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Transferring a Nerve, Saving a Smile

Rebecca Grasso experienced facial paralysis following a complicated procedure for a brain tumor. Today, thanks to a series of nerve transfers at Mass Eye and Ear, her face is close to resembling what it once was.

Rebecca Grasso had just woken up from surgery when she first met Nate Jowett, MD, PhD, FRCSC. His introduction was a surprise; Rebecca did not recognize him from her original team of doctors tasked with shrinking a massive tumor compressing her brainstem.

When he mentioned nerve repair as his specialty, Rebecca could sense something wrong. One look in the mirror revealed the left side of her face wasn’t moving.

“I’m someone who had the nickname ‘smiley’ her whole life,” Rebecca, a 30-year-old physical therapist from Albany, NY, told Focus. “To not recognize that smile—let alone that person staring back at me—was frightening.”

Her facial paralysis could not have come at a worse time. Rebecca had planned to marry her fiancé in a matter of months and had already endured blow-after-blow to her once-perfect health. The tumor in her head manifested from a rare hereditary condition called neurofibromatosis type 2, or NF-2, which had robbed her of her hearing just two years prior.

But Dr. Jowett wasn’t ready to let the condition take away her facial expression. There was still an opportunity to save Rebecca’s smile through a surgical technique known as a nerve transfer, but time was of the essence.

A rare disease that begins deep in the skull

The signs and symptoms of NF-2 vary from person-to-person, according to the National Institutes of Health. In Rebecca’s case, she did not experience symptoms until high school. What began with difficulty listening to her teachers cascaded into an inability to hear her television on its loudest volume. Then, during her sophomore year of college in 2019, she noticed a mysterious ringing in her ears.

“I would constantly ask my classmates, ‘Did you hear that?’” Rebecca explained. “When they had no idea what I was talking about, I knew I needed an MRI.”

Rebecca’s tests revealed non-cancerous tumors—known as schwannomas—on multiple nerves in her head. Two of the tumors had grown on nerves vital for hearing and balance. By the time she finished college and graduate school, Rebecca had lost nearly all her hearing, and the tumors had begun compressing both sides of the brainstem. To halt the growth of her tumors and qualify for an auditory brainstem implant, she enrolled in a clinical drug trial at Massachusetts General Hospital. She would also undergo a series of operations to shrink, or debulk, both tumors.

Rebecca first underwent surgery closer to home in New York to debulk the tumor on the right side of her brainstem. The tumor on the left side, however, continued to grow at a rapid pace, further compressing the brainstem and threatening her life. Debulking the second tumor would risk collateral damage to surrounding nerves, and the facial nerve in particular.

Given the massive size of Rebecca’s tumor, the odds were stacked against her by the time she arrived at Mass General for her second surgery. While the procedure successfully shrank her tumor and relieved her brainstem compression, she awoke to facial palsy.

Rewiring a smile

In some instances, facial nerves can spontaneously recover from injury. During the second surgery, surgeons preserved Rebecca’s facial nerve, hoping the nerve would recover over the next several months. Dr. Jowett, aided by Kerry Shanley, PA-C, and Jenn Mangarelli, RN, monitored Rebecca’s progress and prepared to schedule a microsurgical nerve transfer if her face remained paralyzed.

Nerve transfers re-route nerve fibers from a less critical nerve to a more important target, with the goal of restoring important sensations or movements. According to Dr. Jowett, nerve transfers should be performed within 12-to-18 months after the onset of facial paralysis for optimal results.

“Time is muscle,” Dr. Jowett said. “The efficacy of nerve transfer procedures to restore important movements, such as smiling and blinking, decreases with longer durations of paralysis as muscles become less receptive to neurotization.”

Eight months after her facial palsy started, Rebecca showed no sign of improving function on the left side of her face. So, in an initial procedure, Dr. Jowett worked with fellow Mass Eye and Ear surgeon Tessa Hadlock, MD, to re-route fibers from a nerve branch controlling jaw movement to the branch controlling Rebecca’s smile. Several months later, when Rebecca had not recovered adequate function in the remainder of the left side of her face, Drs. Jowett and Hadlock prepared for a second nerve transfer.

At the height of the COVID-19 pandemic, Rebecca woke up at 2:00 AM for a four-hour drive to Boston from upstate New York. This time, her surgeons opted for a different type of nerve transfer that re-routed nerve fibers responsible for controlling Rebecca’s tongue. The procedure—a hypoglossal nerve transfer—came with its fair share of risks; re-routing too many nerve fibers could result in a paralyzed tongue, while re-routing too few fibers would result in procedural failure.

To minimize the risk of complications, Dr. Jowett utilized a novel surgical approach he and his team had employed on several prior cases. The approach modified a conventional hypoglossal nerve transfer by re-routing additional nerve fibers responsible for controlling small muscles in her neck. Connecting these additional fibers to the facial nerve would, in theory, reduce the proportion of hypoglossal nerve fibers required to restore symmetrical muscle tone in Rebecca’s paralyzed face.

Only time would tell if it would work for Rebecca.

The return of “smiley”

A lot has changed since Rebecca’s last operation. Rebecca and her fiancé bought their first home together and celebrated their wedding on a rescheduled date. Joining the couple for the big day was a familiar sight: The earliest signs of “smiley” slowly—but surely—returning.

“I’m building back my smile using the nerves I use to bite and flick my tongue,” said Rebecca. “It took weeks to see results, but, since my second surgery, I’ve seen my face return a little more each day.”

According to Dr. Jowett, it can take up to two years for a patient to see the full effects of nerve transfer procedures. To Rebecca’s surprise, the surgeries had improved her smile within a few months and her facial tone within a year. After several appointments with Facial Nerve Center physical therapist Mara Robinson to optimize her surgical outcome, Rebecca was ready to schedule her wedding for June 2021—well ahead of her recovery schedule. While her NF-2 still leaves open the possibility of her tumors returning, Rebecca rests assured knowing she has a team of world-class facial function experts looking after her.

You don’t want just anybody operating on your face.” Rebecca said. “You want the best of the best. Mass Eye and Ear really is on the cutting-edge of these nerve procedures, which made seeing Dr. Jowett such an easy choice to make.”

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