Making faces

Every year, approximately 80 children in Manitoba are born with facial issues that affect their appearance and threaten their health. Fortunately, a team of Winnipeg Health Region medical specialists, led by plastic surgeon Dr. Lonny Ross, are there to help.

Keira Martin
Keira Martin
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A delicate procedure

Bio: Dr. Lonny Ross

About the Manitoba Centre for Craniofacial Difference

Winnipeg Health Region
Wave, September / October 2011

No doubt about it, Keira Martin is one adorable little toddler.

With sparkling eyes, curly sandy-blond hair and a warm smile, the 18-month-old is energetic, often laughing, and almost always playing.

Along with her three-year-old brother Alistair, she keeps her parents, Jonathan and Amanda Martin, on their toes. With another child expected in the fall, the Fort Richmond couple expects to be busy for the next few years, enduring the typical stresses of raising three young children.

But one year ago, the family experienced a different kind of stress altogether, and Keira looked strikingly different. Instead of a big wide smile, the most visible thing about her face was titanium pins sticking out of both her cheeks - remnants of a surgical procedure that essentially involved breaking her jaw on both sides and stretching the jawbone away from her skull using a titanium device called a mandibular distractor.

The reason: Keira was born with two facial issues - cleft palate and Pierre Robbin Sequence. Cleft palate affects the appearance and use of the upper lip and roof of the mouth, leading to problems eating and speaking. Pierre Robbin Sequence is a condition in which the lower jaw does not grow to the same size as the rest of the skull. Together, these two conditions not only significantly affected Keira's appearance, they also undermined her ability to eat and breathe.

A few years ago, a newborn like Keira would most likely have had to leave the province to receive the treatment for her conditions - if she was able to get treatment at all. But that changed in 2003 with the creation of the Winnipeg Health Region's Manitoba Centre for Craniofacial Difference (MCCD). Located at the Children's Hospital at the Health Sciences Centre, and led by Dr. Lonny Ross, the centre specializes in surgically correcting facial issues like Keira's.

Each year in Manitoba, an estimated 80 children are born with craniofacial differences, a term used to describe a number of facial issues, including cleft palate, Pierre Robin Sequence, and Treacher Collins Syndrome, among others.

In Keira's case, doctors picked up on the cleft palate soon after she was born. That was enough to merit a visit from Cindy Bonneteau, a nurse clinician from MCCD who meets with all families of newborns with cleft lips and palates or other facial differences.

"For this particular case, it was a bit strange," she says. "She (Keira) was born with a cleft, and that's the reason I went to visit her, to make sure that things would go OK with feeding."

But it didn't take long for Bonneteau to realize that Keira also had Pierre Robin Sequence. "When I got there, I noticed she was having issues that were beyond just a cleft," Bonneteau says. "I thought her jaw was tiny, and she was having trouble breathing, so she needed to see Dr. Ross sooner rather than later."

Ross is one of a handful of plastic surgeons in Canada who specialize in repairing craniofacial differences in children and adults. He leads a team of specialists at the centre that provide comprehensive, cradle-to-adulthood care for individuals in Manitoba with any number of physical differences from routine deformities, such as cleft lips and palates, to serious genetic syndromes that are life-threatening without repair.

"We have one of the most diverse craniofacial and pediatric centres in Canada," says Ross, who has headed up the centre for the last eight years after more than a decade of training both in Winnipeg, his hometown, and abroad where he studied under the world's leading experts in plastic surgery.

Ross is quick to point out that he is just one of many specialists at the centre. While he may be the director, it takes a team of experts working together to address the many needs of these patients.

"Because their five senses are often affected by their facial differences, these patients require the expertise from many different disciplines," Ross says. Patients may need to see a neurosurgeon, an oral surgeon, an ear, nose and throat specialist, a psychiatrist, a radiologist, a dentist and orthodontist - just to name a few. All told, the team at the centre consists of more than 15 health-care professionals, nurses and doctors - all of whom have significant experience in helping children and adults born with facial differences and other health issues.

Ross says Keira's cleft palate was probably exacerbated by Pierre Robin Sequence, which would have caused her tongue to sit further back in her mouth. "The thought process behind that is because the jaw is so small, the tongue is standing upright and prevents the fusion of the palate during development," Ross says. "Numbers as high as one in 10,000 to one in 30,000 births can have this collection of findings where the newborn has a cleft palate and small lower jaw."

Like most newborns with clefts, Keira had difficulty feeding, a concerning enough condition considering newborns desperately need to feed to fuel rapid development during the first few weeks of life.

In most instances, these feeding issues can be overcome with a little knowhow and a special bottle. But even with this help, the Martins still were having a difficult time getting Keira to take to the bottle and put on those necessary ounces of weight.

