Leila Gatmaitan's Story
Celebrating success with our tiniest patients
When Lori and Mike Gatmaitan went to their 20-week ultrasound appointment, like most expectant parents, they were eager to find out the baby's gender and see their new child for the first time. The pregnancy with their son, Dane, two years earlier was completely normal, so they had no reason to expect anything unusual. But during the routine appointment, when the ultrasound technician became very quiet, left the room, and then returned for more images, Lori knew something wasn't right.
"The technician said they wanted additional pictures of the baby's chest. It was a definite red flag," remembers Lori. "My OB called 45 minutes after the ultrasound to let us know our baby girl had a large mass in her chest. We had no idea what to think–it was overwhelming."
The Gatmaitans were referred to the Fetal Diagnosis and Treatment Center (FDTC) at the University of Michigan. They met with Dr. Marjorie Treadwell, director of the Perinatal Assessment Center, who diagnosed the Gatmaitan's daughter with a condition called congenital cystic adenomatoid malformation (CCAM).
A CCAM is an abnormal growth of lung tissue. Experts don't know what causes the malformation, but they do know that CCAMs can get infected or become malignant later in life and the cystic tissue will never function as normal lung tissue. Although most CCAMs don't cause problems during pregnancy, large lesions can be fatal.
"They actually told us that our baby's chest mass was the largest they'd ever seen at U-M. It would definitely require surgery–we didn't even know it was possible to do surgery on a fetus," says Lori.
The Gatmaitans met U-M pediatric and fetal surgeon Dr. George Mychaliska, director of the FDTC, to discuss options. "At first, they thought our daughter's best chance for survival was to remove the mass in utero, but it's risky because once the uterus is opened it can trigger preterm labor, so Drs. Mychaliska and Treadwell opted for a new treatment consisting of maternal steroids."
Leila and her family
"At the FDTC, we are working to better understand lung development–how it happens, what goes wrong in certain situations, and how we can either protect or stimulate the lung to grow and function normally. Philanthropic support is vital to our efforts and will ensure that our research quickly translates into improved care for other babies like Leila." – Dr. Mychaliska
By the 26th week, the ultrasound showed that the mass was occupying the entire left chest. "It was hard to know whether she had any lungs at all–the mass was blocking all but the smallest amount of the left lung. But she was otherwise growing and doing well, so Dr. Mychaliska thought the best option was to perform fetal surgery at the end of the pregnancy," recalls Mike.
Ex Utero Intrapartum Therapy, also known as an EXIT procedure, is a highly modified cesarean delivery in which surgeons partially deliver the baby and perform surgery while the baby is still attached to the placenta. When the surgical procedure is completed and the baby is stable, the umbilical cord is cut and the baby is delivered just as with any other C-section.
"We had no idea what an EXIT procedure was, but Dr. Mychaliska explained the whole thing to us–he actually drew a detailed diagram on the paper covering the exam bed," says Lori. "Because she was still developing and doing pretty well, open fetal surgery during pregnancy wasn't necessary. The EXIT procedure would allow doctors to sustain the pregnancy for as long as possible and then do the surgery just before delivery."
Because large CCAMs may prevent normal lung growth, babies with the condition may require ECMO to prevent pulmonary failure at birth. ECMO–extracorporeal membrane oxygenation– is a heart/lung bypass machine, which mimics the normal gas-exchange function of the lungs, providing oxygen and removing carbon dioxide. ECMO was developed by U-M critical-care pioneer Dr. Robert Bartlett.
"Dr. Mychaliska planned to put our baby on ECMO, since her lungs might be too small after the mass was removed," says Lori. "ECMO is a highly invasive treatment, so we were obviously worried; but Dr. Mychaliska and Dr. Treadwell made sure we understood exactly how it worked and prepared us for what to expect."
As the pregnancy continued, the Gatmaitan's baby began retaining fluid and large amounts of amniotic fluid started accumulating inside Lori's uterus. "The mass in our baby's chest was interfering with the normal ebb and flow of amniotic fluid. I was huge, which was uncomfortable, but the stress on the baby was really concerning. The doctors were keeping a close watch on the fluid building up in her head and body–she looked like a Michelin baby," recalls Lori.
"The CCAM was obstructing the esophagus, which caused amniotic fluid to increase, and compression of the heart was causing both heart failure and fluid accumulation in the baby," says Dr. Treadwell. Because of concern for preterm delivery, Lori received another dose of steroids.
Dr. Mychaliska holding Leila
"Then on a Monday during the 33rd week, we went in for an ultrasound and she looked completely normal. All that extra fluid in her body was gone," says Lori, still amazed. "It was just so incredible– no one could believe it. We had all been so worried about her and then it all just resolved, just like that. In a pregnancy with lots and lots of bad news, it was amazing to have such a great thing happen."
The following Thursday, Lori and Mike headed back to U-M for another routine ultrasound. "It was weird, we hadn't meant to, but for some reason we showed up for our ultrasound appointment three hours early. Our entire team of doctors was meeting about our case, so one of the nurses took us on a tour of the neonatal intensive care unit (NICU), where the baby would recover after the surgery. Then we went in for the ultrasound early," says Mike.
"Looking back on it now, it was really incredible that we got there early. I think I must have known something wasn't right," adds Lori. "The ultrasound showed non-reassuring heartbeats. At first they thought the machine was broken, but the next thing I knew I was being admitted to the hospital and we were told they would deliver the baby that night."
By 7:00 pm, Lori and Mike were waiting for the team to assemble–38 people in all, divided into a team for Lori and a team for the baby. Dr. Clark Nugent would assist in the EXIT procedure and open the uterus and Dr. Mychaliska would operate on the baby. "Before they could operate, they had to drain off most of the extra amniotic fluid that had accumulated in my uterus," remembers Lori. Shortly after the procedure began, Leila's heart rate slowed and she required immediate delivery.
At 8:22 pm on January 14, 2010, Leila Gatmaitan was born. The team was prepared for all possibilities and had an additional operating room ready and waiting. Weighing in at just under four pounds, Leila was whisked away to another operating room. Dr. Mychaliska removed a four-by-four-inch mass from her chest, which filled nearly the entire left chest.
"Her surgery went well and, by some miracle, she didn't need ECMO–her lungs were tiny, but strong enough to breathe with support from a ventilator. The first three days were touch-andgo for her, but in the end she pulled through," adds Mike.
Altogether, Leila remained in the NICU for 41 days. But after close monitoring and dozens of tests that ultimately came back normal, Leila was able to go home. Now, at 14 months, Leila is a happy and healthy baby. Her lungs are growing and she has a bright future.
"We are so grateful to Dr. Mychaliska and the whole team at the FDTC. Through this process, I met so many other women online with CCAM babies who believed that there was no hope for their babies– they had no medical options in their geographic areas and no information about where to go," says Lori. "There are only a handful of places in the world with the expertise in fetal diagnosis and treatment that is required for babies with large CCAMs. We feel blessed to have had access to the medical professionals at U-M."
Improving Outcomes for our Tiniest Patients
To honor Dr. Mychaliska and ensure that his lifesaving research continues, for the benefit of thousands of other children, the Gatmaitan family has helped establish the Mychaliska Research Gift Fund. The Mott Children's Hospital at University of Michigan is one of just a handful of hospitals in the nation that can perform fetal surgeries. To ensure that the field of fetal diagnosis and treatment continues to advance, we hope you'll consider supporting this pioneering research.