Photo courtesy of Allard USA.

When a child is required to wear ankle foot orthoses (AFOs), his or her clothes have to be more than just cute. Trying to match the logistical requirements of AFOs with fashion concerns and psychosocial development can be a challenge for patients and parents alike.

By Shalmali Pal

In the anthology of cute kid stories, there’s at least one chapter about children and their clothes. There’s the one about the toddler who insists on choosing her outfit, proudly exiting the house in a riot of colors and patterns. Or the kindergartener who refuses to attend school unless he wears his superhero costume. Or the tween who must have the same pair of jeans as her friends, no substitutions allowed.

But when the child is required to wear ankle foot orthoses (AFOs), cute can quickly morph into challenging for patients and parents alike as they try to match the logistical requirements of the AFOs with fashion concerns and psychosocial development. LER checked in with some experts for advice on how to make that match happen and what clinicians can do to help.

Infants, crawlers, and toddlers

For small children (aged 5 years and younger), clothes need to be easy-on, easy-off, whether it’s to facilitate a diaper change or make it more efficient to help the child don and doff the AFOs.

Kathy Martin, PT, DHS, said she’s yet to encounter problems with AFOs related to the type of clothing that is typical for infants, such as baby sacks and onesies.

“It’s not been my experience that parents have to choose different types of clothes based on the braces except that, as with all babies, you want to be able to get them off and on easily,” said Martin, professor and Doctor of Physical Therapy program director at the Krannert School of Physical Therapy at University of Indianapolis. “At that age, kids don’t usually have the manual dexterity or strength to get their braces off, so I don’t think you need special clothing to try to stop them from doing that.”

Photo courtesy of Cascade Dafo.

Lara Hartung, CO, of O&P Labs in St. Louis, MO, agreed.

“I tell the parents of these kiddos that anything that snaps open and closed along the bottom is usually the way to go,” Hartung said, adding that this holds true even if the child requires a brace with an abduction bar to treat clubfoot.

Yaron “Ron” Raducanu, DPM, president of the American College of Foot and Ankle Pediatrics, added the reminder that climate will impact clothing choices. An infant living in a warmer region may be able to get away with short onesies that leave the AFOs uncovered. Climates that call for more coverage may require some experimentation to determine what accommodates the AFOs best.

Regardless of the clothing, “one key with AFOs, especially if they are custom made, is that they should not cause any irritation to the wearer,” said Raducanu, who is in private practice in Philadelphia. “It’s up to the practitioner to make the brace as comfortable as possible. I think it’s more important to get that right than to be too concerned with anything that goes over the brace.”

But what happens when children become mobile? How should parents dress a crawler or toddler who requires AFOs?

In young children, experts say, often the AFO itself is more restrictive than the child’s clothing. But Raducanu reiterated that streamlining the AFOs as much as possible curtails problems with children’s clothing.

“The Velcro can sometimes be troublesome and catch on the AFOs, so that’s something that I check for and try to minimize,” he said. “Also, you have to look at the placement of the buckles and latches, so that they don’t interfere with clothing and cause discomfort.”

As for clothing materials, there are no hard and fast rules. The experts agreed that socks made with moisture-wicking materials are best for regulating sweat. Otherwise, it’s mostly a matter of trial and error, and communicating with the child to see if the items are suitable for the AFOs, comfortable, and appealing.

Of course, if the child is preverbal, traditional communication may be difficult, but family therapist Jillian Pizzi, MA, has ways to manage that. First, she suggested, healthcare practitioners can use dolls or stuffed animals to show the child where the AFOs will be placed and how their clothing will accommodate the braces. Then, once the child has donned the AFOs, the outfit, or both, use the same dolls to ask the child to point out areas where the braces or the clothes are uncomfortable.

Photo courtesy of Cascade Dafo.

Pretending to put the AFOs on the doll first allows the child to “buy in” to the braces, explained Pizzi, who is with South Bay Mental Health in Lowell, MA.

“They will feel more comfortable seeing it done [on the doll] first, and this may enable them to relax a bit,” she said.

Pizzi has a unique perspective on pediatric AFOs–she was diagnosed with distal arthrogryposis and underwent multiple surgeries, starting when she was 10 months old and ending at age 4. She wore AFOs off and on until she was 7 years old. She now works with disabled children at her counseling practice.

Even when children are very young, Pizzi advised building trust by taking the time to explain the devices, and then giving them some say in the clothes they need to adapt to those devices.

“Taking the time to explain, show, demonstrate, and check in with kids helps to strengthen their trust of who is in their environment and what is going on,” Pizzi said.

Growing up with AFOs

As children develop personalities and personal tastes, the issue of making clothing work with AFOs is no longer just about comfort; the focus can shift to what’s socially acceptable to children and their peers.

“I think it was around age six that I started to become aware of clothes and how things matched or how they looked,” Pizzi recalled. “I remember that I had a terrible time with jeans. I wore a lot of stretchy pants or sweatpants.”

Parents and practitioners need to be aware of the balance between doing what is best for the child’s health and allowing the child to have some say in his or her life, Pizzi said.

“Telling a child, ‘I’m going to give you your own voice’ can be very helpful. Kids respond better if they feel they have some sense of control,” she said.

The first place to start that process is with the AFOs themselves. The ability to dress up AFOs with colors, decals, or stickers has been a tremendous boon.

First, it’s an opportunity for the child to be involved in the decision-making process and gain a sense of control by choosing a design or color. It also “makes the brace less medical,” Hartung pointed out. “It allows them to make it more about something fun for a toddler, or something fashionable for an older child.”

Martin said that being able to bring that sense of fun or fashion can even tip the balance in favor of the AFOs in terms of patient compliance.

“I’ve had parents tell me that their child goes to school with the AFOs featuring a cartoon character and other kids say, ‘I want those!’ It almost becomes a personal fashion statement. And if it’s more appealing to the kids, then they are more likely to wear the braces,” Martin said.

For specific clothing choices, trial and error is what the experts advised, and easy-on, easy-off shouldn’t be completely discounted. Annette is the mother of an 8-year-old girl with Charcot-Marie-Tooth disease. Her daughter has worn AFOs for two years, and she’s finding the balance between being independent and learning to work within some of the limits presented by the AFOs.

“She generally wears sweats [or] elastic waistband pants to make it easier for her to use the restroom,” explained Annette, who lives in California. “She does want to assert her independence as she’s growing up; she gets herself dressed. She is learning to adapt and do things differently than everyone else.”

The experts emphasized two points: making the AFOs part of the child’s routine and letting them make decisions—within limits.

“As soon as you can, have them learn that the AFO is part of their daily lives,” is a recommendation that Hartung makes to parents. “If they are involved from the very beginning, it’s not something that is so torturous. It becomes part of their everyday activities.”

An example would be for the parent or caregiver to give a daily review of the process of putting on and taking off the AFOs, even if the child may be too young to fully comprehend what’s happening. And older children can be allowed to put on their AFOs and shoes and socks themselves, rather than making it something mom or dad does to them.

Pizzi suggested starting this process in children as young as 4 or 5 years, and framing the importance of the AFOs within a story.

“In the story, the adult could describe the activities that they are going to be able to do today because the child is wearing the brace,” she said. “Or maybe there’s a special occasion coming up like a birthday or a family event. The parent could talk about how the child will be able to participate in that more easily with their braces on.”

One of the major rules of parenting also applies to kids and their AFOs—children like making decisions, but they also need boundaries, and that includes their clothing options.

“I think it’s important to structure the choices in a specific way,” Martin said. “A parent can say, ‘Do you want to wear the blue socks or the red socks with the braces?’ You didn’t give them a choice regarding the braces at all; it’s understood that the braces will be worn. It’s up to [the adults] to structure the choices so we get the outcomes we want.”

Raducanu suggested that parents avoid the trauma of trips to the mall with younger kids to try on clothes and shoes. Instead, parents should go out and do the shopping themselves, choosing items that they believe will work with the AFOs, and then let the child try on and choose from that selection at home.

Having to return the unwanted items will require an additional trip to the store, but he pointed out that it’s a small price to pay to avoid “a huge battle that’s only going to leave everyone upset and feeling resentful. That can impact the child’s attitude towards the braces.”

OMG: Teens and AFOs

Photo courtesy of SureStep.

There’s another chapter in that anthology about the rebellious teen. It’s a time when the desire to fit in is paramount, and having to wear AFOs may not fit the bill at all.

“One of the biggest problems with teenagers is compliance,” Hartung said. “They don’t want to wear something that makes them different.”

Once again, the experts agreed that helping teens fit their fashion needs around their AFOs starts with the devices themselves. The use of light, thin materials, for example, can help AFOs fit under trendy skinny jeans or leggings, Hartung said.

“Our goal is to make sure the brace functions properly and that the patient will wear it,” Hartung said.

If a teenage girl is keen on wearing below-the-knee skirts, but the extra material gets stuck on the Velcro closures, Hartung said she would make it a priority to adjust the closure rather than ruling out the clothing item. Another option for some AFO-wearing teens is to switch from a taller brace to one with a lower profile.

Pizzi pointed out that a militant attitude about AFO compliance can be especially hurtful to an adolescent who is already struggling with the usual teen issues beyond the AFOs. In an essay she wrote as an undergraduate at Rivier University in Nashua, NH, Pizzi recounted a day of shopping for clothes with her mother as “somewhat torturous. If the pants are good, the shoes are not, and if the shoes are good, the pants are not. It is a vicious cycle. I cannot win. So, after…the tenth pair of pants that I had tried that day…I had had enough and stormed out of the store.”

So what’s the best way to manage that kind of frustration, anger, and resentment? Pizzi advised that the teen years would be a good time to introduce psychosocial therapy, which can benefit both the patient and the parents.

Parental protectiveness can be heightened when a child requires AFOs. But teenagers are more likely to misinterpret that caring and become less compliant.

“Who wants their parents nagging them constantly, and what parent really wants to be the nonstop nag? I think it’s important to start empowering teens to more fully understand what they could or should do to help themselves live comfortably with their disability,” Pizzi said. “The idea is that the teen can be a bit more proactive or responsible for his own body and the parent can still [offer] support…and step back or out of the protective role.”

Professional counseling is one option, as are support groups and workshops. Pizzi said she found it very cathartic to pen her college essay.

Photo courtesy of Allard USA.

As for those potentially perilous shopping trips, Pizzi summed up her advice in one word: patience.

“When my mom and I would go to the mall, it could take us five hours to find the right shoes or clothes. We just had to accept that it was going to be a slow, steady process. Eventually, we’d find clothes that were cute and functional, but we both had to be willing to put in the time,” she said.

Ultimately, practitioners and parents alike may have to accept that a teen’s AFOs may spend more time in a closet or school locker than they will on the patients.

“At some point, they may stop wearing the AFOs, or wear them out of the house and then take them off the minute they get to school. And you may just have to let that go. The patient needs to make that decision to wear their AFOs,” Raducanu said.

Adaptive apparel

Parents searching online for clothing options for their AFO-wearing children are likely to come across adaptive apparel. These are clothes and shoes that have been specially designed to work with AFOs. Examples include “hatchback shoes,” that allow the back of the shoe to open up so the foot can slide in; longer, seamless socks that are long enough to cover AFOs of varying heights; and pants that snap closed on the sides so that the front “breaks away” rather than having to be pulled down.

Most of these items are not cheap. Socks can cost as much as $10 a pair, while shoe prices generally run between $80 and $100. Our experts were divided on whether adaptive apparel is worth that kind of money.

Hartung expressed reservations, especially given that kids grow out of shoes and clothes so quickly. She also pointed out that AFOs—and the children who wear them—can take their toll on shoes.

“An older child who is on the go may not take the time to put the shoes on and take them off properly. He might try to jam his foot into the shoe, brace and all, and then collapse the back of the shoe,” she said. “If a parent has spent a lot on a specialty shoe, that’s a problem. I’d rather get them into a regular shoe.”

On the other hand, Annette has opted for adaptive shoes for her daughter and, on the whole, considers them a good fit.

