Dozens of Medical Specialists Rally to Help Newborn Beat Rare Tumor

Dozens of Medical Specialists Rally to Help Newborn Beat Rare Tumor “He’s definitely a survivor. Right away, he had so much spirit and fight in him. My dad says it’s the strong bloodline. But I know we were lucky to find the right doctors,” Espinoza said.

When Sofia Espinoza arrived at her obstetrician’s office in Bloomingdale, IL, for a routine 24-week ultrasound, the second-time mom-to-be thought she knew what to expect.

A few minutes into the appointment, that quickly changed. Her doctor noticed what appeared to be an abnormal growth on the baby’s neck.

“Usually when you go for an ultrasound, everyone’s so upbeat and eager to share the gender of the baby,” said Espinoza, of Addison, IL. “This time there was a scary silence in the room. I felt immediately that something must be wrong. My heart sunk when I was told the doctor wanted to speak with me in her office.”

“Usually when you go for an ultrasound, everyone’s so upbeat and eager to share the gender of the baby,” said Espinoza, of Addison, IL. “This time there was a scary silence in the room. I felt immediately that something must be wrong. My heart sunk when I was told the doctor wanted to speak with me in her office.”

A few days later, a more detailed ultrasound confirmed the mass on the baby’s neck was a cervical teratoma, a rare tumor that affects as few as 1 in 40,000 pregnancies. Though benign, these tumors can hold serious implications for both mother and baby. Doctors informed Espinoza that there was a strong possibility that the tumor would compress the baby’s airway, which meant surviving birth would be an uphill battle.

Frightened and overwhelmed, Espinoza took to the Web in search of more information, but found little. She was referred from obstetrician to obstetrician, each with few answers. Only one thing seemed clear: As the baby grew, so did the teratoma. At 28 weeks, the tumor measured 11 centimeters in diameter, nearly the size of the baby’s head.

Frightened and overwhelmed, Espinoza took to the Web in search of more information, but found little. She was referred from obstetrician to obstetrician, each with few answers. Only one thing seemed clear: As the baby grew, so did the teratoma. At 28 weeks, the tumor measured 11 centimeters in diameter, nearly the size of the baby’s head.

“It was an incredibly stressful time,” Espinoza said. “I was moving from doctor to doctor. They all knew what it was, but they didn’t know what to do next. No one had experienced this. Everyone tried to remain hopeful, but I could see the nervousness in their faces. I had to start coping with the possibility that my baby may not make it.”

Espinoza was told the baby’s best chance for survival would be a specialized cesarean section delivery called an ex utero intrapartum treatment, or EXIT, procedure. During the procedure, a baby is partially delivered to expose the head and neck while continuing to receive oxygen from the mother via the placenta. A high level of skill is required to keep the uterus still and the placenta and umbilical cord intact while a surgeon works to insert a tube into the throat to clear an airway, allowing the baby to breathe once fully delivered.

Espinoza was told the baby’s best chance for survival would be a specialized cesarean section delivery called an ex utero intrapartum treatment, or EXIT, procedure. During the procedure, a baby is partially delivered to expose the head and neck while continuing to receive oxygen from the mother via the placenta. A high level of skill is required to keep the uterus still and the placenta and umbilical cord intact while a surgeon works to insert a tube into the throat to clear an airway, allowing the baby to breathe once fully delivered.

Doctors advised Espinoza that she’d need to travel to a hospital in either Houston or Cincinnati for the complex procedure, a scenario she said added a new level of anxiety. She was relieved to learn the University of Chicago Medicine was able to offer the needed expertise close to home.

“When I heard there were experts here in Chicago that had experience with teratomas, it felt like a tremendous weight was lifted,” she said. “Chicago still seemed like a long drive, but at least I’d have the support of my family.”

“This birth was weeks in planning and required seamless coordination.”

Still afraid and uncertain, Espinoza placed her care and the fate of her unborn child in the hands of experts at the University of Chicago Medicine. With the pending birth only a few weeks away, Fetal Center director Laura DiGiovanni, MD, assembled a multidisciplinary dream team of obstetricians, pediatric surgeons, pediatric otolaryngologists, adult anesthesiologists, pediatric anesthesiologists, neonatologists, radiologists and nurses to orchestrate the complicated delivery. They convened to review images, discuss potential scenarios and draw up a blueprint for the EXIT procedure that included timing, equipment and staffing needs, even the most ideal location for the delivery.

“This birth was weeks in planning and required seamless coordination,” DiGiovanni said. “We all knew the great risks involved with this procedure. We wanted to be able to anticipate any potential problem and be prepared to deal with it. After that initial meeting, there were lots of ongoing discussions. Then we actually brought the entire team together in the OR for a sort of simulation to make certain everything was in place and everyone was aware and comfortable with their position.”

With the dress rehearsal complete, the team was set to carry out the intricate lifesaving mission. The painstaking prep work proved well-timed.

Little did they know that the moment all the planning would be set into motion would come sooner than expected.

Little did they know that the moment all the planning would be set into motion would come sooner than expected. Deborah Boyle, MD

During a regular visit with maternal-fetal medicine specialist Deborah Boyle, MD, the week before the EXIT procedure was scheduled, a monitor revealed Espinoza was having contractions. Feeling no pain, she dismissed any concerns and headed home. At the strong urging of Boyle, Espinoza reluctantly returned to the hospital that same evening for close monitoring.

“There was no doubt we were ready.”

