Cami Santee, with her mother, Tami Dobrinski, had surgery to remove the external part of a tumor while part of her body was still in the womb.
Infant Cami Santee had surgery at the Fetal Care Center of Cincinnati to remove the external part of tumor while still in the womb.
By Pohla Smith Pittsburgh Post-Gazette
Chad Santee and his miniature schnauzer buddy Duncan cheerily welcomed two visitors into the dark green mobile home that sits under a copse of bare trees in Bentleyville, a borough about 40 miles south of Pittsburgh.
But Mr. Santee, a 5-foot-11, burly man of 31, realized that it was neither he nor his longtime companion Tami Dobrinski, 33, whom the reporter and photographer/videographer had come to see. To talk with, yes, but to look at, no.
That honor belonged to the long-limbed, 9-month-old girl stretched across her blond mother's lap drinking from a baby bottle.
Her delicate beauty, crowned with wispy, reddish-blond hair and punctuated by round blue eyes, was reason enough for dozens of still photos and minute after minute of video.
But little Cami Santee is more than just a pretty face. She's a miracle -- one for whom Mr. Santee and Ms. Dobrinski thank God and fetal pediatric surgeon Timothy Crombleholme of the Fetal Care Center of Cincinnati. Cami was delivered two months prematurely after he performed fetal surgery to remove an external tumor that was nearly as big as she was. He did additional procedures to restore function to her intestinal and urinary tracts, which were blocked by an internal tumor that filled her abdomen and pelvis.
Dr. Crombleholme, who is renowned for his fetal surgical skills, left out his own talent when asked what allowed Cami to live.
"I think it was part luck, the persistence of her parents and the support of an excellent maternal fetal medical specialist in Paul Speer" at Magee-Womens Hospital of UPMC, he said.
Dr. Speer, part of the team that cared for Ms. Dobrinski in Magee's Fetal Diagnosis and Treatment Center for several months, recommended and referred the mother to Dr. Crombleholme in case open fetal surgery was needed. Magee's fetal center, in a process of development and expansion, does not yet do those procedures. "We will soon," said Dr. Stephen Emery, director of the Magee center and another maternal/fetal medicine specialist who cared for Ms. Dobrinski.
As it turned out, open fetal surgery was not needed, but plenty of other surgical work was done before Cami was delivered.
Her immense tumor was a sacrococcygeal teratoma, more commonly called an SCT or a teratoma. Of unknown cause, the growth usually hangs from the fetus' bottom but sometimes is entirely internal. It is the most common tumor in newborns, yet still occurs in just one of 35,000 to 40,000 births.
Many fetuses die in utero because of complications from such tumors. "It's those fetuses between 20 and 28 weeks' gestation that get into trouble," Dr. Crombleholme said. Some, for example, die of heart failure. Sometimes mothers go into labor prematurely and deliver before the baby is viable. Other times, the tumor ruptures and the fetus bleeds to death.
But, he said, "those that are born have an excellent prognosis. Most tumors are benign."
Cami's tumor was benign, but her prognosis was far from excellent.
"I wasn't sure that baby was going to make it. I was really concerned," Dr. Emery said.
Cami's teratoma was a type III of four categories, which is considered the worst in terms of size, Dr. Crombleholme said.
It was wreaking havoc internally, interfering with the function of her bladder and kidneys and her rectum and intestines.
In addition, he said, by the time of delivery "Cami's cardiac state was marginal. There was some degree of heart failure."
The doctors had hoped to get Cami to 36 weeks' gestation, but she was born April 16 at nearly 32 weeks after three procedures on the fetus -- two bladder taps and insertion of a shunt in her urinary tract -- put her mother into the delivery suite.
Dr. Crombleholme and his team used surgery called an EXIT, which stands for ex-utero intrapartum treatment. It is a type of delivery, but, although an incision is opened in the uterus to permit fetal surgery, it is not a Caesarean section. The umbilical cord remains attached during surgery so the placenta can continue to function and provide fetal support. "The uterus must be flaccid and relaxed," Dr. Crombleholme said. "The mother is heavily anesthetized and deeply relaxed."
In Cami's case, "we lifted out the external tumor and lower extremities, and the rest of her stayed in the uterus," he said.
Along with removing the external tumor after preserving the affected anus and vagina with a skin flap, he did a colostomy to open up her blocked intestinal tract. His team put diverting tubes in her upper urinary tract because she was not urinating. "Her kidneys weren't filtering anything," Dr. Crombleholme said.
All of that done, maternal-fetal specialist William Polzin clamped off the umbilical cord and sent infant Cami to the newborn intensive care unit of Cincinnati Children's Hospital Medical Center, the institution in which the fetal care center is housed. Ms. Dobrinski was taken to Good Samaritan Hospital. Children's, Good Samaritan and University Hospital operate the fetal care center as a partnership.
Ms. Dobrinski was discharged after four days; Cami was hospitalized for 89 days, during which time the surgeons removed the internal tumor. Then they went home to Bentleyville.
