Geoff Dougherty

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Remaking Maria

By Geoff Dougherty | St. Petersburg Times Staff Writer

When the American doctors arrived in El Salvador, hope traveled along the rutted mountain roads and into Paula Cortez’s dirt-floored home.

Fifteen days after her daughter was born, local physicians attempted to remove the tangerine-sized tumor in the middle of her face. But they failed, leaving Maria with scars across her forehead and a birth defect so stunning that the neighbors were afraid to talk about it.

“The doctors here tried, but they couldn’t help us,” Cortez said. “In order to operate, you need the hand of God.”

Cortez, 24, believes she found it in the Americans, who brought Maria, now almost 3 years old, to St. Petersburg last week. She will undergo surgery Tuesday at All Childrens Hospital to remove the tumor.

The tumor has left Maria so vulnerable to infection that a mosquito bite could kill her. Doctors are amazed that she has lived this long.

Remaking Maria will fix those problems. But the surgery itself is fraught with risks.

Dr. Ernesto Ruas, Maria’s craniofacial surgeon, hopes she’ll return to El Salvador looking like a normal 3-year-old. But building a human face from scratch is akin to assembling a jigsaw puzzle with half the pieces missing and no picture of what the finished product is supposed to look like.

And Ruas will spend much of the 12-hour operation maneuvering around a vein with tissue-thin walls that carries much of the brain’s blood supply. Even a small misstep could lead to a fatal hemorrhage.

“That’s what we call God’s country,” Ruas said.

Maria lives with her mother and father in El Yomo, a village in the mountains about 150 miles from San Salvador. Their home is furnished with two small chairs, a table, and a bed. They cook in the fireplace, and chickens run free outside.

There are no cars, and there is nothing to do at night except sleep, Cortez said.

“We are very poor. When we have some money for food, we have food,” she said. “When we don’t, we don’t.”

During the day, Maria’s father works as a coffee picker. She plays with her brother and another friend, while her mother keeps house.

Maria is the kind of girl who will steal the pen out of your hand because she loves to draw. In a St. Petersburg doctor’s office, she tap-dances across the floor, holding a toy telephone in her ear.

While a physician interviews her mother, Maria outsmarts the child-safe handles on the cupboards and climbs into one of them.

Discovered, she laughs like a girl who knows exactly what kind of mischief she’s up to.

She has charmed the neighbors in El Yomo, as well as her mother.

“She is beautiful, because she is mine,” Cortez said. “They love her a lot because of her sickness.”

But they never mention the tumor.

“It distresses them. It worries them,” she said. “And they don’t talk about it because they don’t want us to worry.”

Cortez is not a worrier. Instead, she dreams about her daughter’s future.

“I want her to be a big person, a doctor or a nurse,” she said in Spanish.

To do that, Maria will need her eyesight. The tumor obstructs her vision, forcing her to turn her head one way or the other to see.

The tumor is the result of a skull that failed to close properly in the womb. Her facial bones and lower forehead never formed, allowing a lump of brain matter to accumulate in the spot that the nose and eye sockets normally occupy.

Ruas believes that doctors in El Salvador probably removed the brain matter, leaving Maria with a sack of fluid connected directly to the inside of her skull. In addition to Maria’s eyesight, the tumor threatens her life.

“If she were to hit something at home and cut (the tumor) her brain contents would be exposed to the outside elements and she would probably die of meningitis. A mosquito bite in the wrong place could kill her,” Ruas said. “It’s amazing that she has survived this long.”

It was Cortez’s dreams that led her to the clinic in Jucuapa, where a group of Tampa-based doctors and medical students set up shop last August.

“So when the gringos go there, it becomes a beacon for hundreds of miles,” said Dr. Roberto Araujo, a Salvadorian native who is now a physician in Pasco County. “It becomes their hope.”

With Maria in tow, Cortez walked for three hours to get there.

“Paula basically came to the clinic thinking we were going to be able to help her,” said David Clendenin, a University of South Florida medical student who volunteered at the clinic. “We didn’t have the people or the equipment to take care of her.”

The doctors turned Maria away. But later, Clendenin found himself wondering if physicians in the United States might be able to help her. A USF faculty member put him in touch with Ruas, who agreed to take the case.

Then Clendenin had to locate Cortez, although he had never known her name or address. He turned to Angela Castro, a dentist in Jucuapa. Castro got photos of Maria that doctors had taken at the clinic. She copied them onto posters, which she taped to lamp-posts all over the region. Finally, someone who knew Maria pointed her toward El Yomo. Once there, Castro found Cortez.

“She thought it was a miracle that people would come looking for her, because the doctors had told her they couldn’t help her,” Castro said.

Cortez agreed to the surgery. Clendenin spent the next few months arranging free airfare and lodging for the family. Over Christmas, Maria’s neighbors in El Yomo said goodbye to her, not knowing whether they would see her again.

“They love this little girl,” Araujo said. “They are all praying that she comes out alive.”

Ruas, 43, the surgeon, grew up as the son of two doctors in central Cuba.

His father worked out of a home office, so Ruas had to walk past waiting patients to reach his bedroom.

“It was not unusual for a farmer to stop by with a big bushel of bananas to pay him,” Ruas said.

Ruas practices a type of medicine that is sharply different from his parents’. The computerized imaging machines that allowed him to diagnose Maria’s tumor didn’t exist when he was growing up. And he spends most of his time in the operating room enlarging breasts and tucking tummies.

