does not worsen.