They had to return to Cincinnati a week later when Cami developed a urinary tract infection. "She was in the hospital almost a week," her mother said. Last fall they went back to Ohio once again, this time for surgery to remove a rectal blockage caused earlier by the tumor.
She has been wearing a colostomy bag, which is scheduled to be removed later this month.
Up until mid-January, her parents had to catheterize her damaged urinary tract every three hours. "Now it's every four hours," Ms. Dobrinski said, "and the doctors said eventually she'll be able to do it by herself."
Cosmetic surgery on her buttocks may be in the future, Ms. Dobrinski said, because the excision of the external tumor left a large scar.
The doctors also have told her parents she will have to wear braces when it's time for her to walk because nerve damage caused by the tumors led to dropsy in her feet. That's a few months off, though, as the premature delivery has left her a little behind developmentally. She takes occupational, physical and early interventional therapy each week.
Still, by mid-January, she had conquered rolling over, and she threw her arms skyward when the visiting photographer engaged her in the "So Big" game. Her parents beamed.
They were not always so upbeat. The first several months of pregnancy were terrifying.
"It was a pretty depressing time," Mr. Santee said.
It began when the couple, who learned of the pregnancy in late summer 2008, went for Ms. Dobrinski's first ultrasound at Washington Hospital's Women's Health Center in January 2009.
"A half-hour, 40 minutes later, they called and said there was something abnormal on the image," said Mr. Santee, who accompanied Ms. Dobrinski to every appointment. They were told to go to Magee "as soon as possible."
They got an appointment for the next day. The first doctor they met was Dr. Speer, who was the physician in the ultrasound division. He knew immediately what the abnormality was and what the size and location of the tumor meant. He also had the unpleasant task of telling the couple.
"In my initial counseling with her, I had a lot of bad news to give them," Dr. Speer said. The tumor could cause the fetus to die or be born with significant disabilities, and it also threatened Ms. Dobrinski. Among the maternal dangers is a condition called "mirror syndrome," in which the mother mimics the fetus' symptoms. If the fetus went into heart failure, Ms. Dobrinski could get high blood pressure and protein in her urine.
He told them their options: "One, do nothing and watch," he remembered. "Another is terminate the pregnancy; some patients, because of the possibility of death or disability, don't feel comfortable with the pregnancy and terminate ... I spoke also on the potential of a referral to Dr. Crombleholme."
He also told them about the many follow-up ultrasounds and other tests that would be necessary.
"She and her husband said, 'We don't want to terminate. We want to press forward,' " Dr. Speer said.
And so they did. The next several weeks were sheer hell as the couple traveled three or more times a week to Magee for ultrasounds and other tests. Doctors involved included other maternal/fetal medicine specialists, neonatologists, pediatric cardiologists and genetics specialists.
Medical personnel explained that the fetus' kidneys might be blocked and not releasing urine. They thought the amniotic fluid within the amniotic sac, which surrounds and protects the fetus from injury, was getting low in volume.
And every week until Tami's 24th of pregnancy, they were reminded that if they were going to terminate the pregnancy they had "x" number of weeks left in which to do so legally.
"Nothing ever got any better," Ms. Dobrinski said.
But they never lost their resolve to see the pregnancy through.
"We always said if she's meant to be here, she will be here," Mr. Santee said.
Finally, with the bladder, rectum and intestines obstructed, Dr. Speer said he felt Ms. Dobrinski should be somewhere with experts in open fetal surgery. Nearest to Pittsburgh are Children's Hospital of Philadelphia and Cincinnati's fetal center.
"They asked who has the most experience," Dr. Speer said. "I said, 'Dr. Crombleholme developed the programs in Philadelphia and in Cincinnati.' "
Dr. Speer made a referral for them with Dr. Crombleholme, and Ms. Dobrinski and Mr. Santee headed to Ohio in mid-March.
When they got to their appointment they met Dr. Crombleholme as well as his team, who did tests on Ms. Dobrinski and her fetus all day.
"It was definitely uplifting," Mr. Santee said. "Dr. Crombleholme was like a ray of sunshine."
"[Dr. Crombleholme] definitely gave us some hope," Ms. Dobrinski said. "I was 51/2 months pregnant when I got to Cincinnati."
After staying for about a week for more tests, they returned home and resumed appointments at Magee, but eventually they decided to move to Cincinnati to await delivery.
They got there for good on Easter Sunday, April 12, 2009.
Cami was born the following Thursday. She weighed 4 pounds, 11 ounces, including the internal tumor, and was 17 inches long, a pretty big baby considering her prematurity and the presence of the tumor.
"We had all the facts," Mr. Santee said. "We were just positive. If she was supposed to be here, she would. Most don't make it."
The couple's faith was proven to have been justified.
"Sometimes I get so happy I just cry," Ms. Dobrinski said during a recent telephone conversation. She's back to delivering mail part-time, Mr. Santee working at his father's carpet store.
In the background was the sound of Cami, who had missed her nap that day and was grumbling about it loudly.