Our patients’ stories: Treating Owen’s encephalocele

When Jennifer and Kevin Sheridan went for a routine 14-week ultrasound of their unborn son Owen, the only surprise the young couple expected was hearing if they were having a boy or a girl. But seconds after the first images of Owen registered on the grainy, black and white ultrasound screen, the Sheridans’ lives changed.

The pictures showed that the top of Owen’s head hadn’t normally closed during early development, creating a sizable opening. Without the skull to encase them, spinal fluid and brain matter seeped through the hole, ballooning and expanding under his skin. The resulting growth, called an encephalocele, threatened to kill Owen during, or shortly after birth.

“We went from thinking about what color to paint the nursery to praying we’d get a least a few minutes with him before he passed,” Kevin remembers. “It was devastating.”

In spite of the prognosis, the Sheridans went through the pregnancy normally. Over the next few months, Owen grew, as did his encephalocele. By 26 weeks, the mass was nearly as large as Owen himself. But despite the size of the growth, tests showed Owen was developing typically. In fact, he was far more active inside the womb than Owen’s older sister, Aubrey, had been a year earlier. And while Jennifer and Kevin didn’t know it then, those prenatal kicks and turns would be the first indications of Owen’s fighting spirit—a spirit that would eventually come to define him.

Defying all expectations

When Owen was born via C-section a few months later, no one in the delivery room knew what to expect. But one thing’s for sure, nobody was ready for the strong, steady cry Owen let out after taking his first few breaths.

“Within seconds of being born Owen was belting out these loud screams,” Kevin says. “He was so responsive, breathing and moving his arms, legs and eyes. He just didn’t seem to be a baby on the verge of dying. He was living.”

A knit cap keeps Owen's encephalocele warm

Doctors carefully monitored Owen and the 11-inch, 7 lbs., mass growing from his skull, but as the hours ticked on it became clear that the original care plan of supporting him for a matter of minutes—maybe an hour—needed to be expanded. Three days later, Kevin and Jennifer took Owen to see specialists in a nearby city hospital who had more experience with encephaloceles. Unfortunately, doctors there had never seen a case so severe and weren’t overly optimistic.

“They said the only long-term solution would be to remove it, but that would far too risky, because of its size and the amount of brain matter and blood vessels involved,” Kevin says. “We were told there were palliative and hospice care arrangements that could be made, but that was about it.”

But as the days passed, Owen was growing and developing like a typical baby; he was responsive to his parents’ presence and tests showed he had a strong heart and healthy lungs. While it was difficult to tell if and how the encephalocele had affected his neurological development, he was developing well enough to give the Sheridans hope.

Around this time, Jennifer and Kevin began revisiting a story they had read early into the pregnancy. It was about Dominic, a Wisconsin boy born with an extremely rare encephalocele, whose condition was treated through complex neurosurgery and facial reconstruction by the team at Boston Children’s Hospital’s Craniofacial Anomalies Program. “We must have read about Dominic a hundred times. It gave us something to work towards,” Jennifer says.We thought if anyone has the experience needed to help Owen, it’s Boston Children’s.”

With that in mind, the family contacted doctors John Meara, MD, DMD, MBA, and Mark Proctor, MD, who together had been key players in Dominic’s case. Even with Proctor and Meara’s long history in treating encephalpceles, Owen’s was the largest either had ever seen. After carefully going over Owen’s information, both agreed that while difficult, it would be reasonable to attempt to surgically remove Owen’s encephalocele and reconstruct his skull with surgery.

Two weeks later, the Sheridans were in a Boston Children’s hospital room preparing themselves for the following morning’s surgery, which would prove to be one of the biggest days of their lives.

Hope in action

Meara checks on Owen after surgery

At 7:30 a.m., Craig McClain, MD, and a team from Boston Children’s Anesthesia Department, began the process of giving Owen anesthesia. Due to the unique nature of his condition and limited ways his head could be positioned—not to mention a developing respiratory infection—properly sedating Owen required a coordinated effort that had been carefully planned in advance.

“I had never worked on a case like this before, but because we had a well laid out, well communicated plan going in, we were confident about the procedure,” McClain says. “Everyone on the team had treated complex cases before, knew exactly what the other people in the room were doing and knew how to work together. Now we just needed to do it for Owen.”

Once Owen was properly sedated, the team began draining much of the excess fluid above his brain, so they could then remove the mass. Next Meara made the initial incisions that allowed Proctor the room he needed to resect the excess brain matter. Because the team had spent so much time reviewing Owen’s case in advance, he was able to carefully note the location of every blood vessel on the head, which helped avoid any complications that could quickly lead to Owen bleeding to death.

Once the encephalocele was removed, Proctor and Meara took a portion of Owen’s skull from near his left temple and used it to repair the area where the encephalocele originally occurred.

“There was very little chance that bone was ever going to grow naturally on the top of his head, so we needed to come up with a long-term solution,” Meara says. “Given how young Owen is we knew that the area of his skull we took from would regenerate quickly. Repurposing it to close off the gap offered an ideal, natural solution to his problem and the end result was far better than we could’ve imagined.”

The long road ahead

In the coming months, Owen might have extra fluid build up in his skull again, requiring an additional surgery to place a small drainage tube. Also, he will be fitted with a specially designed helmet that will guide the shape and size of his head to prevent it from growing abnormally. And because doctors still aren’t sure how his condition affected his brain development, he will need to be closely monitored in the coming years.

Proctor, Owen and Jennifer after Owen's surgery

In spite of the long road ahead, the Sheridans are thankful for the steps that have taken them this far, especially those that led them to Boston. “The courage and expertise of the medical teams at Boston Children’s Hospital gave our Owen a second chance at life,” Kevin says. “They were confident enough to attempt a surgery that few hospitals would dream of, and in doing so they’ve given us the hope we were longing for.”

According to everyone that treated Owen, the confidence Kevin speaks of comes from a full medical team working together like a well-oiled machine, fueled by collective knowledge and experience.

“There’s no denying that Owen’s was a difficult surgery, but together we’ve worked on complex cases before, which makes situations like this far more manageable,” Proctor says. “And that goes far beyond Drs. Meara, McClain and I. It’s really a testament to the experience of everyone involved, the entire nursing staff, the craniofacial team, the anesthesiologists, the ICU staff and all the consultants in the hospital. Without the whole team, we wouldn’t be able to take on a case like Owen’s.”

To learn more about the services it provides, or to speak with one of our experts, please visit the Craniofacial Anomalies Program website.

One thought on “Our patients’ stories: Treating Owen’s encephalocele

  1. Owen will be 6 months old tomorrow! Please continue to do the great work you do! I am a HMS grad, and I will never forget all that I learned at Children”s. I also remember the families who came to present their stories at HMS along with their “miracle” children. I truly hope that Owen can tell his story someday in the MEC and continue the tradition of teaching future doctors.

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