The first surprise for Danielle and James came at the first prenatal appointment at their OB’s office. Instead of the expected one heartbeat, there were two.
The second surprise came seven months later when their identical twin daughters were born on Dec. 29, 2019. A moment after the bigger one, Stella, was delivered, they heard the nurse say, “We have a slight problem.”
“There was a lot of confusion,” James recalls. Stella was born with a mass at the back of her tongue, the oropharynx, that stretched down into her throat. The tumor interfered with her breathing and feeding and had caused a cleft palate, when there’s an opening in roof of the mouth. Neither the mass nor the cleft palate had shown up in any of the many ultrasounds during the pregnancy.
From ‘confusion’ to ‘amazing’
Their local hospital transferred Stella to a regional center, while Danielle stayed at the first hospital with the other twin, Aria. After 12 days of hearing conflicting advice at the New Jersey hospital, Stella was on her way to Children’s Hospital of Philadelphia.
“While I was still in the hospital, I joined a cleft palate Facebook group,” Danielle says. “They told me, ‘CHOP is amazing.’ I had a conversation with Nancy [Folsom, RN, nurse navigator for CHOP’s Cleft Lip and Palate Program], and six hours later the CHOP transport team arrived to bring Stella to CHOP.”
Once at Children’s Hospital, the family met pediatric otolaryngologist Conor Devine, MD, of CHOP’s Head and Neck Disorders Program, which specializes in treating a variety of conditions. Stella had an MRI to identify the location of the tumor and an endoscopy to check her airway. Stella was also examined by plastic surgeon Jesse Taylor, MD, of Cleft Lip and Palate Program.
Coordinated treatment plan
“We learned more in a few hours at CHOP that we had in more than two weeks at the New Jersey hospital,” Danielle says.
“It wasn’t long before we had a game plan,” James says. “Dr. Devine and Dr. Taylor were on the same page. The first order of business was to get the mass removed.”
It turns out that the tumor fit perfectly into the cleft in her palate. When her tongue flopped forward, the two connected, creating a seal that blocked her airway. For the few days before the surgery, Stella was intubated to keep her airway open and oxygen levels up. She also had a nasogastric (NG) feeding tube to maintain her nutrition.
While the two problems were connected, they would be handled by two different teams, the Head and Neck Disorders Program and the Cleft Lip and Palate Program.
“We were so impressed how the two groups worked side by side,” Danielle says. “They knew their part of the care plan, the timing, everything.”
All clear from Genetics
While Stella awaited surgery, she was examined by geneticist Elaine Zackai, MD.
“She explained the whole story of how the cleft came to be,” Danielle says. “The mass came first and prevented the palate from forming correctly. After examining her, measuring everything, Dr. Zackai told us that there are no signs of a genetic syndrome. There’s nothing genetically wrong with her!”
Removing the teratoma, sparing the nerves
On day 19 of life, Stella underwent surgery to remove the mass. Because of the size and placement of the tumor, Dr. Devine needed to remove the half in the oropharynx through her mouth and the half in her throat by coming in through the neck. Each section was nearly an inch across.
“We were a little nervous,” James says, “but we had the utmost confidence in Dr. Devine, that he could remove it without damaging her tongue.”
Dr. Devine carefully excised the tumor without damaging the tiny, delicate nerves that control tongue movement so swallowing and speech won’t be affected.
“He did such a beautiful job with the small incision in her neck crease under her jaw bone that you can’t hardly even see it,” Danielle says.
The tumor was analyzed by Pathology and was deemed to be a teratoma, a benign tumor made up of mixed tissues. Oral teratomas, also called oropharyngeal teratomas, are extremely rare, occurring in approximately one in 35,000 to 200,000 newborn babies.
Cleft palate repair improves speech
After a total of three weeks at CHOP, Stella was able to join her twin sister at home. Stella didn’t need supplemental oxygen, and she was taking all her nutrition by bottle, using a brand of bottle that works for children with cleft palates. Dr. Taylor, pleased by how well Stella was doing, advised waiting until she grew some before repairing her cleft palate.
At 10½ months old, Stella returned to CHOP for the cleft palate repair surgery, which only required an overnight stay. While Stella was under anesthesia, otolaryngologist John Germiller, MD, PhD, also placed ear tubes since she had a lot of fluid and wax in her ears that had been muffling sound.
Once home, Stella’s parents noticed a change right away. “She started making sounds she could never make before,” James says. “Before she would sort of squeal and growl. Because of the cleft, she had lost a lot of function in her sound-making ability. A day after surgery, we start hearing ‘ma ma ma, da da da.’ It was amazing.”
Danielle says, “She caught up to her sister in three days. She will be able to live her life like a normal baby.”
“From start to finish, once we got to CHOP it was a really wonderful experience,” Danielle says. “We are thrilled with the care Stella and our whole family received.”