“We have the means and the teams here to take care of these babies”

By Maureen Gilmer, Riley Children’s Health senior writer, mgilmer1@iuhealth.org

Someday, little Rapha Born will hear the stories about his birth at Riley Hospital for Children.

Or, more precisely, he’ll hear about the surgery he had before he was even born.

Rapha, now 2 months old, underwent a bilateral chest shunt procedure in utero at about 25 weeks’ gestation. His mom, Tess Born, was seen at the Fetal Center of Riley Children's Health/IU Health by Dr. Hiba Mustafa, director of fetal surgery.

The reason for the surgery was to drain fluid that was accumulating in his chest cavity. Failure to do so could have ended in Rapha suffering a condition similar to congestive heart failure.

Instead, Rapha was born healthy in November, and today he is “doing all the baby things,” his mom said.

“He’s cooing, he gets really excited when his sister comes to help with bottles and diaper changes, he sleeps really well, and he’s eating more than I thought was possible for a tiny man.”

Tess and her husband, Tim, who live in Mishawaka, Indiana, were grateful for the maternal fetal medicine team at Riley that stepped in to help, even though that meant the couple had to travel three hours down to Indianapolis to get the care mother and baby needed.

“My husband and I consider Dr. Mustafa an angel on Earth,” Tess said. “She is absolutely fantastic, as is Ari Duszynski.” (Duszynski is Riley’s fetal care coordinator.)

After repeated scans to track the fluid buildup, Dr. Mustafa explained to the couple the risks of doing nothing compared to the risks associated with fetal surgery and placement of a fetal chest shunt.

Tess’ baby was suffering from a condition called fetal pleural effusion, which occurs in less than 1% of pregnancies.

Fetal pleural effusion can cause damage to the development and function of the heart and lungs. Fluid can cause the heart to shift in the chest and make it difficult to pump blood, leading to fetal heart failure, also called hydrops.

It is most often caused by infection, anemia, fetal genetic conditions, fetal anomalies such as congenital heart disease, and abnormal blood vessel or lymphatic development. Dr. Mustafa suspects the latter caused Rapha’s condition, as there was no genetic finding.

There are three scenarios that make it critical to intervene in a pregnancy and place a shunt or chest tube, Dr. Mustafa said: Fluid on one side is so severe that it is pushing the heart to one side; both sides have so much fluid that it is preventing proper lung growth; or baby is suffering from a condition called hydrops, where fluid is affecting the heart’s function, leading to heart failure in many cases.

In Rapha’s case, both sides were severe, and the lungs were so small doctors worried that if left untreated, it would affect lung development or result in hydrops/heart failure.

Dr. Mustafa delivered the news to the couple calmly and thoroughly, Tess recalled, giving them a day to process the information and decide how they wanted to proceed.

“My husband and I went home, and we were both so emotional, we called her and asked a bunch of questions,” Tess said. “She was very calm, explaining to us what was happening, and we decided to go through with it. She was with us every step of the way, making sure I was well-informed. She’s just incredible. I could not imagine doing this without the help of Dr. Mustafa.”

Tess was awake during the hourlong procedure, while Dr. Mustafa talked her through every step. She was even given the option of watching on monitors but declined.

The shunts in question are small (about 2 inches long), flexible, plastic tubes placed in the baby’s chest during pregnancy. One end of the tube goes into the baby’s chest cavity, and the other end remains in the amniotic fluid in the uterus. The tube helps the fluid move from inside the baby’s chest out to the amniotic fluid.

“We recommended delivery at Riley just because these babies might require surgery after birth, and we have the pediatric surgery expertise here, but baby Rapha did not need surgery post-delivery,” Dr. Mustafa said.

There are risks associated with fetal surgery, primarily pre-term labor, she explained, so timing is critical. But not doing the procedure means there is a high risk for loss of the baby. Tess was induced at 37 weeks at Riley, delivering 5-pound, 14-ounce Rapha vaginally. Today, Rapha weighs 12 pounds, 12 ounces.

“I have two little scars, but if anything, they are just reminders of how awesome the procedure went and how well he’s doing,” Tess said.

Many practitioners consider hydrops an untreatable condition. Dr. Mustafa said, but in some cases, it is treatable, particularly when there is no genetic association and the condition is caused by primary pleural effusion, in which case the team can offer shunt placement, or if caused by anemia, can be treated with an intrauterine transfusion.

“There are things we can do – it does not have to be a lethal diagnosis,” the physician said. “We’ve proved that in so many of these cases.”

As the only fetal medicine center in the state, Riley offers a multidisciplinary spectrum of fetal medicine and surgery, she added.

“There is no patient that gets to us who will be referred outside because we have the full spectrum of services and innovative therapies. We have the means and the teams here to take care of these babies.”

For Tess and Tim, sharing their story is something they are eager to do, Tess said.

“Truly anything we can do to help other families going through something similar is such a joy for us.”

Learn more about this condition and Riley’s treatment options here.