But his view of medicine comes from the time he spent in Cuba – the children he saw with untreated birth defects, the kindness his parents lavished upon the less fortunate.

“I remember my mother picking up a child, a little kid off the street, and bringing him home,” Ruas said. “She bathed him from head to toe and gave him a new set of clothes.”

Decades later, Ruas does much the same thing. Once a year, he travels to Central America with a group of plastic surgeons to fix facial birth defects for free. And when Clendenin approached him about Maria’s surgery, Ruas was quick to offer his services.

“I said yes, we can fix that. It won’t cost her a penny,” Ruas said. “It just seemed like the natural thing to do.”

Ruas’ goal is to leave Maria with no signs of the birth defect that now dominates her face.

“My goal is to make her look normal,” he said. “From a conversational distance, you might notice that she has a little scar, or you might not. That’s probably doable.”

To get there, Ruas will have to disassemble Maria’s face, discard the unnecessary pieces and fashion new ones. The operation begins Tuesday morning when Ruas and a neurosurgeon cut Maria’s scalp from ear to ear.

Then the doctors will make a half-moon incision in her forehead bones, arcing along the hairline straight across the eyebrows. At that point, they’ll be ready to open her skull.

From there on in, it’s God’s country.

Scar tissue from the earlier operation has probably surrounded the sagittal sinus vein, grown over the brain’s lining, and glued them both to the frontal bone. Removing the bone without tearing the vein and causing a fatal hemorrhage won’t be easy.

“You move slowly,” Ruas said. “Very slowly.”

With the frontal bone off, the surgeons will determine where the tumor sits in relation to the sagittal sinus.

“We can’t just cut off the tumor,” Ruas said. “Because we’d go right through the sagittal sinus. It’s a big pipe. There’s blood everywhere, and she’s history.”

The vein could go through the tumor, or lay on top or underneath it. The doctors may have to tie it off, forcing the blood to find other routes back to the heart. Or they may simply work around the vein. In either case, the wrong move could cause a fatal rupture.

Then the surgeons will cut through the tube of dura connecting the tumor to the brain. Oncethat’s accomplished, the tumor should deflate, because it will no longer have fluid running into it.

The neurosurgeon will sew closed the dura, sealing Maria’s brain. The tumor will be gone, the risk of infection removed. But she will have a fist-sized gap in the center of her face, where most people have a nose and eye sockets. Ruas, after hours of standing over an operating-room table, will now give Maria the face she has never had.

Because of the size of Maria’s tumor, Ruas believes her eyes are probably set too far apart. So he’ll saw through the orbital bones that anchor the eyes into the skull, move them together and then reset them with titanium plates.

Then he’ll take an inventory, noting which bones are there and which aren’t.

“First you find the normal anatomy. Then you make a judgment about what’s missing,” Ruas said. “Then we have to put Humpty-Dumpty back together again.”

In Maria’s case, he figures that she was born without a nasal bone and is missing the inner third of her eye sockets. To replace them, Ruas will turn to the human body – the source of Maria’s trouble and of the spare parts that will fix it.

The bone that will form Maria’s nose and eye sockets is likely to come from the back of her skull, because it maintains its strength when transplanted.

Then Ruas will draw a template for each missing piece of Maria’s face. Working at a second operating table, he will cut replacement parts to match.

The curvature of Maria’s skull, tightened a bit with a pair of pliers, should provide a natural arc for her eye sockets. The same curve, flattened a tad by the same pliers, should match the gentle left-to-right arc of her lower forehead.

Working like a jeweler in search of the perfect fit between diamond and setting, Ruas will cut the bone, set it in place and then remove it. A few twists with the pliers, a swipe with a tiny file, and then he’ll try it again.

“Sometimes I can spend an hour creating one piece of bone, just chipping away at it,” he said. “You put it in, you don’t like it, you take it out.”

Once perfected, some of the pieces will be fixed into place with tiny titanium plates. Ruas will secure others using a type of craftsmanship that’s akin to tongue-and-groove joints on furniture. The eye sockets, for example, will probably be held in place by other sections of bone that overlap them.

And then comes the most painstaking task of an already difficult day – setting the corners of the eyes into place.

“The eyes are the first thing you look at when you look at somebody,” Ruas said. “A little discrepancy can make you look odd.”

Because Maria’s tumor has displaced the inside corners of her eyes, Ruas will need to disconnect the tendons that anchor them in place. Then he will need to move each of the corners inward.

“If you bring it in too far, she’ll look funny, like she’s almost cross-eyed,” Ruas said. Too far the other way is equally as bad.

Complicating matters are the tendons that fix the corners in place. They are about the length of an eyelash and need to be sewn in exactly the right location to give the face a normal appearance.

For Ruas, that agonizing bit of stitching is the difference between medicine and art.

Even if the surgery is a success, the weeks afterward will be difficult for Maria. She will probably spend several days recovering in the hospital.

“Her eyes will probably be swollen shut. She will be a miserable little girl,” Ruas said.

After she heals, Maria will return to El Yomo with her mother. Clendenin and his fellow medical students would like her to attend college, and have established a trust fund to help pay for her education.

For the moment, though, she’ll still spend her days playing soccer or rocking back and forth in her hammock.

But for the first time, Cortez said, neighbors will feel comfortable talking about her daughter’s face.

“I think they’re going to say that the doctors made her a nose that was perfectly, perfectly good,” Cortez said. “God is going to work a miracle on her.”

January 12, 1998