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Sag Harbor man gets pioneering brain-tumor surgery

Dr. Alfred-Marc Iloreta of the Skull Base Surgery

Dr. Alfred-Marc Iloreta of the Skull Base Surgery Center at The Mount Sinai Hospital in Manhattan on Oct. 7, 2016, standing next to a scan image showing a tumor, in yellow, that once was developing in the skull of James O'Shaughnessy of Sag Harbor. Credit: Craig Ruttle

While stocking shelves in his Southampton shop last year, welder and blacksmith James O’Shaughnessy was struck on the head by a heavy-gauge rod, a blow that left him dazed.

Doctors at nearby Southampton Hospital diagnosed a concussion, but their MRI images also unmasked the unexpected — a tumor of unusual shape and size developing at the base of O’Shaughnessy’s skull, threatening his brain.

“They believe that it had been there for quite a number of years, growing,” said O’Shaughnessy, 44, of Sag Harbor.

Although benign, the tumor — called a schwannoma — can snuff out hearing, invade the brain and cause tremendous, even deadly pressure in the skull.

What happened next, he said, was a medical odyssey that consumed months and involved hospitals and doctors who were unable to help until he found a team at The Mount Sinai Hospital in Manhattan that was capable of removing the growth without a single external incision.

O’Shaughnessy landed in medicine’s most advanced arena, the emerging technological frontier where a tumor is modeled by way of a 3-D printer before surgery, providing the physician with a facsimile of the actual growth.

This method of approaching tumors also includes GPS-like surgical technology, allowing the doctor to circumnavigate unexplored corners of a living skull and the secret byways of the brain itself.

Both techniques aided O’Shaughnessy’s doctors at Mount Sinai, where parts of the large, serpentine tumor were removed through an incision in the roof of O’Shaughnessy’s mouth and the rest through his nose.

The operation, which took more than eight hours, was performed in April, a full year after the rod struck his head.

The GPS-guided surgery, usually performed by a neurosurgeon, marked a groundbreaking effort by an ear, nose and throat specialist, Mount Sinai officials said Friday.

O’Shaughnessy’s minimally invasive operation differed dramatically from conventional surgery for such a difficult-to-reach tumor — an ordeal that could have taken up to 15 hours, his doctors say.

Dr. Alfred-Marc Iloreta of Mount Sinai’s Skull Base Surgery Center, the surgeon who removed O’Shaughnessy’s tumor, said the abnormality was growing near “sensitive real estate.”

“His tumor was located at the base of his skull, right below his brain and close to his throat. It’s difficult to access that space,” said Iloreta, who used a 3-D printer to produce a model of O-Shaughnessy’s tumor and the structures it threatened.

Three-dimensional printing is a process — also known as additive manufacturing — in which a digital 3-D image is turned into a physical object, layer by layer.

Having a reproduction of O’Shaughnessy’s tumor, as it was arrayed in his head, allowed Iloreta to map out his surgical strategy and choose the most appropriate instruments before his patient was wheeled into the operating room.

Once in surgery, the GPS system provided real-time navigational guidance through O’Shaughnessy’s head.

“Because of the technology, we didn’t have to touch his brain or remove his jaw or any of the bones in his face,” Iloreta said.

The American Brain Tumor Association in Chicago estimates that schwannomas account for about 8 percent of all primary brain tumors. The growths derive their name from nerves known as Schwann cells, which are involved in the production of myelin, the insulating substance that protects nerves. The exact cause remains unknown, although some can result from a gene defect.

O’Shaughnessy described a circuitous path to the team at Mount Sinai. His first stop was a Long Island ear, nose and throat specialist, who said the tumor was beyond his level of expertise.

That doctor recommended Memorial Sloan Kettering Cancer Center in Manhattan. But after seeing a physician there once, O’Shaughnessy said he was told the institution didn’t accept his insurance.

A doctor there, O’Shaughnessy said, did explain how the center approached schwannomas.

“I was told I had two options: One was the cranial surgery, which was to remove the top of my head,” he said.

“I was shocked at the time they told me. It was so barbaric. The other option was radiation. Six weeks, six days a week, 15 to 20 minutes a day,” he said. “After I did some research on that, I didn’t want the radiation, either.”

The Long Island specialist ultimately recommended Mount Sinai, O’Shaughnessy said.

Dr. Eric Genden, who chairs Mount Sinai’s department of otolaryngology and is co-director of the Skull Base Surgery Center, said O’Shaughnessy’s tumor was remarkable for its size, which he estimated to be about 8 centimeters.

He said even though schwannomas are benign, their growth patterns are not.

“They can cause significant, permanent disability,” Genden said. “When they occur at the base of the skull, any nerve that’s in the way, any area such as the throat, vision, the brain itself can be impacted as they grow.”

He said the tumor caused O’Shaughnessy to lose his hearing in one ear, but O’Shaughnessy did not initially realize his hearing deficit could be traced to a growth in his head. After the tumor was removed, O’Shaughnessy’s hearing returned.

O’Shaughnessy, a Limerick, Ireland, native who has lived in the United States for 21 years, said he was unaware his operation involved such innovative technology.

He still works at his shop, Anvil Ironworks, fashioning white-hot steel, bronze, brass and iron into gates, fireplace screens and chandeliers.

O’Shaughnessy, who speaks with the indelible lilt of the Emerald Isle, said the tumor has helped him realize what’s most important in life.

He said he cherishes the weeks his mother spent with him during his weekslong recuperation at home, and deeply appreciates having close friends.

“This has been literally life-changing for me,” he said. “I now have a totally different outlook on life.”