French neurofibroma patient fights off infection, flourishes

September 1, 2007

Creteil, France - A man with neurofibromatosis who received a new nose, mouth, chin and partial cheeks in a 15-hour procedure in January is now doing very well, according to surgeon Laurent Lantieri, M.D.

Creteil, France - A man with neurofibromatosis who received a new nose, mouth, chin and partial cheeks in a 15-hour procedure in January is now doing very well, according to surgeon Laurent Lantieri, M.D.

"Everything has gone as planned" except for a drug-resistant cytomegalovirus (CMV) infection that proved difficult to control, he says.

"This infection was detected biologically; it had no clinical impact. But we treated it because the patient was on immunosuppressive drugs" and doctors didn't want the infection to worsen, Dr. Lantieri says.

Functionally, Dr. Lantieri says the patient is slightly ahead of schedule. By early June, the patient had movement in his transplanted tissue, and his nerve regrowth has been so complete that he requires anesthesia for routine biopsies taken every 15 days, Dr. Lantieri says.

"He now has positive movement, which has been confirmed by EMG," he says.

Rather than movement that can be induced by the patient's pre-existing muscles, Dr. Lantieri says, "These are the muscles we transplanted, because all the muscles on both sides under the zygomatic arch had been completely removed and replaced," with the necessary nerves attached to the origin of the facial nerve.

Additionally, Dr. Lantieri says the patient's ability to speak and eat continue to improve.

"There's less and less drooping" around the patient's mouth now that sensation and motility are returning, he says.

Not spontaneous

Though the movements are voluntary, Dr. Lantieri says, "These movements are not yet spontaneous, and he still needs therapy" to reach this goal. "The brain has not yet completely made the connection. It will take some months before it does."

In the next year, Dr. Lantieri says surgeons plan to perform corrective surgery on the patient's mandible, which had been altered by the presence of heavy tumors that began appearing around age 4.

Because neurofibromas don't recur when a surgeon removes them and repairs the damage with autologous skin grafts or flaps, Dr. Lantieri says, "We don't see how neurofibromas could recur" in tissue donated by a patient who didn't have the disease.

"For now," he says, "we don't want to touch anything, because he's still improving daily."

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