That turned out to be a wise decision -- just a couple of hours after Espinoza was settled in a bed, her water broke. Her baby, John Carlos Guzman, was ready to make a Valentine’s Day debut.

That turned out to be a wise decision -- just a couple of hours after Espinoza was settled in a bed, her water broke. Her baby, John Carlos Guzman, was ready to make a Valentine’s Day debut. Fuad Baroody, MD

Fuad Baroody, MD, director of pediatric otolaryngology, received the page at roughly 3 a.m. In his decade at Comer Children’s Hospital at the University of Chicago Medicine, the renowned head and neck surgeon had performed the critical task of securing an airway during several EXIT procedures and other cases involving complicated airway restriction. He knew no two cases were ever the same.

“I knew this would be one of our more complex cases,” Baroody said. “I was very hopeful that we’d be able to insert the breathing tube through the mouth and avoid cutting the baby’s neck to find the windpipe -- that would be a far more difficult situation and require more time. But we were set to do it if we had to. We’d been preparing for every possible scenario since the moment we learned about this case. There was no doubt we were ready.”

Baroody arrived at the hospital a short time later as the medical team was beginning to assemble. Although they’d been called together earlier than planned, all of the essential players were present. By 6:30 a.m., the delivery was underway.

Baroody arrived at the hospital a short time later as the medical team was beginning to assemble. Although they’d been called together earlier than planned, all of the essential players were present. By 6:30 a.m., the delivery was underway. Kenneth Nunes, MD

When Espinoza was wheeled into the operating room, more than 30 physicians and staff were waiting -- one crew to tend to the mother and another devoted to the baby. Kenneth Nunes, MD, medical director of labor and delivery, joined Boyle for a final assessment of the uterus via ultrasound to determine the ideal location for the incision. They skillfully made an opening directly over the baby’s crown and cautiously withdrew his head to reveal the large mass dominating the right side of his neck and face.

“So like every other facet of this case, all the groundwork left us primed for the task.”

Baroody then went to work executing the intubation and found that the size and position of the tumor presented a challenge: The windpipe was displaced far to the left. To overcome this obstacle, he elected to use the tiniest of light tubes, just 2 millimeters in diameter, to guide a path to the trachea. A test confirmed the breathing tube had been successfully inserted. Baroody gave the green light for the delivery to be completed.

Baroody then went to work executing the intubation and found that the size and position of the tumor presented a challenge: The windpipe was displaced far to the left. To overcome this obstacle, he elected to use the tiniest of light tubes, just 2 millimeters in diameter, to guide a path to the trachea. A test confirmed the breathing tube had been successfully inserted. Baroody gave the green light for the delivery to be completed. Michael Schreiber, MD

John Carlos’ first hurdle now was behind him. Neonatologists, led by executive vice chairman of pediatrics Michael Schreiber, MD, swiftly moved the baby to an adjacent room to check his vitals, place intravenous lines and ensure he was stable enough to make the short trip on the medical campus from Mitchell Hospital to neighboring Comer Children’s Hospital.

Just hours later, John Carlos underwent a delicate surgery to remove the tumor. For Baroody and pediatric surgeon Deborah Loeff, MD, a specialist in congenital anomalies, the biggest challenge was to cut away the large teratoma, now 12 centimeters in diameter, without damaging healthy tissue and the hair-like nerves nearby that would allow the baby to swallow, cry or smile.

Just hours later, John Carlos underwent a delicate surgery to remove the tumor. For Baroody and pediatric surgeon Deborah Loeff, MD, a specialist in congenital anomalies, the biggest challenge was to cut away the large teratoma, now 12 centimeters in diameter, without damaging healthy tissue and the hair-like nerves nearby that would allow the baby to swallow, cry or smile. Deborah Loeff, MD

“It was very difficult working around major arteries, nerves and the delicate structures such as the thyroid grand, especially with everything pushed out of its normal position,” Loeff said. “But our review of the images had been a critical part the pre-op assessment and planning. So like every other facet of this case, all the groundwork left us primed for the task. Despite the complexity, everyone involved worked together as an incredible team with great professionalism.”

“His recovery has been truly amazing.”

In an operation that lasted more than five hours, the tumor was successfully removed. John Carlos proved strong, surviving the surgery and continuing to grow and defy any concerns during his three-week stay in the neonatal intensive care unit. Aside from a scar on the neck and the extra skin once filled by the tumor, there are no traces of the serious medical challenges he once faced. Doctors say he's expected to live a normal, healthy life. Tumor specialists will continue to follow up with him long-term to monitor for reoccurrence.

In an operation that lasted more than five hours, the tumor was successfully removed. John Carlos proved strong, surviving the surgery and continuing to grow and defy any concerns during his three-week stay in the neonatal intensive care unit.  Aside from a scar on the neck and the extra skin once filled by the tumor, there are no traces of the serious medical challenges he once faced. Doctors say he's expected to live a normal, healthy life. Tumor specialists will continue to follow up with him long-term to monitor for reoccurrence.

“His recovery has been truly amazing,” Espinoza said. “When I saw the pictures of him with the tumor, I gasped. You just can't prepare yourself for that. But look at him now. He’s definitely a survivor. Right away, he had so much spirit and fight in him. My dad says it’s the strong bloodline. But I know we were lucky to find the right doctors.

“When I was searching everywhere for positive stories about teratomas this large, there were none. That’s why I want to share my experience with other parents.”

May 2013