“They are expensive—it cost me about eighty dollars-plus to order her a pair of shoes…and it’ll take two to three weeks before we’ll get them. They can be a good investment since they easily fit her braces,” she said. However, Annette warned that shoes styles are limited. “They only have one pink color for girls and a Mary Jane-type shoe.”

Pizzi remembers her mother investing a few hundred dollars in a couple pairs of adaptive shoes.

“They were very expensive, and she knew I’d outgrow them pretty fast,” Pizzi said. “But they were beautiful shoes, and I did wear them without any complaint.”

Shalmali Pal is a freelance writer based in Tucson, AZ.

X-rays show femur with nail inserted before length- ening (left), after lengthening (center), and after the lengthened bone has healed (right). (Images courtesy of John Herzenberg, MD, FRSCS.)

Intramedullary nail is ‘a step forward’

By Samantha Rosenblum

Preliminary evidence supports the use of a new internal technique for limb lengthening, according to data from the International Center for Limb Lengthening (ICLL) at the Rubin Institute for Advanced Orthopedics of Sinai Hospital in Baltimore.

The ICLL has used an internal lengthening nail in 82 patients, 48 of whom were younger than 18 years, in the past two years and reports that patients experience less pain, less joint stiffness, and fewer general complications compared with lengthening using external fixation.

The nail, an extendible intramedullary rod for lengthening the femur and tibia, utilizes a magnetic-powered external remote controller that lengthens it noninvasively. Use of the nail addresses many problems associated with external fixation, such as pin-site infections, muscle tethering, and pain, according to the ICLL.

In a paper presented at the American Academy of Orthopaedic Surgeons 2014 meeting in New Orleans, researchers at the ICLL asked 15 patients (or parents of young patients, if appropriate) who had undergone limb lengthening with both external and internal techniques to compare the experiences. The average age at external fixation was 9 years; for internal lengthening, 14 years.

On a 10-point scale, patients on average ranked visual analog pain associated with external fixation at a 7, while the average pain associated with internal fixation was a 3. Use of pain medication averaged 11 weeks with external fixation and five weeks for internal fixation; time to full weight bearing averaged 21 weeks for both techniques. The internal device was associated with higher levels of patient satisfaction regarding ease of physical therapy, cosmetic results, complication rate, day-to-day function, and return to activity. Earlier results from the same study were presented in July 2013 at the annual meeting of the Limb Lengthening and Reconstruction Society.

Joshua Hyman, MD, associate professor of orthopedic surgery at Columbia University College of Physicians and Surgeons in New York City and director of the pediatric orthopedic fellowship at Morgan Stanley Children’s Hospital of New York, has yet to use the nail but noted that external fixation is associated with a number of potential risks.

“I always have a discussion with patients and their parents about the risk of superficial infection, which I describe as about one hundred percent,” Hyman said. Other risks include injury to nerves and blood vessels at pin sites or when the bone is cut.

The benefits of internal lengthening are not limited to physical advantages, according to John Herzenberg, MD, FRSCS, director of Pediatric Orthopedics at Sinai Hospital and of the ICLL, who presented the study findings.

“It’s certainly psychologically better for the patient because they don’t have a huge external scaffolding contraption outside their leg attached to them for months at a time,” Herzenberg said. “If someone has [internal lengthening], you wouldn’t know if they were sitting next to you. It takes the whole process and makes it more comfortable.”

Smaller versions of the original nail are becoming available, which will increase its potential for use in young children. The latest generation of the internal system is also four times stronger than the original, Herzenberg said.

Hyman says he sees a future for internal lengthening.

“It gives us another tool for treating deformity. And, when we speak about deformity, most of us think about a wavy or a curvy bone. But it also means a short bone, and it gives us another way to address this,” he said.

There have been about 700 nails implanted worldwide. But the new technique has not solved all the problems associated with limb lengthening, Herzenberg said.

“We’re still stretching muscles, we’re still stretching bone, and the bone still has to heal,” he said. “It’s a step forward, but not a panacea. It doesn’t cure all the problems of making limb lengthening a simple process. It’s still a very serious procedure.”

The new nail is also a straight nail, which limits its ability to lengthen crooked bones.

“If this is the case, you could use an external fixator with hinges that allows straightening of the fixator as well as lengthening,” Herzenberg said. Another alternative might be to perform an osteotomy to straighten the bone and then use the nail once the bone has healed.

Samantha Rosenblum is a journalism student at Northwestern University in Evanston, IL.

Sources:

Herzenberg JE, Standard SC, Landge V, Specht SC. Comparison of internal and external fixation for limb lengthening patients who have experienced both. Presented at the American Academy of Orthopaedic Surgeons 2014 Annual Meeting, New Orleans, March 2014.

Herzenberg J, Standard S, Conway J, et al. Satisfaction of patients who have undergone lengthening with both internal and external fixation: A comparison study. Presented at the 23rd Annual Scientific Meeting of the Limb Lengthening and Reconstruction Society, New York, July 2013.

Position statement cites warning signs

By P.K. Daniel

Despite the slim odds of securing an athletic scholarship and the even slimmer prospect of playing professional sports, there’s an overemphasis today on success in competitive youth sports, including specialization and elite-level training. As such, and with the proliferation of travel teams and club sports, practitioners have seen an increase in overuse injuries and burnout.

Starting a child in sports before he or she is developmentally ready, committing early to one sport, and overtraining are cited by practitioners as risk factors for overuse injuries and burnout in a recent position statement by the American Medical Society for Sports Medicine (AMSSM).

The current literature reports that overuse injuries comprise half of all sports injuries. Lower extremity overuse injuries include medial tibial stress syndrome, osteochondritis dissecans, Osgood Schlatter disease, and Sinding-Larsen-Johansson disease (SLJD). The prevalence varies by sport, ranging from 37% (skiing and handball) to 68% (running). The AMSSM, however, suggests overuse injuries are underestimated since many of these injuries don’t result in loss of participation time.

But the lack of time loss is not because the injuries don’t warrant rest.

“If they have an overuse injury and they’re not losing time, it’s more because people are not recognizing them or ignoring them, and that can then lead to the injury becoming worse,” said Joel Brenner, MD, MPH, FAAP, a coauthor of the AMSSM position statement, which was published in the Clinical Journal of Sport Medicine and the British Journal of Sports Medicine.

“The main thing for parents and coaches to understand is that since most kids are not going to become professional athletes and most are not going to get the college scholarship, we want them to have fun and learn lifelong physical activity skills,” said Brenner, chair of the American Academy of Pediatrics Council on Sports Medicine and Fitness. “The big thing is making sure that young athletes are not participating in one sport year-round, and also that they’re having some time off throughout the year from a particular sport to prevent these overuse injuries and burnout.”

Practitioners need to address sports readiness in terms of cognitive, social, and motor skill development, as age is not an indicator of whether a child is physically and developmentally prepared. This can also help to prevent overuse injuries and burnout.

“One of the problems we have is parents who expect their children to do certain sporting activities before they’re ready,” said Greg Landry, MD, a report coauthor and faculty member in the departments of pediatrics and orthopedics at the University of Wisconsin School of Medicine and Public Health in Madison. “A lot of times [children] get involved in team sports before they have the skills or the cognition to really do it.”

An excessive focus on early intensive training and competition—rather than skill development—can lead to overuse injury and burnout. These injuries can require extended recovery, and sometimes can lead to long-term complications. Ultimately, they can endanger future participation.

“[Youth athletes] often play with pain when they should be resting,” Landry said. “That potentially can result in long-term problems.”

Participating on multiple sports teams (in the same or different sports, particularly if the sports have similar components, eg, soccer and track) during a single season leads to overtraining and an increased risk of overuse injury. A 2008 study of 2721 high school athletes showed a relationship between hours of sport participation and risk of injury. Training more than 16 hours per week was associated with a significantly increased risk of injury requiring medical care.

Robbie Bowers, ATC, head athletic trainer at Rancho Bernardo High School in San Diego, likened the AMSSM position paper to one the National Athletic Trainers Association published on pediatric overuse injuries in 2011 that recommended no more than 16 to 20 hours per week of vigorous physical activity by pediatric athletes.

“With overtraining you break your body down before it has time to respond and restore itself to a stronger level before it gets broken down yet again,” Bowers said. “It’s going to find the weak spots and a lot of times it’s those apophyses [cartilaginous growth centers].”

Repeated injuries, coupled with extended recoveries and long-term effects, can affect an athlete’s quality of life.

“One of the things we see in the office is that they often have one overuse injury after another,” Landry said. “I think over time they begin to enjoy their sport less and less because of all the injuries. It does impact the fun of participating.”

P.K. Daniel is a freelance writer and editor based in San Diego, CA.

Sources:

DiFiori JP, Benjamin HJ, Brenner JS, et al. Overuse injuries and burnout in youth sports. Br J Sports Med 2014;48(4):287-288.

DiFiori JP, Benjamin HJ, Brenner JS, et al. Overuse injuries and burnout in youth sports. Clin J Sport Med 2014;24(1):3-20.

Rose MS1, Emery CA, Meeuwisse WH. Sociodemographic predictors of sport injury in adolescents. Med Sci Sports Exerc 2008;40(3):444-450.

Valovich McLeod TC, Decoster LC, Loud KJ, et al. Prevention of pediatric overuse injuries. J Athl Train 2011;46(2):206-220.

Second study assesses ‘creep’ risk

By Larry Hand

Sometimes, even when new treatment materials are available, it may be better to rely on traditional options. Such may be the case when it comes to choosing a molding material to form casts for children with clubfoot or fractures.

Researchers from the University of Vermont College of Medicine (UVM) in Burlington reached that conclusion after comparing three cast-molding materials.

“Molding plays a pivotal role in the nonoperative treatment of many conditions like clubfoot casting and children’s fractures,” said first author Steven B. Daines, MD, clinical instructor in the departments of orthopedics and rehabilitation at UVM. “A mater­- ial’s failure to hold a mold could mean a poor correction of a deformity or a loss of reduction. This could result in the prolongation of treatment, the failure of treatment causing a need for surgical intervention, or a poor outcome.”

The researchers compared 12.7 cm-wide casts of plaster, fiberglass, and soft cast. They prepared the casts in 40° C water, five layers thick, and placed over them two layers of cotton padding on 5.1 cm- and 15.2 cm-diameter foam cylinders. They used loading devices to simulate the thumb loads physicians apply when molding casts for clubfoot and other conditions that included developmental dysplasia of the hip (DDH) and femoral fracture.

For clubfoot, the loading device applied a thumb-shaped 50-N load on the 5.1-cm model for seven minutes. For DDH, the device applied a 100-N load on the 15.2-cm model, as did the device for femur fracture.

The researchers made five casts of each material and, when the molded casts were removed, they photographed them and compared maximal deformation areas of molded and unmolded casts. A large maximal deformation area meant the molding was less precise.

They found plaster was more precise than fiberglass and soft cast for clubfoot, DDH, and femur fracture because it had the best moldability, or ability to retain the desired shape.

“Our study has further emphasized the importance of considering the benefits of various materials when using them in the clinical setting,” Daines said. “At UVM, we have utilized various casting materials based on the advantages of their material properties. For example, many of our clubfoot casts are made of plaster, which helps us carefully mold casts to correct the clubfoot deformity. Some of our children’s fractures, like buckle fractures of the distal radius, do not require precise molding. We have used removable soft casts with waterproof liners to treat these fractures, maximizing patient comfort and minimizing follow-up appointments as parents can remove the casts at home.”

The UVM study, published online in February by the Journal of Pediatric Orthopedics, comes on the heels of an August 2013 study looking into the mechanical performance and displacement (or “creep”) of three materials used for casting clubfoot patients. Using a cast-testing device built to model clubfoot correction, including the internal force on the cast coming from the foot, researchers analyzed the rotational displacement and linearity of the limb-cast composite during three 10-minute intervals after the cast had set.

The results suggested that at least 65% of cast creep occurs during the first 10 minutes after setting, according to study coauthor Tamara Cohen, a PhD candidate in biomedical engineering at Marquette University in Milwaukee, WI. The amounts of displacement were small for all of the materials, indicating that any could be used with the Ponseti method of clubfoot treatment. However, displacement occurred to the greatest extent with plaster (2°), followed by semirigid fiberglass (1°) and rigid fiberglass (.4°), and those differences were statistically significant.

