This little girl is 10 years old and has a generalized disorder of growth that may be Proteus Syndrome. She has a progressive scoliosis that has progressed quickly in the last two years to 70 degrees – that is a very significant curve. The curve is stiff and even with maximum pressure only reduces to 60 degrees. I made a decision to treat her surgically, talked to the parents and after careful discussion consideration they agreed to let this American doctor operate on their daughter.
We did exhaustive testing, x-rays, bending x-rays, CT, three dimensional reconstructions of the spine from the CT, MRI, blood tests…we arranged for the sophisticated instruments that would be needed to implant and correct the curve and fuse the spine. We arranged for special spinal cord monitoring to help avoid catastrophic neurological injury. The cell saver was used to salvage the shed blood and allow it to be given back to the patient.
Surgery day came and as anticipated it was a challenging case – I entered the operating room at 0900 and left at 1800. There were undiagnosed spontaneous fusions at the apex of the curve that required bone removal from the spine. The stiffness could only be overcome by fastidious releases of all the connecting ligaments and insertion of screws and rods.
The surgery went beautifully and she was back in her room at 2100 the day of surgery. The following day she was talking to me – The family relieved and happy.
By David P. Roye M.D.
