Our patients’ stories: Dominic’s craniofacial surgery

by Tripp Underwood on January 28, 2013

Dominic-1Dominic Gundrum’s smile is truly special. Or, more accurately, there’s something really special about his smiles. They light up a room, even though they’re the result of a rare and extremely difficult to correct birth defect. Still, despite how atypical they seem at first, Dominic’s giggling smiles are surprisingly disarming.

 

To have something look so different—but still spread such joy—is truly unique. And, in a way, that uniqueness defines Dominic perfectly.

An uncertain beginning

During a routine 20-week ultrasound in their home state of Wisconsin, Dominic’s parents, Mark and Mary, were excited to find out if they were having a boy or girl. But when doctors looked at Mary’s grainy ultrasound they discovered more than Dominic’s sex. Though it was hard to tell for sure, the ultrasound image showed that Dominic’s skull hadn’t fused together properly early in the pregnancy, leaving a large, triangle-shaped gap running from his upper lip through the middle of his nose and forehead—a condition known in the medical world as a Tessier midline facial cleft.

But the cleft wasn’t the only issue Dominic was facing: some fluid and tissue from his brain, normally encased in the skull, had seeped outwards through the cleft, forming a golf ball-sized bubble underneath the skin of his forehead. (A condition called an encephalocele.)  

The combination of Tessier cleft and an encephalocele is very rare, especially in the United States. As few as one child every two to five years may be born with both conditions, and most craniofacial surgeons will go their entire careers without ever seeing it outside of medical textbooks. As a result, the Gundrums’ medical team wasn’t sure what the future held for Dominic.

“At first we were told he would probably die within a few hours and that we should do our best to enjoy the pregnancy,” remembers Mark. “Over time his prognosis got better, and the doctors agreed he’d survive birth. But after that, they had no idea what may happen to him. It was a real no man’s land territory for all of us.”

MearaGiven the unknowns of the situation, the Gundrums took to the Internet for answers. Their search led them to a story about a boy named Dumanel—born in Haiti with a severe encephalocele—who was successfully treated by John Meara, MD, DMD, MBA, plastic surgeon-in-chief, and Ed Smith, MD, a neurosurgeon at Boston Children’s Hospital. The story gave Mark and Mary hope that like Dumanel, Dominic could one day live a happy and healthy life. They emailed Meara a picture of Dominic’s ultrasound asking for advice, and a day later received a phone call.

“I’ll never forget when Dr. Meara called and said he had seen this before and that everything was going to be OK,” says Mary. “We’d heard so many things over the course of the pregnancy, but ‘OK’ was a word that was rarely used. It was such an overwhelming feeling of joy, I called Mark immediately and said, ‘I think we’ve got to go to Boston.’”

A few months later Dominic was born without complication and in good health, aside from the Tessier midline cleft and encephalocele. The Gundrums were beyond relieved when it became apparent that Dominic was going to live to see surgery, but it also meant the real work was about to start in Boston, where Meara and his colleagues were already getting ready for Dominic’s arrival.

All hands on deck

Treating Dominic’s encephalocele would require a number of specialists with a wide range of expertise. First, Mark Proctor, MD, director of Boston Children’s Brain Injury Center, would need to carefully remove the brain tissue and fluid that had pooled inside Dominic’s encephalocele. Once Proctor had finished, Meara would perform extensive reconstruction of Dominic’s face, repairing the cleft and aligning the previously disconnected sections of skull while making sure his features were symmetrical. Altogether the surgery would involve over 15 people and last for several hours.

“To correct a condition as complex as Dominic’s takes a lot of work, from many very talented people,” says Meara. “An operation like this can be dangerous and lengthy; if you don’t have the right team it could take up to 12 to 18 hours. But I knew with the right preparation we could work more efficiently, posing less risk to the patient.”

Meara, Proctor and Susan Goobie, MD, from Boston Children’s Anesthesiology department, spent weeks discussing Dominic’s case and exploring every possible complication that could arise. After much planning and revision, the team was confident they were ready.

A well-oiled machine

At 7:30 a.m. on the day of the surgery, Mary and Mark handed Dominic over to Goobie, and prepared themselves for several long hours of waiting. They were nervous, excited and even a little sad.

“We’ve fallen so in love with Dominic over the past few months that the idea of changing him in such a dramatic way is a little bit difficult,” Mary said on the eve of the operation. “In the long run it’s what’s best for him, but so much will change in one day. It’s exciting, but there is a small bit of sorrow knowing that tomorrow this baby I’ve grown to love so much will look so different.”

Once Dominic had been placed under anesthesia, Proctor and Meara began their work. In a few hours they had successfully removed parts of Dominic’s skull and the encephalocele, and then repositioned a good portion of his frontal lobe, which had been displaced. Then over the next several hours they carefully reconstructed the bones in Dominic’s jaw and eye sockets, as well as replacing the fragments of skull that had been removed to allow Proctor enough room to resect the encephalocele.

It was very precise work, but thanks to months of planning the surgery went more smoothly than anyone had anticipated. By 4 p.m., Dominic was recovering in Boston Children’s Intensive Care Unit (ICU).

“I was pleasantly surprised with how well things went and how efficiently our team worked,” says Meara. “Everything went according to plan thanks to the amazing efforts of the whole team.  From Dr. Proctor to Dr. Goobie, the nursing staff, our residents and fellows, everyone’s role was perfectly executed. It was like working with the dream team of craniofacial surgery―this is why I work at Boston Children’s; there is no other team like this.”

Facing the future

Dominic spent the next three weeks in Boston Children’s ICU, where he was expertly cared for by an entire team of attendants. As he continues to heal, Meara and other specialists—both at Boston Children’s and in the Gundrum’s home state—will be monitoring him to make sure his development continues on a normal trajectory. As he grows he may need to see an ophthalmologist to help his vision, as well as a craniofacial specialist, in case any of his features affected by the surgery change as he grows.

Even though Dominic’s medical journey is far from over, Mark and Mary are very grateful their son will have the chance to travel the long road to recovery.

“The day Dominic was born, a group of friends had a tree planted in our yard in his honor,” says Mark. “I remember thinking, 10 years from now, I’m either going to look at that tree and think of the son I lost, or watch him climb its branches. Right now, thanks to so many wonderful people at Boston Children’s and beyond, I’m excited to watch him hanging from that tree as they both grow bigger and stronger.”

Please click here to make an appointment with the specialists in Boston Children’s Craniofacial Anomalies Program. Visit their website for more information.

To read more about Dominic and his treatment, please see this article in the Boston Globe.

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