The two studies don’t conflict with each other, they just look at mechanical properties in different ways, according to Cohen and coauthor Peter Smith, MD, a pediatric orthopedic surgeon at Shriners Hospital for Children in Chicago.

The earlier study addresses changes over time, while the new study addresses how perfectly a cast conforms to initial molding. Both studies, Cohen and Smith said, add information on a topic that, previously, was basically ignored in the literature.

“We’ve changed over the years to using more of the soft casts for clubfoot, because it can be removed more easily. But, for the ones that are difficult to mold, the ones where we think that it would change the outcome, then we definitely use plaster. It’s what works,” Smith said.

Larry Hand is a writer in Massachusetts.

Sources:

Daines SB, Aronsson DD, Behnnon BD, et al. What is the best material for molding casts in children. J Pediatr Orthop 2014 Feb 28. [Epub ahead of print]

Cohen TL, Altiok H, Wang M, et al. Evaluation of cast creep occurring during simulated clubfoot correction. Proc Inst Mech Eng H 2013;227(8):919-927.

By Jordana Bieze Foster

Foot orthoses made with advanced design and manufacturing technologies aren’t just technically impressive—they’re also clinically relevant, according to the results of two studies presented in June by keynote speakers at the Orthotics Technology Forum (OTF) in Chicago.

In one study, an advanced design technique that incorporates plantar pressure data into the traditional shape-based method was used to create foot orthoses that significantly reduced forefoot plantar pressures and ulcer recurrence in high-risk diabetic patients more effectively than conventional foot orthoses. The findings of the randomized controlled trial were published in the July issue of Diabetes Care.¹

“Most of us worship at the altar of foot shape at the moment,” said study coauthor Peter Cavanagh, DSc, PhD, who presented the findings at the OTF. “When you are choosing where to put an offloading intervention [eg, a metatarsal pad], the chances of getting it in the right place are very small. So we end up with trial and error, where the trial is your best guess and the error is the development of a foot wound.”

Cavanagh is Endowed Chair in Women’s Sports Medicine and vice chair of research for Orthopaedics and Sports Medicine at the University of Washington in Seattle and a cofounder of State College, PA-based DIApedia, which has commercialized the orthotic design algorithm used in the study. Because it is more expensive to create foot orthoses that incorporate plantar pressure data, Cavanagh and colleagues have been working to persuade Medicare officials of the need to establish a new billing code. The researchers are expecting to hear more in November, he said.

“A change in payment is required based on the evidence of efficacy and cost effectiveness,” he said. “I think we are one of the few disciplines in healthcare that is unaccustomed to making an investment in hardware.”

The second study looked at advanced manufacturing techniques, concluding that personalized foot orthoses created using two different types of additive manufacturing were at least as
effective as standard polypropylene orthoses for improving biomechanics and subjective measures in 15 patients with early rheu­ma­toid arthritis. Each study participant wore all three types of devices, each for seven days in random order. The findings were epublished in July 2013 by Arthritis Care and Research.²

The study also found that outcomes with the devices made using the two additive techniques, fused deposition modeling (FDM) and selective laser sintering (SLS), did not differ significantly from each other. Of the foot orthoses rated by patients as ineffective, none were SLS devices and just three were FDM devices, versus five standard devices.

While FDM is typically done in a clinic, SLS is more often factory-based and utilized by what the researchers call small-to-medium enterprises (SMEs).

“I think this has given the SMEs confidence that they’re headed in the right direction,” said study coauthor Prof Jim Woodburn PhD, FCPodM, interim director of the Institute for Applied Health Research at Glasgow Caledonian University in Scotland, UK, who presented the findings in Chicago.

Options in subtractive milling materials still outnumber those for additive machines. (Image courtesy of Delcam Healthcare Solutions.)

By Emily Delzell

Practitioners and orthotic laboratory owners at the fourth annual Orthotics Technology Forum (OTF) thinking about switching from traditional design and manufacture methods to digital technology got an up-close look at two distinct systems—additive and subtractive—that promise to deliver quality devices in less time and at lower cost.

Both additive and subtractive machines are highly automated, allowing for rapid orthotic prototyping and manufacture, but their capabilities and compatibility with the needs of individual practitioners and practices are quite different.

OTF presenter and biomechanist Géza F. Kogler, PhD, CO, compared the subtractive process with sculpting marble; to create a 3D structure, the artist begins with a solid marble block, then “subtracts” material to realize his design. Subtractive machines can use either 3D data or 2D CAD data to produce an object.

Additive manufacturing, also called direct digital manufacturing, creates 3D objects by joining, fusing, or solidifying materials layer by layer.

CAD-CAM manufacturer Delcam Healthcare Solutions, a forum sponsor along with Freedom Machine Tool, Stratasys, nora, Acor, Tekscan, Kiwi, JMS Plastics, and Fisher/Unitech, brought in two office-sized systems for attendees to compare and consider.

Subtracting

Subtractive manufacturing in the modern sense has been around since the 1940s, but in recent years engineers have developed smaller, more powerful, and easier-to-use computer numerically controlled (CNC) routers.

Patrick Bollar, CEO of Diversified Machine Systems (DMS) in Colorado Springs, CO, introduced OTF attendees to one of the newest examples of subtractive technology, a 3-axis machine designed with input from Delcam and first introduced in April at the Pedorthic Association of Canada’s annual PAC symposium.

The enclosed machine has a 20″ by 16″ workspace and is designed to fit safely into an office environment—and through a standard office door. Its features include a grid fixture-type vacuum table, which Bollar said can cut material of any hardness below that of steel, and an industrial controller instead of the more common PC-based control.

Machines controlled by computer-fed data, said Delcam North America’s Vice President of Sales Maida Koller, who worked with DMS to develop the router, are limited by the rate at which data can be fed into the machine.

“We call it ‘starving for data,’ and the result is that you can’t get as smooth a finish,” she said. “The type of controller on this machine is the kind that’s typically found only on big, far more costly industrial machines, and eliminates that problem.”

Bollar said the machine, which can be used with a large range of materials, can produce about three pairs of foot orthoses every half hour.

Attendees were enthusiastic about subtractive technology, and at least one, Rick Prenger, CPed, who owns Walking Wellness in Ottawa, Canada, has made the move into digital technology since the conference, purchasing a subtractive system, digital scanner, and CAD-CAM software.

Using traditional plaster casts and foam box impressions, two people working in Prenger’s lab can currently turn out 10 to 12 pairs of orthoses a day. Going digital, he said, will eventually push that figure up to 30 pairs a day.

The old way of doing things, he said, is no longer economically viable, and his urgency to make the switch played a role in his purchasing decisions. The software, he ultimately bought, for example, wasn’t his first choice, but his top choice wasn’t available immediately and would have pushed his implementation date from this September to January 2015.

He’s been researching various systems for about a year and attending conferences to glean information. The OTF, particularly the opportunity it provided to talk with other practitioners and sales reps about different systems, helped solidify his plans, he said.

“I really liked the openness [of the OTF],” he said. “At some conferences people are very guarded about their trade secrets; here everything was wide open.”

Although Prenger has chosen subtractive technology for now, he said he plans on bringing in an additive machine in a couple years.

“The technology [additive] isn’t quite there yet—I think there are lots of glitches to be worked out, but the potential to build things into layers, with different areas being harder or softer as needed, is incredible. It will change how we’re looking at people’s feet, how we’re looking into gait pattern, and how we’re going to build the orthotic for the problems that we see.”

Adding

Changing the way practitioners assess patients’ feet and design orthoses was a theme echoed by Kogler, director of the Clinical Biomechanics Laboratory at Georgia Institute of Technology in Atlanta, who presented pilot study data showing that different foot regions vary in stiffness.

Additive machines allow practitioners to design almost any structural feature they can imagine. (Images courtesy of Delcam Healthcare Solutions.)

Kogler and his team measured the loading orientation of 19 foot regions with a 2-axis inclinometer, finding significant variation in initial stiffness and displacement in different regions.

“Most current orthoses have the same stiffness throughout, which is good for production, but we need more—we need to understand these variations to create optimal devices that control stiffness in different regions of the foot,” Kogler said.

Additive technology, with its ability to fuse different materials and create complex shapes of varied density in different areas of a single orthosis, will allow orthotic practitioners to draw on mechanical engineering principles in new ways that take advantage of research like Kogler’s.

The range of materials that can be used in additive machines is still limited compared with subtractive machines, but capabilities are expanding rapidly; the Stratasys 3D printer demoed at the OTF, for example, can use up to 14 different materials at once.

Additive technology appealed to two clinicians working in an orthopedic practice with a strong focus on foot and ankle surgery.

“We have multiple surgeons performing delicate and complicated surgery daily,” said Hannah Dwyer, a pedorthic resident and manager of orthopedic footwear at Midwest Orthopaedic Institute in Sycamore, IL. “Our main use would probably be using MRI-reading software to build surgical practice models. A tool that could potentially cut down on surgery times would allow us to have patients in and out faster, not only boosting productivity of surgeons, but also helping our patients.”

Thomas Dwyer, DPM, who works alongside his daughter at Midwest Orthopaedic, said, “I can see both technologies fitting into our practice. If any of our surgeons want to do templates, we would get the additive technology and use the machine for orthotics as well. If they’re not interested now, I would buy subtractive technology. When the additive technology adds more capabilities, maybe printed shoes, and drops in price, I would switch.”

Photo courtesy of MD Orthopaedics.

Clubfoot researchers have begun to report long-term data that continue to solidify the superiority of the conservative method over surgical intervention in most cases. But variations to the traditional Ponseti method are arising, particularly in developing countries, and may alter outcomes.

By Larry Hand

As most clinicians treating children for clubfoot have shifted over to the Ponseti method from comprehensive surgery, more information is surfacing on long-term outcomes, as well as short-term outcomes in lower- and middle-income countries.

Although many physicians say that, even today, practitioners should faithfully follow the method developed by the late orthopedist Ignacio Ponseti, MD, at the University of Iowa Hospitals and Clinics in Iowa City, some variations exist in daily practice and other variations are cropping up in published research papers. No one is arguing, however, about the effectiveness of the Ponseti method.

The traditional Ponseti method is ideally applied one to two weeks after birth and involves gently stretching the tendons and plaster casting the leg, changing the casts every four to seven days for about eight weeks. Often, prior to the last cast, a physician will perform a tenotomy, snipping the heel cord; the child wears the final cast for two to three weeks. To prevent recurrence of the deformity, children then wear a brace consisting of shoes mounted on a bar for 23 hours a day for three months, and then at night for several years, usually until children are aged 3 to 5 years.

“The Ponseti method will always be best, as it spares the joints,” Angela M. Evans, PhD, senior lecturer in the Department of Podiatry at La Trobe University in Bundoora, Victoria, Australia, told LER in an email. “However, in more complicated cases [eg, arthrogryposis multiplex congenital] or very rigid cases, some surgery—mainly posterior release and Achilles lengthening—may be required. But initial casting may reduce the extent of surgery needed, which will likely be better in the longer term.”

“Surgery is almost a last resort, but nonetheless, every now and then it’s a necessary thing to do,” said Susan T. Mahan, MD, MPH, assistant professor of orthopedic surgery at Harvard Medical School in Boston.

Mahan, herself, uses the Ponseti method.

“I will attempt serial casting techniques in all patients with clubfoot, because I think the results of that are just the best,” she said. “The truth is that for most healthy kids, the Ponseti technique does terrifically. It’s really the rare kid that we end up having to reach for the surgical option.”

Long-term outcomes

Extensive surgical procedures involving soft tissue releases, which were popular before the advent of the Ponseti method, resulted in scarring, stiffness, and even overcorrection, which necessitated additional surgical procedures in many patients, according to David A. Spiegel, MD, associate professor of orthopedic surgery at the University of Pennsylvania Perelman School of Medicine in Philadelphia.

“In contrast,” he explained, “the Ponseti method is minimally invasive and does not disrupt joints and tends to preserve motion. Ponseti feet may be mildly undercorrected, but this is usually not apparent clinically. Basically what you’ve got is a foot that moves better and functions better over the long term into adulthood.”