"At the time we were just so confused about why she couldn't eat," says Amanda. "You're just trying to feed your baby and you're thinking, 'Why can't she eat more, why isn't she gaining weight?'" More concerning to Bonneteau and Ross was that Keira was also having trouble breathing.

The Martin family, from left to right: Alistair, Jonathan, Keira and Amanda.

The Martin family, from left to right: Alistair, Jonathan, Keira and Amanda.

Like many children with clefts, Keira needed surgery to repair the problem, a procedure that is usually done at about 11 months of age. For many children with Pierre Robin Sequence, however, the cleft repair procedure is delayed to see if the jaw will grow and catch up with the rest of the skull structure. But Keira's breathing problems forced the issue.

After a sleep study, often done for children with more severe clefts or conditions like Pierre Robin Sequence, they discovered Keira suffered from sleep apnea, which meant she basically stopped breathing many times while sleeping because her tongue was obstructing her airway.

By May 2010, when Keira was four months of age, Ross recommended she undergo a leading-edge surgical procedure to repair her jaw called mandibular distraction osteogenesis. It involved Ross breaking Keira's jaw on both sides and stretching the jawbone away from her skull using a titanium device called a distractor to simulate normal growth and pull her tongue out of her throat.

Ross is the only plastic surgeon in Manitoba who performs the procedure. It was originally pioneered by famed French craniofacial plastic surgeon Dr. Paul Tessier, someone who has been a large influence in Ross's career. Ross studied under one of Tessier's students, Dr. Stephen Schendel, and he still consults frequently with his mentor as well as with other experts throughout North America.

Plastic surgery is a constantly evolving field of medicine, so keeping abreast of the latest developments is crucial in providing the best care possible, says Ross. To that end, he is working to get his centre accredited with the American Cleft Palate - Craniofacial Association, a move that will ensure the standards at the MCCD remain high.

"If you're co-operating with larger centres and working with smart people, you can do things that will push you forward and create better care for the patients," says Ross.

The jaw distraction osteogenesis procedure Ross recommended for Keria has been performed in Canada for less than a decade. The concept behind the procedure involves using the body's own processes to heal itself. The surgeon breaks the jaw, stretches it using a distractor, and the bones eventually grow in to fill the gap, creating a bigger jawbone.

In Keira's case, Ross broke her jaw on each side and inserted the distractor footings on both sides of the break. He then fastened the distractor to the bone with screws. In the earlier days of the procedure, the distractor rested outside the skin on long pins, but recent advances have reduced its size and now it can be inserted under the skin with only the activation arms protruding from the skin - as was the case with Keira.

Other technological advances have helped speed up the procedure. The surgery used to take an entire day because the surgeon would have to take several measurements in the operating room to adjust the distractor to fit the jaw precisely. Today, using computer technology, the surgeon can create an exact three-dimensional replica of the patient's skull before surgery to adjust the distractor to a perfect fit to the actual jaw for use during the procedure. This advance helps decrease time in the operating room from more than seven hours to often less than three hours.

Two activation arms, one on each side of the face, protrude from the skin. They are turned twice a day for ten to 14 days following surgery to slowly pull the broken bones apart - like a vice in reverse.

This process can help lengthen the jaw by two to three centimetres, which may not sound like much, but it is. "If you stretch out the jaw, on average, about two millimetres a day for about two weeks, that's almost three centimetres," says Ross. "On a little baby, that's huge. Keira's jaw measured only four centimetres prior to the surgery."

But the genius behind the procedure lies less with technology and more with Mother Nature.

Over the weeks to follow, the gap will fill with bone to complete the metamorphosis. Once bone healing is complete, the distractor is removed. Keira underwent surgery to remove the distractor in September 2010 - four months after her first surgery to lengthen her jaw.

The result has been nothing short of remarkable, says Jonathan Martin.

"She was a very small baby before the jaw surgery, and she was very quiet," he says. "She slept all the time because she wasn't getting the proper sleep - it was a life-changing surgery for her."

A few months after the first operation, Keira underwent surgery to repair her palate, at 11 months of age, like most other children born with a cleft. Without the distraction procedure, the palate procedure would have worsened her breathing and would have been delayed, thereby delaying the start of normal speech development.

Her journey, however, doesn't end with her last operation. She will be followed by Ross and other members of the MCCD team throughout her development until adulthood.

"If you are born with a cleft lip, we will follow you your whole life, and when you turn five, I would set up that appointment for you to come to the Craniofacial Team Clinic for a day," says Bonneteau. "It's kind of like having your five-year-old check-up, but with a team."

Patients also attend the Craniofacial Team Clinic at age 12 and then 15, usually around the time children have major growth periods and may require changes to their care or even another surgery. Bonneteau's primary role is to co-ordinate the care for all cleft and craniofacial patients to ensure they see the right specialist at the right time.

