Shortly after Mary Gundrum found out that her unborn son was developing with part of his brain outside his skull, she found herself searching the internet to find out more about the baby's diagnosis and potential treatment.
Her next search would be for infant-sized coffins.
"He was only supposed to live a couple of hours," she said.
Instead, baby Dominic was born "screaming and kicking and full of life," but he had a Tessier midline facial cleft, which means the sides of his face didn't fuse together properly during early pregnancy, causing it to split down the middle. Dominic also had an encephalocele, a rare cranial abnormality that means a sac of brain tissue and fluid formed outside the skull. Dominic's encephalocele filled the space between the cleft in his face, splitting his nose in two with a fluid-filled bubble and causing some of his brain matter to sag into his mouth.
Click here to see Dominic's X-ray and other unusual conditions captured in medical imaging.
Dominic was not in immediate danger, but potential life-threatening complications down the road for a child with his conditions included a puncturing of the sac or infection.
Today, Dominic is seven months old, and developing normally for his age. Thanks to a six-hour craniofacial surgery, the only sign of Dominic's birth defect is the thin scar on his forehead that fades with each passing day.
"It's superexciting to think about his future," said Gundrum, who homeschools her seven other children in Milwaukee. "I never would have imagined such a small, tiny line that's already continuing to fade. It's beautiful."
Dr. John Meara, the chief plastic surgeon at Boston Children's Hospital, led the surgery that saved Dominic's face this December, prompting the family of 10 to move from Milwaukee to Boston for five weeks in December.
Meara specializes in craniofacial surgeries like the one Dominic needed, having done three encephalocele surgeries in six years during his time in Boston. Before that, he worked at the Royal Children's Hospital in Melbourne, Australia, where he researched craniosynostosis, or birth defects associated with skull development. Because of Melbourne's proximity to Southeast Asia, where many children are born with clefts and other birth defects, Meara got a lot of practice treating them.
"In the 20 years I've been in the specialty, I've not seen a case exactly like this," Meara said.
He said that the occurrence of a Tessier midline cleft and an encephalocele together in one person is a rarity.
"I'd be lying if I didn't tell you that I had a number of sleepless nights," he said. "But I felt comfortable in the sense that we had an excellent team."
Surgeons would have to move the brain into its proper position, close the two halves of Dominic's face, and create a floor for Dominic's brain (and a roof for his mouth). Blood loss during the surgery was a concern because Dominic only had about 650 ccs of blood in his entire body – about as much as "two cans of Coke," Meara said.
But Meara and his team, including neurosurgeon Dr. Mark Proctor, rehearsed the procedure's 37 steps on an acrylic model of Dominic's head. They went into the operating room at 7:30 a.m. on Dec. 18, began the surgery at 9:30 a.m. and finished up at 3:30 p.m. It took the Boston Children's Hospital team only six hours, compared with the 18-to-20 hours it took doctors when they first started doing these procedures in the 1970s, Meara said.
In that sense, Dominic was lucky, said Dr. William Magee, the plastic surgeon who co-founded Operation Smile with his wife in 1982. Operation Smile has provided more than 200,000 free surgeries for cleft palates, lips and other facial deformities in more than 60 countries to date.
"What you see is a lot of this in developing countries," Magee said of facial deformities, adding that the children who survive childhood are often branded as outcasts. "They certainly don't go to school. They certainly don't get employed. It's horrible, and it's a very correctible problem in the right hands."
He added that about 80 percent of the world's population doesn't have access to surgeons like Meara. A child is born with a cleft every three minutes worldwide, according to Operation Smile, and the Centers for Disease Control and Prevention estimates that one in 10,000 babies in the United States are born with an encephalocele.
Still, Dominic risked infection and other complications, Meara said.
Despite a bout of possible fungal meningitis, which Meara said he thinks was perhaps not a true case of the illness, Dominic is doing well.
Dominic still has some issues with his pituitary gland -- which was moved to its proper place during surgery – causing problems with sodium levels, hemoglobin and water balance, Meara said. In the three weeks Dominic has been home in Milwaukee, Meara has checked on him with a camera-equipped robot in the Gundrums' home. Dominic has not had to return to the hospital.
"I have to say, handing my son off to them [the surgeons] was the most difficult thing I think I've ever done in my entire life," Gundrum said, adding that she knew her son would never look the same again. "As much as his face was different, we fell in love with that face. I don't know that anyone can really understand that."
Gundrum remembers one of her 11-year-old sons pulling a blanket over Dominic's face in a store shortly after Dominic was born so that shoppers wouldn't say anything mean about his deformities. But Gundrum took the blanket off and taught her son to show others he was proud of Dominic the way he was. Her 13-year-old daughter would later comment that she thought normal babies' noses were "weird" because she was so used to seeing Dominic's split button nose.
"You mourn the loss of the look and face that you fell in love with because that's the baby you knew," she said. "Now, there's a new Dominic, and we're falling in love with him the way he is."Also Read