Few studies have published long-term outcomes associated with the Ponseti method, but a case-controlled study reported in Clinical Orthopaedics and Related Research¹ earlier this year (see LER:Pediatrics, February 2014, page 4) offers some details about long-term outcomes of the Ponseti method compared with comprehensive surgical release.

Researchers at the Shriner’s Hospital for Children in Chicago examined the records of individuals treated between 1983 and 1987 at two separate institutions with different treatment methods; 24 were surgically treated and 18 underwent the Ponseti method.

The Ponseti group had significantly greater ankle plantar flexion range of motion (ROM) and ankle plantar flexor and evertor strength, a lower incidence of ankle and foot osteoarthritis, and less pain compared with the surgical group. Both clubfoot groups had reduced ROM and more weakness and pain compared with controls who did not have clubfoot, findings that point to a need for better methods of improving these variables to achieve the best possible function in adulthood.

Undercorrection and overcorrection after Ponseti treatment can be hallmarks of congenital clubfoot, and problems can crop up in adulthood or at younger ages, according to a 2010 study.² Issues included valgus deformities from overcorrection, varus deformities from undercorrection, and degenerative conditions such as arthritis that may result from the underlying congenital deformity or its treatment.

Global popularity

Now that the Ponseti method has reached global popularity, some variations in protocols and methods have evolved. In a systematic review published in April 2014 in Clinical Orthopaedics and Related Research,³ Chinese researchers delved into reported variations in manipulation, casting, and percutaneous Achilles tenotomy, as well as whether bracing type and protocol made a difference in relapse prevention.

A prenatal ultrasound image of a child with bilateral clubfoot. (Image courtesy of the Boston Children’s Hospital Department of Orthopedics.)

Of the 19 studies evaluated, they found that researchers described their methods poorly in 11 and clinicians didn’t follow the main Ponseti principles in three. The brace deviated from the recommended type in three studies, and in another three, the prescribed brace wear times differed from those of the traditional Ponseti method. In addition, indications for recognition of relapse and its management differed from the traditional Ponseti method in some studies. The researchers concluded that clinically important variations in outcomes might have resulted from deviations from the traditional Ponseti method’s manipulation, casting, tenotomy, and bracing techniques and relapse recognition and management protocols.

In the same journal the following month,⁴ Spiegel called for journals to require that, “as a minimum,” authors provide detailed treatment protocols in their methods sections so that the results of different studies can be adequately compared.

“Do it exactly as the man says, or don’t say you’re doing the Ponseti method,” Spiegel said.

That has already begun to happen with some researchers—many of whom have had to adapt protocols to accommodate challenging conditions in developing areas around the globe.

“The impact of Ponseti casting on low-income countries is exciting,” said Luke S. Harmer, MD, MPH, an orthopedic trauma fellow at Carolinas Medical Center in Charlotte, NC. While at the University of Calgary in Alberta, Canada, he coauthored a paper on providing care in these areas.⁵

“With this [Ponseti] technique we are able to treat children with a low-tech, low-risk, low-infrastructure method that provides results better than our previous surgical algorithm,” he said.

Evans’ group in Australia has been conducting a sustainable clubfoot program in Bangladesh called Walk for Life (WFL).⁶ Since 2009, the group has been evaluating Ponseti clubfoot treatment in children using a Bangla clubfoot tool, a measuring system they
developed based on existing validated instruments and adapted to local factors, including overcrowded clinics, difficulty traveling to clinics, lack of basic facilities, and poor healthcare infrastructure. The Bangla tool incorporates three domains: parent satisfaction determined by interview, gait function, and clinical foot evaluation.

In an evaluation of the first 5000 feet treated in the program,⁷ Evans and colleagues found that the WFL results demonstrate that “rapid case ascertainment is possible in a developing world setting with appropriate logistical support.” Among the adaptations to local factors was the use of physiotherapists and paramedics to do the casting, along with ongoing training and regular clinical review of their technique by physicians.

In a two-year review of the WFL program, epublished in May 2014 by the Journal of Pediatric Orthopaedics,⁸ Evans and colleagues described results achieved at the 10 largest WFL clinics. Among 400 cases selected randomly from 1442 children, 99% could walk independently. According to Bangla clubfoot tool scores, parents were highly satisfied but said the cost of the treatment (3000 Taka, or $38.48) was not affordable.

“We found that if the treatment were not free, most children would not have had access, and so would not be corrected and walking,” Evans said. “We estimate there are some five thousand new cases born annually [in Bangladesh]. The significance of clubfoot is enormous in the developing world where walking avails working and earning, or not.”

Among local adaptations to traditional Ponseti methods, she said, are more frequent cast changes in which each cast stays on for about three days rather than a week.

“It can at least halve the time a baby is in plaster. We used this in [the Polynesian kingdom of] Tonga and wrote up the cases,”⁹ she said.

In that paper,⁹ Evans and colleagues reported two cases of “fast casting” in which children were cast and recast four times in one week followed by tenotomy, with both babies showing consistent correction of the foot deformity 10 weeks after start of treatment. The researchers noted they used “more rather than less” manipulation prior to casting.

Rosalind Owen, BSc, MSc, of the Institute of Child Health at University College London, UK, said, “One variation which has shown comparable results to Ponseti is the ‘accelerated Ponseti technique.’ This has shown good outcomes in Malawi and reduced the amount of time parents accompanying children for treatment have to stay away from their work and families.”

In that Malawi randomized clinical trial,¹⁰ researchers found no significant outcome differences among 40 patients (61 feet) who were randomized to either standard weekly cast changes or to changes three times a week. The median number of days in plaster was 16 for the accelerated group, compared with 42 for the control group, and researchers followed 36 of the 40 patients for six months. Researchers said it is too early to know how or if fast casts would affect long-term outcomes.

Context

In a 2012 review of clubfoot treatment outcomes in low- and middle-income countries,¹¹ Owen and colleague Gayatri Kembhavi, PhD, found the Ponseti method was more effective than other conservative techniques but not directly comparable to surgery. They found that “contextual factors” influenced outcomes, but that clinicians who used the Ponseti method took more measures to overcome those factors than did clinicians using other techniques.

The factors include resources needed to provide care, materials needed for treatment, appropriate training for providers, and parents’ abilities to collaborate with providers.

Some variations can have negative effects, Owen said.

“Incorrect ‘Ponseti-style’ treatment can include using below-knee casts,¹² not using the foot abduction brace [or not using it long enough], not doing tenotomy, and failure to identify the lateral head of talus and use it as the fulcrum of movement in casting,” she said. “These would result in probable slipping of the casts and noncorrection of the deformity, relapse of deformity, incomplete correction of deformity, and ineffective manipulation and casting, respectively. These are only some of the variations with negative outcomes.”

But not all variations have such consequences, Mahan of Harvard said.

“I think there will always be some variations in everyone’s interpretation [of the Ponseti technique],” she said. “One basic variation is whether to use plaster or fiberglass casts. The type of bracing and the length of bracing that’s done after serial casting is another variation. Some kids will get a tenotomy and some kids won’t. I think most of us follow Ponseti guidelines, but even within his guidelines, there’s some wiggle room for variation.”

Ponseti vs open surgery

In a meta-analysis published in 2013 in the World Journal of Orthopedics,¹³ researchers analyzed results from 12 studies published between January 1950 and October 2011 comparing clubfoot treatment with the Ponseti method or open surgery. They found that, while patients managed with the Ponseti method had higher rates of excellent or good outcomes than surgical patients, the difference barely missed statistical significance (p = .053). Still, they recommend the Ponseti method. Follow-up in the studies averaged 15.7 years.

“Serial manipulation and casting has been widely accepted as the initial treatment of idiopathic clubfeet, and surgical soft-tissue release is reserved for clubfeet that cannot be completely corrected, in case of relapse of the deformity or in case of nonidiopathic clubfoot, such as clubfoot associated with arthrogryposis,” lead author Marios G. Lykissas, MD, PhD, a pediatric orthopedic surgeon at Cincinnati Children’s Hospital Medical Center in Cincinnati, OH, told LER in an email.

The researchers found a correlation between a larger anteroposterior talocalcaneal angle and higher rates of excellent or good results, but no other significant correlations with functional or radiographic outcomes.

“Noncompliance of the family to follow the brace protocol is associated with unexpected high recurrence rate ranging from thirty to forty five percent and subsequent early onset arthrosis and pain if the recurrence is left untreated,” Lykissas explained. “There is no association between poor bracing compliance and families’ educational level, income, or cultural origin. Distance from the treatment centers and accessibility to the healthcare system are important parameters that may also adversely affect compliance, and secondarily, the success rate. In addition, concurrent illnesses may affect management of clubfeet with the Ponseti method.”

As always, compliance

“Compliance with casting and, even more importantly, boots-and-bars bracing, is important to outcomes,” Luke Harmer said. “This is part of why family education is so integral in the care of these children.”

For Mahan, who has authored a paper on factors predicting prenatal detection,¹⁴ family education begins before the baby is born.

“For me, it’s a conversation that often starts prenatally, because I often meet with these families prenatally or certainly start talking with them early on about the importance of longtime bracing, and I start that discussion at the first encounter,” Mahan said. “The serial casting and the initial correcting of the clubfoot, pretty much all families comply. They can almost see from the first cast the improvement of the foot. But once the foot’s corrected, the greatest challenge we have is to try and encourage the foot not to return to its clubfoot position, and the most reliable way to do that is consistency with the brace.”

Larry Hand is a writer in Massachusetts.

Some evidence suggests that neuromuscular training before puberty can help further reduce anterior cruciate ligament (ACL) injury rates. But young children respond differently to instruction than their older counterparts, which means early intervention requires some creativity.

By P.K. Daniel

A 10-year-old boy arrived at a Santa Monica, CA, sports medicine clinic with a knee injury. He had suffered a noncontact injury that occurred late in a soccer game. Diagnosis—partially torn anterior cruciate ligament (ACL).

The clinician described the boy as “a good little athlete.” He plays year-round soccer, going from American Youth Soccer Organization competition to club play. Then there’s the tournament season, followed by all-stars. His injury will require a three-month layoff and eventually neuromuscular training (NMT)—exercises designed to target motor control deficits.

The benefits of neuromuscular training for correcting bio­mechanical patterns associated with increased risk of ACL injury have been well documented.¹ But the vast majority of those studies have been done in adolescents and young adults. Now researchers and clinicians are exploring the potential rewards and challenges of implementing similar training programs in younger children.

The problem

The rate of ACL injuries in athletes younger than 18 years has increased in the past two decades.² The incidence of ACL injuries in skeletally immature patients is relatively low, but is growing as more children participate in organized sports and specialize at an early age.²

“The incidence curve is continually shifting to the left,” said Holly Silvers, MPT, director of research at the Santa Monica Orthopedic & Sports Medicine Research Foundation.

Silvers said ACL injury in children was practically unheard of 15 years ago, but she is treating it more and more. The reasons are multifold.

“Too many young athletes are specializing in one sport earlier and earlier, in addition to losing time in free play,” said Julie Eibensteiner, DPT, owner of Laurus Athletic Rehab & Performance in Minneapolis, MN. “As a result, kids are lacking the development of physical competence and ability to interact well with their physical environment and self regulate their physical activity.”

Although the documented evidence is limited, some of these same experts think children can benefit from integrative neuromuscular training.

“We [as a society] are not doing any preparatory conditioning for our youth,” said Greg Myer, PhD, research instructor of sports medicine at Cincinnati Children’s Hospital. “Our youth used to be outside. We used to have them playing. They used to develop these neuromotor control patterns naturally. Those opportunities are diminishing. There’s less opportunity for free play.”

Like Eibensteiner, Myer also blames an increase in sports specialization.

“I’m not a supporter of that because I value the components and the massive motor control adaptations that occur between sports,” he said. “If they are going to specialize early, they’re going to need this integrative neuromuscular training even more.”