It's common for children with congenital differences that arise from non-genetic causes, such as Keira, not to require future surgeries. Patients with syndromic - or genetically related - abnormalities, however, often need several surgeries as they grow into adulthood. "It's a four-dimensional problem, the fourth dimension being growth," Ross says.

Samantha Prior, 23, is one such individual. She was born with a rare disorder called Treacher Collins Syndrome. This hereditary disorder is found in about one in 10,000 births, and causes parts of the face to not form properly, including the ears, nose, eyes and jawbones.

For most of Prior's childhood and adolescence, she had to travel from Winnipeg to Toronto for care and surgeries, and, at the time, treatment for problems like Pierre Robin Sequence - a condition that resulted from her Treacher Collins - was quite different than today.

Prior had to undergo a painful bone graft at age five, in which pieces of her ribs were removed and grafted to her jaw to increase its size.

"Her jaw was very tiny, similar to Keira's when she was born, and the option that they chose was rib bone grafts to lengthen the bone, because that's back at a time when they weren't doing distraction osteogenesis," Ross says. "The problem with that procedure is it's very unpredictable how the rib was going to grow."

By her mid-teens, Prior's jaw outgrew her face and, again, she had to travel to Toronto for another surgery that took pieces of her hip-bone and grafted them to her jaw to replace the overgrown rib-bone in her jaw. At the same time, they inserted cartilage to act as a hinge for her jaw.

But this, too, led to unsatisfactory results. Prior was unable to open or close her mouth because the jaw bone fused to her skull. "From that whole thing, what hurt most is the bone graft from my hips," says Prior, an avid reader of books on psychology, including the writings of Carl Jung, a famous 20th century psychiatrist.

Samantha Prior plans to become a volunteer mentor at Dr. Ross's clinic.

Samantha Prior plans to become a volunteer mentor at Dr. Ross's clinic.

Then a couple of years ago, she was referred to the care of the craniofacial team at HSC to meet with a dentist. "Because she couldn't open and close her mouth, her oral hygiene was bad," Ross says. "She couldn't brush her teeth because she couldn't open her mouth wide enough to get a tooth brush in there, for example, so we had to pull all her teeth."

Soon, she was in discussions with Ross for surgery to restore movement in her jaw, and in June 2010, a few weeks after Keira had her jaw distraction, Prior underwent hers.

Only Prior's was somewhat different.

"In Samantha's instance, I wasn't creating new bone," Ross says. "Instead of allowing two raw surfaces of bone to be separated slowly by the distractor so they could grow together, I stretched the jaw very quickly, too fast for new bone to form."

The soft tissue - scar tissue - then grows between the break to form a cushion-like joint in her jaw, restoring some movement. The procedure, however, was just the beginning for Prior.

"It was one of the hardest procedures I've had, but it was weird because it was more involved in terms of what I had to put in post-operatively," Prior says.

Because she hadn't used her jaw in years, its muscles had atrophied. Now she exercises the muscles several times a day to build them back up and to ensure the scar tissue - which naturally wants to contract - remains flexible.

"Samantha's worked very, very hard on the exercises, and without that, you don't have anything," Ross says. "I can do the surgery and if she doesn't do the exercises, her jaw will re-fuse."

Because of Prior's dedication to her rehabilitation, Ross says they can move onto stage two of the surgical process: aligning her jaw correctly for implants and dentures.

Prior says the procedures are painful - though not as much as bone grafts - but they are worth it if they improve her quality of life.

She also could have surgery to cosmetically alter her appearance to make her appear less different from most people, but she says that doesn't interest her.

"Honestly, I'm content. I feel good about myself as I am. I think it's been proven in this day and age that you don't need to look ugly to feel ugly."

Ross says Prior has the right attitude.

"Samantha has very reasonable expectations and knows herself well enough that I feel comfortable treating the needs that she has as she asks for them."

She, in turn, credits Ross and the team with providing her with excellent care, in large part thanks to the strong relationships they develop with patients and their families.

"Dr. Ross is awesome; he and his team are great. Across the board, I can't say enough," she says, adding she plans to volunteer as a mentor at the clinic for younger patients. "It's odd to say that I enjoyed having surgery, but that is the case - at least, as enjoyable as it can be, right?"

The Martins too are overjoyed with Keira's medical treatment at the centre. Aside from some small red scars under her jaw, she looks as if she's never gone under the knife.

"The scarring is fading more and more all the time," says Amanda Martin. "The procedures she received basically will let her live a very normal life."

Joel Schlesinger is a Winnipeg writer.

Wave: September / October 2011

About Wave

Wave is published six times a year by the Winnipeg Health Region in cooperation with the Winnipeg Free Press. It is available at newsstands, hospitals and clinics throughout Winnipeg, as well as McNally Robinson Books.

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