The tiny patient, still inside his mother’s womb in August, was struggling against a tumor that was stealing his life. Without intervention, the tumor would win.
The baby’s moms, Angela McCray, and her pregnant domestic partner, Theresa Delgado, were gripped with an array of emotions, from fear and anxiety to disappointment and anger. The abnormal growth stunned the Far Rockaway moms during what was supposed to be a routine obstetric visit.
Within days of the discovery, the parents-to-be were ushered into the rarefied and complex world of fetal medicine.
Now four months old, Caleb is faring well despite nerve weakness in his legs. He gurgles and smiles and sports a knitted hat on cold days that reads: Dude. His birth has been called a miracle by his doctors, who turned to daring procedures, even breaching the inner sanctum of the womb to spare the boy from a potentially deadly defect.
“We had gone in for her 20-week exam. That’s the one where they do a detailed scan,” said McCray, an emergency medical technician with the New York City Fire Department. The ultrasound images revealed an abnormal mass emerging at the end of her son's spine.
“I was overwhelmed, but angry as well. I wanted a healthy baby,” McCray said.
Delgado, a New York City police officer, was emotionally waylaid by the ultrasound: “It was overwhelming, shocking, sad,” the soft-spoken Delgado said, recalling her reaction to the images of her son’s back.
The couple’s obstetrician recommended NYU Winthrop Hospital in Mineola, the only institution in the region where complex fetal surgery is practiced. Doctors there routinely perform surgery on the unborn — Lilliputian-size patients, some weighing barely a pound.
Upon viewing the ultrasound images, Dr. Martin Chavez, chief of maternal-fetal medicine, and Dr. Brian Gilchrist, chief of pediatric surgery, diagnosed a spinal tumor known as a sacrococcygeal teratoma, or SCT.
“It’s benign, but the bulk of the tumor — its size — is what causes problems,” said Chavez, who is also director of fetal surgery. He said the rare mass occurs in one out of every 50,000 pregnancies.
The National Organization for Rare Diseases considers the tumor a rare condition because of the paucity of expectant moms whose babies develop the growth.
The mass emerges at the end of the spine — the tailbone, formally called the coccyx. Once diagnosed, these tumors invariably require surgical removal. Most these days, according to the organization, are discovered before birth during routine prenatal examinations, as was the case for Delgado.
Gilchrist said although relatively small at first, he was surprised not only by the growth of Caleb's tumor, but its complexity. Medical science, Gilchrist added, has yet to definitively answer why these tumors occur.
“I have been doing these cases for 22 years and I have never seen one as extensive as this one,” Gilchrist said.
SCTs develop a multitude of blood vessels that allow them to tap into the fetal blood supply. This somewhat parasitic relationship — tumor and fetus — favors the abnormal mass, the doctors said.
“With a sacrococcygeal teratoma, the blood supply, rather than being returned to the fetus, stays in the tumor, where it swirls around,” Gilchrist said. He explained that because the tumor captures blood that should nourish the fetus, the growth amasses blood and increases in size. Unless tended to, SCTs have been known to cause fetal death.
“It’s a steal,” Gilchrist said of how the tumor is capable of purloining fetal life.
Chavez noted that as the tumor siphons blood, the action causes the fetal heart to work extraordinarily hard in an effort to pump enough blood to sustain life. The effort, he added, can lead to heart failure. Fluids can abnormally increase in the unborn whose circulation is out of balance.
“At first it was really about monitoring it,” McCray said. “It took time to figure out if surgery was an option. But the doctors said it was going to be a very demanding pregnancy.”
At the 30-week gestational point — a full-term pregnancy is 40 weeks — Chavez said it was time to move forward with a delicate procedure that would prevent the tumor from robbing the fetal blood supply.
“He told us the tumor was getting larger in size and his goal was to stop so much blood from being taken from the baby,” Delgado said.
Aside from the tumor that impacted her son, Delgado was at risk of the exceptionally rare condition known as “maternal mirror syndrome,” her doctors said.
The sometimes life-threatening obstetric complication is typified by high blood pressure, fluid retention, swelling of the extremities and protein in the urine. It mirrors fluid retention in the fetus. Documented cases of maternal mirror syndrome have been associated with fetal infections and fetal deformities, studies show.
With mother and fetus at risk, there was no time to lose, Chavez said.
In a dimmed operating room that allows the monitor above him to more sharply magnify his advances into the uterine environment, Chavez prepared to block the tumor.
At 30 weeks, a fetus is about 15 inches in length and accustomed only to a darkened world enveloped in amniotic fluid. Chavez said any maternal-fetal procedure is especially delicate because it involves a patient inside a patient.
“We go on ultrasound guidance,” Chavez said of viewing the procedure on the screen in the operating room as he maneuvers to block a tentacled tumor from stealing fetal blood.
“We sealed them off with energy from a laser,” Chavez said of a diode laser used to coagulate the tumor's blood vessels. “We closed off as many as we could, about 80 percent. Then, after that, when we saw on ultrasound that everything was sealed off, we made sure there was no bleeding.”
The tumor, however, was defiant because by the 35th week it was still stealing blood and was so large that even a cesarean section wasn't immediately possible. Delgado had to be wheeled into surgery a second time for Chavez to drain fluid. Afterward, the C-section was performed.
The surgical procedures, however, didn’t end there.
Urgent surgery was required to save Caleb, who had an enormous tumor still growing at the base of his spine. The plan was to minimize neurological damage.
A multidisciplinary Winthrop team pulled out all the stops: Gilchrist, the pediatric surgeon, and Dr. John Grant, a pediatric neurosurgeon, were part of a seven-hour operation to remove the tumor and repair the areas of Caleb's body that had been invaded by the mass.
“He was actually a little early. He was delivered at 35 weeks. They didn’t want to push it more than that,” McCray said, referring to the C-section delivery. She is expecting a baby girl in June.
“Overall, he’s doing well,” McCray said of her son, who regularly undergoes physical therapy to gain full use of his legs. “It was a very invasive surgery. The neurologist told us it would take six months to a year for the nerves to regenerate.”
Delgado sees the worst of Caleb’s ordeal behind him.
“From where he came from to where he is now,” she said, “I think he is doing great.”