Although both boys and girls experience almost equal numbers of ACL injuries, female adolescent athletes have the greatest risk. The incidence rate per total exposure for female athletes is four to six times higher than their male counterparts in similar sports.² Female athletes aged between 15 and 20 years account for the largest number of ACL injuries.²

Prior to the adolescent growth spurt there’s no observed difference in male and female athletes’ relative risk for ACL injury, but during puberty those rates increase sharply, especially in girls.² Decades of research have shown that the relative risk of ACL injuries, however, can be reduced by one-half to two-thirds through neuromuscular training. The American Academy of Pediatrics reported in April that ACL injury risk can be reduced by as much as 72%, especially in women aged 14 to 18 years.²

“You can absolutely reduce ACL injury rates pretty successfully and pretty consistently,” Silvers said.

Early intervention: A bit of a conundrum

With pubescence now occurring at earlier ages than in the past, and maturation not having a one-age-fits-all delivery, Silvers subscribes to early intervention. She also acknowledges the challenges.

“We have a bit of a conundrum,” she said. “Obviously, the earlier we get them the better. Before these patterns are well entrenched from a motor-plan perspective, before these things become habitual, we want to get them. But it’s very difficult to do that in an eight-year-old.”

The best time to implement preventive measures is debatable. Some suggest it is when neuromuscular risk factors are the highest—during early pubertal maturation. However, others argue this is too late.

“If you wait until after puberty, they’re already playing for several years at high risk because they have the increased mass, and that’s what’s tearing the ACL,” Myer said. “They may have had these control patterns—they were deficient when they were younger but they just weren’t large enough to tear their ACL.”

The question is whether this prevention training can be successfully applied to younger athletes.

Some researchers, including Myer, have suggested that neuromuscular training intervention needs to be incorporated as early as possible. However, the research is still out on how effective NMT is among younger children.

“The solution is you have to start early,” said Myer. “You have to start training younger kids before they hit puberty, and then provide them complementary and supplementary preparatory conditioning as they go through puberty. Then they come out the backside with a stable neuromuscular profile that [we can continue to fine-tune through training]. That way you’re not starting from scratch at a very difficult stage.”

Too often, kids are going from being inactive to jumping into competitive sports programs without integrative neuromuscular training, Myer said.

“They’re not doing these training programs that build that profile of safety and enhance their movement mechanics and motor skills,” he said.

Myer acknowledged that at the maturation level everything goes haywire, but suggested that having a solid neuromuscular foundation before puberty sets in can still be helpful.

“Even what they’ve developed, they have to relearn it and manage those large changes in their anatomy and control measures, and they have to be able to adapt,” he said. “The issue is if they’ve never learned these mechanics prior to puberty, you have to teach them during that period—the phase when it’s most difficult and control is hardest. So that’s not the best solution.”

Skepticism

Tim Hewett, PhD, director of the Ohio State University Health and Performance Institute in Columbus, isn’t convinced that this training is transferable to a child who doesn’t already have the problem. An 8-year-old isn’t yet experiencing what he described as a “post­puberty machine-motor mismatch.” When girls go through puberty they get bigger without the corresponding muscle power. Their motor isn’t powerful enough to manage the machine at a level that keeps the body at great neuromotor control in very high-velocity, high-force, high-torque situations.²

“You have to have that mismatch to teach how to control it or how to increase the power, increase the control,” said Hewett. “If you do it prior to that taking place, I have doubts whether it would be effective. A pill doesn’t work until you actually have the condition.”

Hewett said the research shows that just at or after the adolescent growth spurt is when you want to institute the neuromotor training.

“I don’t think we can prepare someone in advance of that,” he said. “It’s optimistic to say let’s get to them early and make sure this doesn’t happen. I don’t know that we can alter that pattern.”

The research

A 2009 study led by Lindsey DiStefano, PhD, ATC, assistant professor in the Department of Kinesiology at the University of Connecticut in Storrs, may support that. The study involved an injury prevention program focused on landing biomechanics, after which the older children (aged 14-17 years) showed more gains than the younger children (aged 10-13 years).³

However, Myer said some research suggests kids can benefit from integrative neuromuscular training. He was part of a research group led by Avery Faigenbaum, MD, a world leader in exercise training in children, that looked at fitness performance in 7-year-olds.⁴ Integrative neuromuscular training was performed two times a week over an eight-week period for the first 15 minutes of physical education (PE) class. Forty children (24 girls) participated.

The boys performed better than the girls at baseline in the push-up, standing long jump, single-legged hop, shuttle run, and .8-km run. However, the girls responded better to the integrative neuromuscular training effects relative to the control group on the curl-up, long jump, single-legged hop, and .8-km run. The boys did not demonstrate similar improvements from the intervention.¹¹

This likely indicates that girls were more responsive to the intervention, Myer said. It might also suggest that boys need increased intensity, or a higher-level stimulus, to influence adapta­tions, he said, noting that this was not tested in the study.

“That tells me it’s in those early stages that [the girls] can start to adapt,” said Myer, a study coauthor. “We had huge gains in their power and performance.”

The study, published in the March-April issue of the Journal of Athletic Training, concluded that NMT is an effective and time-efficient addition to PE for enhancing motor skills and promoting physical activity in children.⁴

The authors did a follow-up study⁵ with the same kids for another eight-week period. During this period there was a cessation in integrative neuromuscular training due to school vacation. Substantial decreases in performance were observed. A child’s neuromuscular profile, Myer said, is highly plastic, modifiable, and responsive to its environment.⁵

“They respond to the stimuli very well,” said Myer. “Children need to be exposed to a high level of motor control developmental strategies as they’re maturing and can actually achieve their neuromuscular control potential.”

Injury outcome data studies have not been completed. Myer said he is currently involved in a randomized controlled trial of the same type of PE-based intervention in younger versus older children. The responders, Myer hypothesized, are going to be most prevalent among the younger kids.

“That said, I think the programs are effective in older kids, and they are effective in males, but I think our best money is going to be training younger athletes,” Myer said.

Challenges

But there are challenges, such as attention and compliance.

“Compliance is a huge factor in the effectiveness of the neuromuscular training programs,” said Eibensteiner. “Those who are more compliant have significantly less risk of injury.”

Silvers is in favor of introducing the subject of neuromuscular training to kids and making them more body aware.

“We just want to start the education and initiate the conversation,” she said.

Her foundation is in the sixth year of a 10-year study on early intervention funded by the National Institutes of Health. It involves 250 children from across Southern California’s Coast Soccer League, one of the largest soccer leagues in the country. The children were aged 8 and 9 years at the outset of the study and will be followed until they are aged 18 and 19 years.

Silvers and colleagues also utilized Coast Soccer League participants when the group developed the Prevent injury, Enhance Performance (PEP) Program in 2000 to reduce ACL injury risk.¹³ The prepractice program consists of a warm-up, stretching, strengthening, plyometrics, and sport-specific agility drills to address potential deficits in the strength and coordination of the stabilizing muscles around the knee. PEP was initially intended for those aged 12 years and older. However, an addendum for those younger than 12 is now available.

PEP includes drills using cones. Adaptations for younger children include shorter distances between the cones, jumping over only visual lines on the field or a flat 2-inch cone, and landing with two legs instead of one. The emphasis during plyometric activities is on the landing technique, not the height of the object they jump over. Performing the exercises with the correct technique in the allotted time frame is emphasized over repetition.

There are different approaches when incorporating neuromuscular training for children. The training has to adapt to their learning capacities. Keeping kids’ attention through a simple, progressive, and fun approach is recommended.

“Training younger children is focused on feedback and their mental capacity,” said Myer. “For the very youngest kids we use balloons to help control their focus. As they get a little bit older, we can take away that supportive tool.”

The children hold the balloons while squatting, lunging, and moving in different directions to enhance muscle strength as well as agility, balance, and coordination, Myer said. The balloons help by slowing down the children’s catching and kicking movements, enabling them to master the new skills and experience success.⁴

Silvers’ programs don’t involve balloons, balls, or other equipment. She doesn’t want any distractions for the short period of eight to 15 minutes.

“We just want them to be aware of [their bodies] in space,” she said. “As they get older you can layer the program, make it more challenging. In the beginning, keep it simple.”

FIFA 11+ is an injury prevention program designed for soccer players aged 14 years and older. The program has proven to reduce overall injury rates⁷ and lower extremity injuries significantly.^7-9 Hewett has been working on a child-friendly version of FIFA 11+ with neurosurgeon Jiri Dvorak, MD, who is the chair of FIFA’s Medical and Research Centre (F-MARC) and FIFA’s chief medical officer professor, and Mario Bizzini, PhD, also of F-MARC.

The three-year study is less than a year old. It is targeting several thousand kids between the U-9 and U-14 age groups. The training is simplified and focuses more on play while still addressing the same neuromotor deficits. Randomized controlled trials in Germany are currently being conducted.

“Theoretically, it’ll work,” Hewett said. “Truthfully, we don’t really know, but it’s definitely worth a try. It still gets at these potential imbalances of ligament dominance, quadriceps dominance, leg dominance, trunk dominance. It teaches better neuromotor control of the body, posterior kinetic chain activation, control of hip and knee joint. It really focuses on the kids not allowing their hip and knee to collapse in but in a simpler, more play-like approach.”

Another challenge is ensuring these no-cost or low-cost programs are implemented with high fidelity. The American Academy of Orthopaedic Surgeons has joined other organizations, such as the National Athletic Trainers Association, in promoting injury prevention among kids through a campaign called STOP (Sports Trauma and Overuse Prevention). The message is being conveyed through public service announcements, social media, and other mediums in a multidisciplinary approach.

To assist with the proper implementation, many of these neuromuscular training programs are available digitally, either online through streaming video or in CD form. An app for smartphones is currently being developed for the PEP program.

An adolescent male patient with left-sided Blount dis- ease who continued to gain weight despite realign- ment of his leg. (Photo courtesy of Sanjeev Sabharwal, MD, MPH.)

Weight loss requires other tactics

By Hank Black

Surgical correction of the varus alignment that is characteristic of Blount disease does not lead to greater patient activity or reduction in body mass index (BMI), according to a recent study.

“Many parents and physicians see excess weight as a product of immobility due to limb deformity and believe if the limbs were aligned properly, the child’s activity level would increase and obesity would resolve. We conducted a retrospective study to answer that question,” said study coauthor Sanjeev Sabharwal, MD, MPH, professor of orthopedic surgery and director of pediatric orthopedics at Rutgers-New Jersey Medical School in Newark.

Blount disease occurs in early-onset and late-onset forms, with some authors recognizing an intermediate (juvenile) type. The disease is characterized by progressive multiplanal deformities such as tibial varus, procurvatum, and internal torsion, along with limb shortening. The result can be gait deviations and limb-length discrepancy (LLD), which may contribute to premature knee osteoarthritis.

Children with Blount disease tend to be heavier than their peers and have higher rates of obesity. In a 2007 study published in the Journal of Bone and Joint Surgery, Sabharwal and colleagues found a significant relationship between BMI and severity of radiographic deformities in children with early-onset Blount disease, but not in those with late-onset disease.

Some milder cases of early onset disease may spontaneously resolve, and long-leg braces may be helpful in some young children. After age 4 years, however, surgery to restore limb alignment and correct LLD is utilized more frequently.

The current Rutgers study, which was published in the March 2014 issue of the Journal of Pediatric Orthopaedics, assessed 51 children with Blount disease (23 early onset, 28 late onset). The 47 who had gradual surgical correction with external fixation also received six to eight weeks of inpatient rehabilitation that included dietary control and nutritional counseling. They were followed postsurgery for an average of 48 months.

The surgical treatments (osteotomy, growth modulation, or both) significantly improved mechanical axis deviation and LLD. BMI, however, increased relative to preoperative measurements in 76% of the children. At follow-up, the average BMI of the cohort had increased from 35 preoperatively to 38, and the number of morbidly obese (BMI ≥ 40) children had increased from 12 to 20.

“Now we have more information for parents, but unfortunately not good news,” Sabharwal said. “We could only influence the child’s diet for the rehabilitation period, and it’s also difficult to impact long-standing eating habits when so many patients are economically disadvantaged minorities with limited food choices.”

