Her Nose Knows the Importance of Children's Hospital

By John Kelly
Wednesday, December 10, 2008

One day late in summer, Rahul Shah got up early, breakfasted on a doughnut, then pondered the day ahead. A pediatric ear, nose and throat surgeon at Children's National Medical Center, Dr. Shah had a half-dozen cases that day. Most were what he called "bread-and-butter" cases -- removing tonsils, putting drainage tubes in ears -- but the first one most certainly wasn't.

He had been thinking about the case for weeks, studying the patient's charts and reading the literature whenever he had a few free moments. His patient, a 3-year-old from Glenn Dale named Amber Rudy, was prone to nosebleeds.

But saying that Amber was prone to nosebleeds was to severely underestimate her problem. Her mother, Stacie, likened the experience to turning on a faucet, a frightening gush of blood that sometimes happened as often as three times in a single day. It had gotten so bad that Amber had needed blood transfusions.

"It's really rare to have nosebleeds requiring transfusions," Dr. Shah said. "It bothers me in terms of thinking of a cancer."

Growing inside Amber's left nostril was a vascular lesion, a growth shot through with blood vessels. Although it had been relatively slow-growing, in the weeks leading up to the surgery it had tripled in size. Was it just an abnormal but relatively benign bundle of cells? Or did Amber have a highly malignant cancer known as rhabdomyosarcoma?

Whichever it was, it needed to come out. If Amber had not been a 3-year-old girl -- had been, say, a middle-aged man -- a surgeon might have gone in from the side, slicing open the nostril "Chinatown"-style to expose the growth. But Dr. Shah didn't want to leave Amber with that kind of scar. The reason he had spent so much time reviewing her case was because of how he intended to get at the tumor: from the inside.

On the morning of her surgery, Amber rested in an anaesthetized slumber in Operating Room 10, her body draped in so many blue towels that only her mouth and button nose were exposed. Dr. Shah reminded the others in the room what they were there for: "We're going to do a mid-face degloving for excision of a tumor."

Jan Groblewski, a pediatric otolaryngology fellow, lifted Amber's upper lip and started cutting along the bone at the top of her gums with a tool called a cautery. Dr. Shah reminded him to leave a "cuff," a ridge of tissue that could be stitched when they were done.

"You're going to be [mad] at yourself if you don't leave a good cuff," Dr. Shah said.

When that was done, an incision was made in Amber's septum and threaded with a length of rubber tubing. As Dr. Shah pulled up on the tubing, it became clear that the face is just a mask that floats on our skull. By peeling back the middle of Amber's face, he had exposed the inside of her nose.

Dr. Groblewski inserted a tiny video camera in Amber's nostril, and a reddish blob filled a monitor in the OR. Even with this view, there was something they didn't know: Where was the tumor attached?

"What if we snap it off, take the septum?" Dr. Shah asked his colleagues. "If we take that piece of cartilage, don't we take the tumor?"

But removing the entire septum was a last resort. They continued to probe the tumor. They looked at it on the screen and poked to feel the difference in texture where healthy tissue gave way to cancerous tissue.

"Is that our stalk?" Dr. Shah asked, confident he had found where the growth was attached. "Wonderful. Go straight back on your tumor. . . . Let's go step by step. This is what we waited a month for, guys."

With a careful snip of a pair of scissors, the tumor slithered out. Shah was shocked at the size: as big as a man's thumb.

He turned to a nurse. "Can you call the parents and tell them the tumor's out?" he said. "We're very lucky."

Three Months Later

Pathologists at Children's determined that the growth was benign. Amber went home the next day. Three months later, her mother, Stacie, said, "She's like a brand new kid."

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