Congenital Diaphragmatic Hernia (CDH): Emerson's Story

Joshua and Stacie Oliveri knew that when their daughter Emerson was born they wouldn’t hear her cry. But nothing could have readied him for what they experienced in the delivery room.

“We weren’t prepared for the squeaking of suffocating,” he remembers. “You could hear her trying to get air in; it was unsettling.”

Emerson Rose Oliveri was born on Dec. 3, 2007, with a left-sided congenital diaphragmatic hernia (CDH), a defect that occurs when the diaphragm doesn’t close, creating an opening that allows abdominal organs to migrate into the chest and restrict lung development — an opening that needs surgical repair. Infants with CDH are remarkably fragile and can suffer multiple complications.


"The baby with congenital diaphragmatic hernia needs expert multidisciplinary care from the first moment,” says surgeon Holly L. Hedrick, MD, of the Center for Fetal Diagnosis and Treatment (CFDT), whose clinical specialty is caring for CDH babies. “It’s one of the most challenging diagnoses we handle.”

Severe CDH, but some hope

The Oliveris looked for a center with the experience to repair Emerson’s CDH after receiving advice from their local hospital to terminate the pregnancy.

“They didn’t see the point. They held no hope at all,” says Joshua.

At the CFDT, the Oliveris found the hope they were looking for — even though their unborn daughter faced frightening odds.

The hole in Emerson’s diaphragm was massive. She had the largest documented CDH defect of any survivor treated at CHOP.

“Our records you don’t want to hold,” says Joshua. “It was rib to rib. She had a 25 percent chance of survival.”

Multidisciplinary CDH repair

The CDH was so bad that Emerson’s liver, stomach, spleen and intestines were crowding her chest. Her heart was turned on its axis and shifted completely out of place. Immediately after she was born, the CHOP team worked to stabilize her so that the CDH could be repaired.

“We need all hands on deck,” says Hedrick. “There are multiple specialties involved with CDH. It requires a lot of people to get the baby through.”  

Emerson required an oscillation ventilator to support her breathing, but doctors were not optimistic because her CDH was so severe. They called Joshua in to see her.

“She looked horrible. Her entire upper torso was white,” he says. Oliveri and his family readied themselves to say goodbye to their newborn daughter. While they waited for the news of her death, Emerson was busy struggling to live.

'The first of 7,000 miracles'

Four hours after she was born, Emerson was off the oscillating ventilator and had become a viable candidate for ECMO (extracorporeal membrane oxygenation), a cardiopulmonary bypass technique that provides long-term breathing and heart support that would tide her over until the CDH repair.

“She pulled herself off death’s doorstep,” says Joshua. “That was the first of 7,000 miracles.”

Emerson’s first CDH repair surgery was performed while she was on ECMO. It was a challenging operation, and surgeons worked tirelessly to get the synthetic patch to hold.

Following that first CDH repair, Emerson lived for seven months in the Newborn/Infant Intensive Care Unit at CHOP. The Oliveris’ older daughter stayed with grandparents while Joshua completed his police academy training. Stacie moved to the Ronald McDonald House to be close to Emerson after the repair.

“Stacie was there all day every day and I think that played a huge role in Emerson surviving,” says Joshua. 

Ongoing challenges

But even after Emerson was released, the baby with the record-breaking CDH was not out of danger. She required a G-tube (gastrostomy tube) and around-the-clock nursing at home to manage her pulmonary hypertension and the long list of meds and oxygen she required for that problem.

Then at one year out, Emerson’s CDH reherniated and again required repair. The size of Emerson’s CDH was making it difficult for a synthetic patch to hold, especially as she grew.

A different approach achieves success

In just another year, it was becoming obvious that Emerson would need yet another repair. Fetal surgeon Alan Flake, MD, decided to take a different tack in repairing the toddler’s CDH. In a novel approach that would assure a patch large enough to cover the hole caused by the CDH, he performed a latissimus dorsi muscle flap repair, cutting part of Emerson’s own lat muscle away and using it as her diaphragm.

"It’s a rare procedure, we don’t do it very often,"  says Dr. Flake. And it was another record for Emerson — she was one of the first children to ever receive this type of repair for CDH.

That CDH repair held, and today, Emerson is a feisty and outgoing 3-year-old.

"The amazing thing about Emerson is that she survived despite having one of the worst CDH defects I have ever encountered," says Dr. Flake.

She has hit all of her developmental milestones and is enjoying preschool. “Other than the G-tube and a handsome set of scars, you wouldn’t know anything ever happened to her,” says Joshua. “To a lot of the doctors and nurses at CHOP, she’s famous."

Originally posted: March 2011