Brian Shaw, MD, an associate professor at the University of Colorado in Denver and Children’s Hospital Colorado in Aurora, said he was not surprised by the findings but agreed the information could be useful for parents.

“The study answered a valid question for patients with Blount disease, although I wouldn’t have expected any other result—previous studies of orthopedic procedures such as knee joint replacement have shown no positive effect on BMI or obesity,” Shaw said. “However, we can now counsel patients more knowledgeably that lower BMI should not be a part of expectations of treatment. The study does support research into whether bariatric surgery and other non­orthopedic surgery methods can address patients’ obesity and lack of activity.”

In fact, the one patient who demonstrated the greatest BMI and weight reductions in the Rutgers study was an adolescent who refused the orthopedic procedures and underwent bariatric surgery.

“Perhaps weight-loss surgery before or after realignment surgery may be an option for some,” Sabharwal said, “but it’s clear that [realignment] surgery alone is not enough.”

Future research might offer additional insights.

“Our study had no control group for comparison, so it would be interesting to do a prospective study comparing these patients either with Blount patients not undergoing a weight-reduction program or compare their activity level with other obese children who have normal limb alignment,” Sabharwal said.

Orthopedic surgeon John G. Birch, MD, of the Texas Scottish Rite Hospital in Dallas, said he is pleased that doctors are using growth-modulation techniques for lower leg alignment.

“Deliberate deceleration of growth on the tibia’s outer part so the inner part will have the power to grow into alignment produces less morbidity than osteotomy,” Birch said. “A well-designed trial of long braces for Blount disease would be useful, [but] it would be very difficult to get off the ground since this is not a common condition.”

Hank Black is a medical writer in Birmingham, AL.

Sources:

Sabharwal S, Zhao C, Sakamoto SM, et al. Do children with Blount disease have lower body mass index after lower limb realignment? J Pediatr Orthop 2014;34(2):213-218.

Sabharwal S, Zhao C, McClemens E. Correlation of body mass index and radiographic deformities in children with Blount disease. J Bone Joint Surg Am 2007;89(6):1275-1283.

Birch JG. Blount disease. J Am Acad Orthop Surg 2013;21(7):408-418.

This child, aged nearly 4 years, was diagnosed with microcephaly, autism spectrum disorder, and hypotonia. She uses sensomotoric orthoses in Piedro boots and receives regular physiotherapy for gait, gross motor skills, motor planning, and strength. She also undergoes occupational therapy. (Photo courtesy of Therapies for Kids.)

Study finds link to social milestones

By Shalmali Pal

In children with autism spectrum disorder (ASD), lower fine and gross motor skills are associated with higher disease severity scores, according to new research that supports the concept of earlier motor skills intervention in this population.

Kids, aged 12 to 33 months, whose mean scores for gross motor skills were almost six and a half months behind typical milestones for their chronological age, also had similar deficits related to social-communicative skills, reported Megan MacDonald, PhD, an assistant professor of Movement Studies in Disability at Oregon State University in Corvallis, OR.

Improving an autistic child’s motor skills early in life may provide a better foundation for improving other areas, such as social communicative skills, language, and adaptive behavior, MacDonald and colleagues wrote in the April issue of Adapted Physical Activity Quarterly.

Early therapy for communication and behaviorial issues has proven worthwhile, MacDonald said, but with this study, her group sought to answer the question: How can we make early intervention better?

“Motor skills is a content area where I think there is room for improvement,” she said.

The group evaluated 159 children (mean age, 27.6 months), the majority of whom (110) had a confirmed diagnosis of ASD.

The Mullen Scales of Early Learning, which includes a subscale focused on gross motor skills, was used to assess cognitive development. Autism severity was measured with the Autism Diagnostic Observation Schedule, a semi-structured assessment of communication, social interaction, and play.

The results indicated that gross motor skills were significantly related to calibrated autism severity. They also found that having a confirmed ASD diagnosis, age, and nonverbal problem solving were significant predictors of calibrated autism severity.

“Fine motor skills were 9.5 months behind chronological age and gross motor skills deficits were 6.4 months behind chronological age,” they wrote, adding that the relationship of motor skills and calibrated autism severity held constant across variables such as nonverbal problem solving, age, and gender. “In other words, this relationship is not driven entirely by intellectual ability.”

From a clinical perspective, the findings suggest that a lack of motor skills may hinder an autistic child’s success in achieving social-communicative milestones, MacDonald explained.

“In our interventions for teaching communicative skills, we use an approach called active play,” she said. “If we can improve motor skills first, how does that help active play? If we improve the motor skills set, will that give the child more opportunities to participate in active play and social interactions?”

While the standard of care in pediatric ASD generally calls for addressing social-communicative issues first, would it make sense to first focus intervention on building and improving gross motor skills? MacDonald said it was too soon for a definitive answer to that question.

“It is another potential avenue to consider for interventions. If our goal is to reach developmental milestones, then it may be worthwhile to address those motor skill milestones early on. I think [early intervention] is a chance for healthcare providers to work together,” she said.

Jason Henry, MSPT, LO, of Hope Orthotics in Spring, TX, pointed out a child with ASD who has delayed gross motor skills– especially due to hypotonia–would benefit overall from the use of corrective orthoses that provide proprioceptive feedback and help to control excessive malalignment issues dynamically.

“A child with hypotonia should be able to pronate and supinate within reason, so that appropriate motor learning can take place,” Henry added.

Clare MacFarlane, PhD, of Therapies for Kids in Sydney, Australia, concurred that improving gross motor skills can serve as the foundation for other therapies, based on the developmental pyramid theory.

MacFarlane explained that in this pyramid, the base level of development starts with basic sensations (taste, touch, etc). Higher levels of the pyramid include gross and fine motor skills, communication, and advanced cognitive function.

“If there are pieces of the pyramid missing…the higher levels of pyramid may not have the best opportunity to develop,” she said. “I think [giving a child] independence with sitting, movement, crawling, walking, and investigating their environment will assist with the more advanced skills, such as communication and comprehension.”

MacDonald said her group is working on a pilot project to test an intervention protocol that focuses on motor skills. She also said they’d like to do a longitudinal analysis of how weak motor skills at a young age manifest in children with autism when they are older.

Shalmali Pal is a freelance writer based in Tucson, AZ.

Source:

MacDonald M, Lord C, Ulrich DA. Motor skills and calibrated autism severity in young children with autism spectrum disorder. Adapt Phys Activ Q 2014;31(2):95-105.

Robotic training device setup. (Reprinted with permission from Sukal-Moulton T, Clancy, T, Zhang LQ, Gaebler-Spira D. Clinical application of a robotic ankle training program for cerebral palsy compared to the research lab- oratory application: Does it translate to practice? Arch Phys Med Rehabil 2014 May 1. [Epub ahead of print])

Home device use could be next step

By Hank Black

A robotic system developed at the Rehabilitation Institute of Chicago (RIC), previously shown to have efficacy in a research lab setting, is also effective when used in a physical therapy clinic for ankle training in children with cerebral palsy (CP), according to a new RIC study.

In the clinic study, researchers demonstrated the feasibility and effectiveness of using robotic-assisted therapy in a busy after-school setting. Their protocol was associated with significant improvements in participants’ plantar flexor and dorsiflexor range of motion (ROM), strength, spasticity, mobility, balance, and selective control of the lower extremity, although not the gross motor function measure.

The in-clinic findings appeared online in May 2014 in the Archives of Physical Medicine and Rehabilitation. The results compared favorably with outcomes from the pilot lab-based study, which was published in the May 2011 issue of Neuro­rehabilitation and Neural Repair.

“A critical component of the training may be the use of the robotic device with an intensive, therapist-guided program to maximize gains achieved with the device, but this question remains to be formally investigated,” said first author Theresa Sukal-Moulton, PT, DPT, then a doctoral student and physical therapist at RIC and now a postdoctoral fellow at the NIH Clinical Center in Bethesda, MD.

In addition, recent advances in haptic feedback and gaming were included in the clinic study to enhance challenge and motivation with goal-oriented motor learning.

The pilot study showed the robotic device was an effective tool for passive stretching combined with active movement training and demonstrated improvements in joint biomechanical properties, motor control performance, balance, and mobility over baseline.

“We wanted to see if similar gains could be seen in a busy clinical setting with all its noise, distractions, and diverse patient diagnoses,” Sukal-Moulton said.

Participants in both studies had mild to moderate spastic CP. The 12 participants in the lab-based pilot study (average age, 7.8 years) received a one-hour therapy session three times a week for six weeks. All sessions employed the robotic device.

In the real-world study, 28 participants (average age, 8.2 years) were seen in 75-minute sessions twice a week for six weeks. Physical therapists who were not part of the core research team had significant input into the study design, resulting in the use of the robotic device for the first 30 minutes of each session, with the remaining 45 minutes devoted to individually tailored, functional movements.

The robotic training included 10-minute periods each of passive stretching, active-assisted movement, and either active or active-resisted movements. In the active-assist mode, participants played a video game controlled by their ankle movement, scaled to their ankle’s passive range of motion, and including a time delay before the device offered assistance. Utilizing the system’s touchscreen, therapists could add resistance to movements and change the game to require more or less force to move a cursor. Various gait, balance, and strengthening exercises were employed to maintain or extend the ROM gains achieved during robotic training.

“A robotic system can provide well-controlled instruction for children with CP, but it is unlikely that changes in range of motion alone can explain the clinical improvements we saw,” Sukal-Moulton said. “The critical component is more likely the large repetition of practicing a movement with an enhanced range, either in isolation or in a functional context.”

Eventually, the system might be used in the home environment, with initial set-up and periodic monitoring by a physical therapist.

“In the home, the number of days with device use might be increased for an additional boost, particularly when children have growth spurts,” Sukal-Moulton said. “This is in line with the current trend in physical therapy toward intensive bouts of interventions followed by the opportunity to do other things that are not therapy driven.”

CP specialist and surgeon Jon R. Davids, MD, said he looks forward to a comparison with other conventional therapies in a controlled trial.

“In addition, it is intriguing to anticipate the use of these devices in the home setting,” said Davids, a professor of orthopedic surgery at the University of California-Davis and at Shriners Hospitals for Children in Sacramento, CA, where he also is medical director of the Motion Analysis Laboratory. “Although the technology’s initial expense is significant, the potential increase in number of therapeutic sessions may offset that cost.”

Hank Black is a medical writer in Birmingham, AL.

Sources:

Wu YN, Hwang M, Ren Y, et al. Combined passive stretching and active movement rehabilitzation of lower-limb impairments in children with cerebral palsy using a portable robot. Neurorehabil Neural Repair 2011;25(4):378-385.

Sukal-Moulton T, Clancy, T, Zhang LQ, Gaebler-Spira D. Clinical application of a robotic ankle training program for cerebral palsy compared to the research laboratory application: Does it translate to practice? Arch Phys Med Rehabil 2014 May 1. [Epub ahead of print]

By Megan Smith, CO

Case Study One: Addison

Addie was prescribed SMOs (supramalleolar orthoses)  when she was aged 18 months and  presented with developmental delay, hypotonia, and pronation. Prior to receiving her SMOs, she had been pulling to stand for four months and cruising for three months. She had just begun to take some independent steps, but was still very unstable.

Addie started pulling to stand around age 14 months (five months delayed) and had mastered walking with one hand held at age 18 months (six months delayed). Prior to receiving her SMOs, her gross motor skills rate of change (months/skills) was .61, compared to .43 for a typical child of the same age.

After receiving her SMOs, Addie gained gross motor skills much faster, at a rate of .52, compared to 1.03 for a typical child of the same age. By the end of the study, Addie was walking backward and running, and had closed the gap to be only two to three months behind her peers. She gained three months of skills in four months.

Case Study Two: Sawyer

Sawyer was prescribed SMOs (supramalleolar orthoses) at age 18 months, when  he was demonstrating pronation, hypotonia, and ligamentous laxity. He was pulling to stand and cruising but not yet standing independently. In addition to the SMOs, he received physical therapy once a week for the duration of the study.

On the day he received his SMOs, Sawyer was cruising and taking some steps with both hands held for assistance. Relative to a typical child, his developmental delay was eight months. Four months later, he was walking with a medium guard and narrow base of support. He had mastered eight to nine months of gross motor skills in four months, putting him about three to four months behind his peers. His gross motor skills rate of change (months/skills) after receiving his SMOs was .28 compared to .51 for a typical child of the same age.

Case Study Three: Jeremiah

Jeremiah was prescribed SMOs (supramalleolar orthoses) at age 15 months, when he was demonstrating pronation, hypotonia, and ligamentous laxity. He was pulling to stand and cruising but not yet standing independently. In addition to the SMOs, he received physical therapy once a week for the duration of the study.

On the day he received his SMOs, Jeremiah was cruising and taking some steps with both hands held for assistance. Relative to a typical child, his developmental delay was six months. Four months later, he was walking with a low guard and narrow base of support. He had mastered eight to nine months of gross motor skills in four months, putting him about one to two months behind his peers. His gross motor skills rate of change (months/skills) was .34 compared to .51 for a typical child.

Case Study Four: Kate

Kate was prescribed SMOs (supramalleolar orthoses) at age 18 months, when she was demonstrating pronation and hypotonia. She was pulling to stand, cruising, and taking some steps with trunk support, but not yet standing independently.

On the day she received her SMOs, her developmental delay was eight months compared to a typical child. Immediately after receiving her SMOs, she started to stand independently and take steps with just one hand held. Three months later, she was walking with a low guard and narrow base of support. She had mastered eight months of gross motor skills in three months, ultimately putting her three months behind her peers. Her gross motor skills rate of change (months/skills) was .25 compared to .51 for a typical child of the same age.

Background: Each child in this case series was assessed every other week for 16 weeks (12 weeks for one patient who moved out of state) to determine mastery of items 23, 26-28, 30-39, 41, 42, and 45 (ranging from “pull to stand” to “run”) on the Peabody Developmental Motor Scale. Test instructions were modified as needed for children to understand them. Parents were included in each session and encouraged to play with the child in order to demonstrate the targeted skills. Graphs illustrate age of mastery for each item number for the hypotonic child compared to a “typical” child, with linear trend lines illustrating rate of change, and demonstrate the improved mastery of skills after prescription of SMOs.

For this family, one tiny extra chromosome led to a journey of self discovery

By Suzi Klimek

When our son was born, we prayed for a healthy baby with 10 fingers and 10 toes. Our prayers were answered. Three years later, those same prayers were said for baby number two. And, once again, our prayers were answered. Baby number two was also born with 10 fingers and 10 toes. But there was one microscopic addition: an extra copy of chromosome 21. Otherwise known as: Down syndrome.

Being told that your child was born this way—it was a shock. It’s just not something you think will happen to you.

I remember when the doctor came into my room. He sat down next to me and explained that our daughter likely had trisomy 21. I asked, “What is trisomy 21?”

At the time, my husband was at home with our 3-year-old son. I called him crying and confused. When he arrived at the hospital, he reassured me that everything was going to be OK.

“Julia is our daughter, and we love her unconditionally, so let’s just move forward and figure it out as we go,” he said.

I can’t say that I rolled with it quite the same way. I cried—a lot. I kept asking, “Why us?” People kept telling me, “God doesn’t give you more than you can handle.” And I would reply, “Well…I think God confused me with someone else!”

It wasn’t until I received the first sympathy card that it all made sense to me. I tried not to be offended as I knew the intention was good. It was then that I realized I didn’t have any reason to be sad. I was, and still am, blessed to have this child. She is our winning lottery ticket. But that didn’t mean it would be easy.

Within days of Julia’s birth, we were connected with the Indiana First Steps Early Intervention Program. Julia started physical therapy at four weeks old. Developmental, occupational, and speech therapies soon followed. Julia was not born with any additional medical needs. However, because most children with Down syndrome have hypotonia, or low muscle tone, we knew that needed to be addressed. Because of the hypotonia, our physical therapist said that Julia would benefit from supramalleolar orthoses (SMOs) to help her walk. She specifically recommended SureStep.

We are lucky to live near the SureStep corporate headquarters. Julia was 18 months old when she first started wearing SureStep SMOs, and she was walking shortly after that. Since then, she hasn’t stopped. Now 10 years old, she loves to play soccer, ride her bike, and climb on the playground jungle gym.

It’s funny how things happen for a reason. If it weren’t for Julia, my path would have never crossed with Bernie Veldman, owner and developer of SureStep, and I wouldn’t be the company’s executive director of marketing. I’m thankful that I can work for a company that is so passionate about helping children with special needs.

As people in our community meet Julia, they are drawn to her charming personality. She is a celebrity of sorts. At a local clothing store that we frequent on mommy–daughter days, one of the employees adores Julia and always asks her to model her clothes when she tries them on. Our son’s baseball team has adopted her as a member of their team.

It’s no different at school. Her classmates high-five her in the hallway, help her with her backpack, and open the door if she needs assistance. They even write her letters to tell her that she is their best friend.

Julia is too young to understand her celebrity status, but I know that all the love and attention she receives gives her the confidence she needs to develop into an even more amazing young lady.

As I look back on these 10 years, I wonder why I ever shed a tear about having a child with Down syndrome. It was the path our family was chosen to take, and I know we’re up for it. I could not imagine life any other way. Without a doubt, our family and friends have been profoundly affected by this gift. The people we’ve met, the challenges we’ve faced, and even my career choice have all been influenced by one little girl.

Her name is Julia.

Suzi Klimek lives with her husband and two children in Granger, IN. Originally from Wayzata, MN, she relocated to Granger after graduation from Saint Mary’s College in Notre Dame, IN. Klimek is the executive director of marketing for SureStep.

A version of this article was previously published in the June 2011 issue of Family magazine.

New research underscores years of positive clinical results

By Cary Groner

When it comes to orthotic management of pediatric patients with hypotonia, the medical literature is only beginning to document the effectiveness that clinicians have been reporting anecdotally for years.

“The efficacy of treating low tone with orthoses is very poorly studied, but it is the standard of care because I feel intuitively that we can help these kids, and because we’ve seen good clinical results,” said Kathy Martin, PT, DHS, a professor and director of the Doctor of Physical Therapy program at the University of Indianapolis.

Important considerations related to orthotic management of children with hypotonia include which children need what type of device (whether an ankle-foot orthosis, a supramalleolar orthosis, or an in-shoe foot orthosis) and how early to intervene.

The evidence

There is, at least, some research that examines the case for bracing. Lisa Selby-Silverstein, PT, PhD, coauthored a 2001 paper reporting that foot orthoses affected the gait of children with Down syndrome; effects included reduced heel eversion and transverse plane foot angle during gait, but also a decrease in walking speed.1 And, in a small (two-subject) 2012 study, researchers found that a flexible SMO improved functional motor performance, whereas a less flexible one impeded it.²

Martin published the results of a more robust and compelling study in 2004. Seventeen children with Down syndrome (mean age 5 years, 10 months) were given flexible SMOs and tested three times over 10 weeks on a number of measures that included standing, walking, and running. She found that the devices were associated with significant improvements in postural stability both at the time of fitting (week 3) and after seven weeks of wear (week 10). Martin noted that, the more challenging the task, the more time was needed to see significant improvement, but also that degree of joint laxity did not affect results.³

Martin and a couple of colleagues—Julia Looper, PT, PhD, assistant professor of physical therapy at the University of Puget Sound, in Tacoma, WA; and Shelby-Silverstein, who is associate professor of physical therapy at Neumann University in Aston, PA—have put together a proposal for a multisite clinical trial to assess treatment variables, but, even if funding becomes available, results are likely years away.

Individual practice

As noted, clinicians have seen results in their own practices.

“When a normal child is learning to walk, their arms come out, their knees are straight, they’re moving from side to side, trying to balance,” said Curt Bertram, CO, National Orthotic Specialist for Hanger Orthopedic Group who works at Children’s Hospital of Wisconsin, in Milwaukee. “That’s normal in the prewalking child; but a child with Down syndrome may be in that phase at twenty-eight months, and the condition of their foot isn’t going to improve. So in those cases I think it’s important to realign the foot in an SMO, to provide a more stable base of support so they can get the proprioceptive feedback they need for balance.”

Bertram said if the orthosis is flexible enough, it will allow the child to explore movement range but still return them to a more normal biomechanical alignment in the gait cycle.

“I’m in favor of treating as soon as possible because they’re already delayed,” he continued. “These kids don’t have normal biomechanics or muscle tone. We sometimes see older children with Down syndrome who haven’t been treated with orthotics, and they typically end up with very deformed, rigid, stiff foot deformities.”

The needs of individual patients with hypotonia can be complex and challenging, however.

“We work very closely with the physical therapist in making these decisions,” said Jason Henry, MSPT, LO, the practice manager at Hope Orthotics in Spring, TX. “You’re looking at age, anatomy, range of motion, strength, coordination, and functional status. I start with an SMO, then work my way up the chain. Are they able to stand independently? If not, they may need a little more support than you get with an SMO. Do they go into a great deal of recurvatum? If so, how are we going to address that?”

Henry prefers flexible SMOs because they allow all the important motions in the sagittal plane—plantar flexion and dorsiflexion at the ankle, forefoot extension, and the like.

“The child can pronate and supinate out of subtalar neutral, which is what you want,” he said.

He too believes in bracing earlier rather than later.

“Some people say you shouldn’t put braces on kids until they’re twenty-four to thirty months old, and I don’t agree with that,” he said. “They’re getting further behind on developmental milestones, and the body is maturing. The limbs are elongating and they’re motor learning things in improper alignment, so they’ll walk, but what’s the quality of the gait?”

Changes

Faye McNerney, PT, DPT, a pediatric physical therapist in Troy, OH, has seen the field evolve over the course of her 36 years of practice.

“I used to lock up the foot using a rigid SMO or AFO, because I was afraid of what would happen to their foot if they kept rolling inward,” she said.

A 2008 study helped quantify the deleterious effects of such approaches, she noted, though it didn’t include children with hypotonia. In a British case study of a woman who had her lower leg immobilized following a foot fracture, researchers measured substantial and rapid loss in leg muscle volumes both proximal and distal to the immobilization site. Recovery remained incomplete up to two months after the cast was removed.4 And, in another study by the same lead author, children immobilized due to hip osteochondritis showed increased ankle stiffness throughout the immobilization period.⁵

McNerney has seen the positive effects of SMOs as her own practice patterns have changed over the years.

“With the flexible braces we immediately see their feet come closer together,” she said. “Over time, we don’t see the flat-footed gait pattern; a heel-toe pattern develops. In my adult patients with Down syndrome who don’t have these flexible SMOs available, I see a much more flat-footed gait pattern.”

When to start

One controversy that has arisen in recent years has to do with the point in the child’s development when orthotic intervention is most appropriate. In her studies of Down syndrome patients, for example, Julia Looper has argued that intervening prior to the acquisition of independent walking may interfere with the child’s motor-learning skills.^6-8 Kathy Martin acknowledges that this may be the case but points out that it is also important to consider the larger context of cognitive and emotional development.

“We agree that once these kids have learned to walk, orthoses help them,” Martin said. “Where we disagree is that Julia’s research suggests we should not intervene prior to the acquisition of independent walking. My clinical experience has shown that if you put kids in an orthosis when they are interested in pulling to stand, they start walking sooner. And once they gain independent mobility, their cognitive and social-emotional development explodes. So when I look at a child with Down syndrome who already has cognitive delay, I think the earlier we get them exploring their environment independently, the better their ultimate cognitive function will be.”

In her conversation with LER, Looper addressed this collegial disagreement by conceding the point.

“She’s right,” she said of Martin. “My take is that, in kids who are going to be wearing orthoses forever, it doesn’t matter that much if they develop control of their ankle. They will be in the orthoses anyway, so exploring their environment and getting the cognitive development is more important.”

Looper’s position now is that children with milder conditions, who may need to wear orthoses for a shorter period of time, might benefit from watchful waiting.

“I think there is a gradation, and it depends on how much calcaneal eversion we’re seeing, and what’s going on generally with the child,” she said.

Martin emphasized, however, that orthotic interventions have to be carefully selected and matched to the child. Too little is a problem, but so is too much.

“When you and I walk, we pronate and supinate around midline; that’s what normal gait is,” she said. “Being stuck in pronation throughout the gait cycle isn’t normal, but putting a child in a rigid orthosis that holds them in midline constantly isn’t normal either. That’s where some of the newer SMOs come in; they are more lightweight plastic, thin and flexible and dynamic, so they can bring a child back to midline but not rigidly hold them there. They can pronate and supinate around midline as they walk, and that should be the goal.”

Early intervention

If there’s a practitioner who has redefined the meaning of early intervention, it’s Debbie Strobach, MA, PT, a pediatric physical therapist and splinting specialist at Mercy Children’s Hospital in St. Louis, MO.

“We splint children with AFOs before they leave our neonatal intensive care unit,” Strobach said.

According to Strobach, preemies born earlier than 30 weeks have a higher risk of hypotonia and muscle imbalance, and may present with significant ankle and hindfoot eversion. This, in turn, causes problems with adjoining muscles.

“We see the fibularis become more powerful than the anterior and posterior tibialis, stretching the medial structures of the foot and putting children at risk for pronation when they’re getting ready to stand,” she said. “I want them in good alignment so that, as they grow, their tendons and ligaments can support the ankle and foot correctly. That helps the adjoining muscles contract and work as they should, as well.”

Strobach and her colleagues use customized solid ankle-foot splints similar to AFOs to counteract such forces. She has been able to assess the effects of her interventions simply by comparing them to children who didn’t receive them.

“Kids from other hospitals who weren’t splinted in the NICU come to us at nine, twelve, eighteen months of age, and they may even have contractures in the fibularis muscles. We find that the babies can wear splints for maybe a month in the NICU, and then we’ll follow them up as outpatients for another month or two. In the NICU, they wear the splints three hours on and three hours off, coordinated with their feeding and handling times. We find that if we splint them early, very few need splints later when they start to stand and walk independently.”

Strobach and her colleagues also deal with older children with benign hypotonia, who typically get SMOs or UCBL (University of California Biomechanics Laboratory)-style splints.

“Some of those kids are still not walking by sixteen or eighteen months, so we start them as soon as we get a referral,” she said. “Most of them graduate into a plantar orthosis by the time they are four to six years old, and then they’re done. The key is having splints that don’t inhibit the muscles so you can strengthen them and align the foot correctly throughout the day, for good muscle balance.”

Bringing research home

Such approaches, however convincing, remain more a matter of personal clinical experience than evidence-based medicine. Given the paucity of controlled trials, then, clinicians are increasingly documenting their results.

Megan Smith, CO, director of clinical research for SureStep, a maker of flexible SMOs, presented the findings of four recent case studies at the O&P World Congress in Orlando in September 2013.

“The four kids were fifteen or sixteen months old, and presented with diagnoses of developmental delay, benign hypotonia, and significant pronation,” she said. “We put them in flexible SMOs and followed them for sixteen weeks, with film every other week.”

When the children had mastered a skill on the Peabody developmental motor skills scale (eg, pulling to stand, cruising, taking steps, walking fast), Smith noted the child’s age and compared those numbers with the Peabody norms.⁹ Although the participants began their evaluations at a baseline of about five months’ developmental delay, by the end of the sixteen weeks of study, that disparity had been cut to only a month (for more recent case studies, see “Orthotic success stories: Four cases in a series,” pages 20-27.)

“We found that the kids who wore the SMOs had a rate of change 1.8 times greater than normal,” she said. “In other words, they were acquiring gross motor skills nearly twice as fast as normal kids and catching up to their peers.

The future

As clinicians continue to define and refine the effectiveness of their interventions, and if better funding becomes available to conduct randomized trials, ideally the kind of protocols described here will become better documented, adjusted, and, where appropriate, standardized. It will make life easier for practitioners, of course, but the ultimate beneficiaries will be children whose development has been hindered by hypotonia.

Cary Groner is a freelance writer in the San Francisco Bay Area.

Early intervention can help provide a solid foundation

By Shalmali Pal

Many kids with Down syndrome, autism, and other neurological conditions may experience biomechanical limitations in the form of delayed development of gross motor skills. One of the drivers of that delay can be hypotonia.

LER spoke with a group of experts on the link between hypotonia and the development of gross motor skills. They explained why early intervention for hypotonia is worthwhile, eventually putting the child in a better position all around.

“I like to think of the body as a building and the feet as the foundation; if your foundation is stable, it is easier to build levels on your building and keep it stable,” explained Clare MacFarlane, PhD, a physiotherapist at Therapies for Kids in Sydney, Australia. “If your foundation is a bit wonky or unstable, building levels, or gross motor skills, can be harder to achieve.”

Finding their stride

For a healthy child, the acquisition of gross motor skills starts at one month, and includes capabilities such as lifting the head for a few seconds while lying on the stomach and bringing a hand to the mouth while in a supine position.

Pinpointing hypotonia by assessing gross motor skills in infants can be difficult as they progress at their own rates, and some may gain the skills faster than others. In addition, the degree of hypotonia can influence when symptoms appear. In the case of mild hypotonia, the lack of gross motor skills may not be evident until the child is considerably older.

However, in an infant with more severe hypotonia, the child may simply struggle to gain control over the head, said MacFarlane, who, along with David Wong, DPM, runs a podiatry clinic at Therapies for Kids.

“So, depending on the child, the severity, and the age, there could be a range of gross motor skills affected,” she added.

As a general rule, a 1-year-old child should pull himself to standing while holding on to support and take a few steps, an action known as cruising. Again, while gross motor skill development will vary by individual, the ability to stand unaided and walk independently usually comes at 15 months.

It’s at the cruising stage that a child with hypotonia may start to experience problems with gross motor skills, which can often be summed up in one word—stuck, said Megan Smith, CO, director of clinical research at SureStep in South Bend, IN.

“They pull to stand on time, but then they don’t start cruising within or close to a couple of months of that,” she explained. “Eventually, they may start cruising, but then they get stuck on that; they don’t really let go [of the support]. Parents will often say, ‘They don’t seem to be getting anywhere.’ Then it becomes a bit of a waiting game to see if they progress.”

The cause of this slow progression from assisted standing to independent walking is most likely an unstable pronated base at the foot and ankle, explained Jason Henry, MSPT, LO, practice manager at Hope Orthotics in Spring, TX. A child with hypotonia will lack the ability to support themselves on one leg for that brief moment before taking a step.

Of course, many people pronate to some degree, and toddlers will pronate quite a bit given that they have a fat pad on the inner border of the foot that “hides” the arch. As a result, they tend to look a bit flat-footed until the arches develop, usually by age 6 years. But children with hypotonic muscles demonstrate excessive pronation and ligamentous laxity, Smith said.

“We have to make that distinction between normal pronation and what is excessive,” she said.

Physiological signs of excessive pronation may include the heel tilting into valgus and a medial weight shift that will cause the midfoot to collapse. The knees and hips will become misaligned because of the pronation in the feet.

Subsequently, a child with hypotonic muscles who attempts to stand or cruise is likely to present with lower limb alignment issues, poor balance, and an inability to advance to jumping, going up or down stairs, running, or kicking a ball, MacFarlane said.

Older kids may show a lack of motivation to be active or suffer from excessive fatigue after short bouts of activity. Other signs that hypotonic muscles are impacting gross motor skills include poor posture, poor lower limb biomechanics and alignment, decreased coordination, motor dyspraxia, and decreased proprioception and body awareness, MacFarlane added.

Treating hypotonia: The sooner the better?

If hypotonia is making it difficult for the child to reach milestones in gross motor skills, MacFarlane said, then orthoses are in order, and are prescribed based on the child’s specific presentation.

For instance, a rigid orthotic device may reduce excessive pronation and stabilize the foot and ankle. A gait plate may be an option to reduce in-toeing or out-toeing, she said.

Specialized sensomotoric orthoses with protrusions under certain musculotendinous junctions in the foot create a push or pull effect, thus either relaxing or activating the muscle. These dynamic orthoses are prescribed for many conditions, such as cerebral palsy and toe walkers, MacFarlane explained.

“We have found a combined approach of pediatric physiotherapy and podiatry to be a hugely successful intervention collaboration for the children we treat, especially those with low tone or combined needs,” she said.

For Smith, the goal with orthotic treatment of hypotonia is to improve tone, and by extension, improve the child’s chances of gaining appropriate gross motor skills.

“[Supramalleolar orthoses] allow some normal supination and pronation, but we can put the child in a much better biomechanical position, and let the muscles be recruited properly to help with those gross motor skills,” she said.

The experts agreed that earlier is better when it comes to treating hypotonic muscles. The idea that the child may “outgrow” the excessive pronation and develop muscle tone over time isn’t a viable one, MacFarlane emphasized.

“Unfortunately there are a lot of professionals with advice to wait until seven years of age before intervention, but this simply cannot be applied to all children,” she said. “In some cases, there will be correction with time. But if I have a child coming to me at the age of five presenting with sore knees, poor coordination, delayed skills…I will definitely prescribe orthoses [along with standard physiotherapy intervention for the presenting problems].”

Henry said he also prefers early intervention for hypotonia, although usually not earlier than age 10 months to 1 year.

“It depends on the situation and presentation of the child, but we are rarely providing functional lower extremity bracing prior to early standing activity,” he said.

However, he added that they often don’t see the child at his practice until after he or she has already struggled with standing for some time, which isn’t ideal.

“Early intervention for true hypotonia can optimize a child’s ability to function with quality gait, running, and activity in general,” he said.

The point of the orthoses in this patient population is to provide dynamic stability and optimize dynamic alignment throughout activity for efficient function.

“This is much different than traditional orthotic thinking, which involves the use of rigid orthoses locked in subtalar neutral,” Henry explained. “That school of thought places alignment as the sole goal and function is left out of the picture. We do not function in constant subtalar neutral, so to ask a hypotonic child to do so doesn’t make sense.”

What does make sense? An orthotic device that gives the child with hypotonia proprioceptive feedback, working for—and not against—them to control excessive malalignment issues dynamically, Henry said.

“A child with hypotonia should be able to pronate and supinate within reason so that appropriate motor learning can take place,” he added.

MacFarlane said that, at her clinic, there is not necessarily a “correct age,” to address low muscle tone.

“We have been known to treat on a case-by-case basis, which allows some degree of freedom with ‘minimum age,’” she said. “I have referred a twenty-two-month-old for orthotics, as he had incredibly poor foot biomechanics and foot posture due to low tone and hypermobility, which was impeding his walking skill, stability, and confidence. He was just starting to walk at this age, which is defined as delayed.”

Hypotonia: A piece of the puzzle

The term “benign congenital hypotonia” applies to children with low muscle tone who have no other underlying conditions. In these cases, the hypotonia is considered mild and outcomes are generally favorable with appropriate treatment.

But in many cases, the hypotonia is linked to an overall neurologic condition, such as autism and autism spectrum disorder (ASD), Down syndrome, cerebral palsy, muscular dystrophy, and Tay-Sachs disease.

Other symptoms that can be associated with any of these disorders—sensitivity to touch, anxiety, attention-deficit/hyperactivity disorder, behavioral problems—may, in addition to low muscle tone, play a role in the delayed development of gross motor skills.

In fact, a theory gaining ground in the medical community is that helping children with hypotonia reach gross motor skills milestones can ultimately drive improvements in other areas, such as communication skills.

Megan MacDonald, PhD, an assistant professor of Movement Studies in Disability at Oregon State University in Corvallis, recently authored a study that found a link between motor skills and disease severity scores in children with autism and ASD. That study was published in the April issue of Adapted Physical Activity Quarterly.

For lower extremity professionals, working with these children and their hypotonia may mean having to indirectly manage their nonbiomechanical symptomology to get them to cooperate during their treatment course.

“Behavioral issues seen in clients with any symptomology is hard,” MacFarlane agreed. “Appointments can be successful or a disaster, a lot of which is dependent on not only the client-practitioner interaction, but the input of the parent as well.”

The experts agreed that engaging the parents of these kids prior to the first appointment can make a world of difference. MacDonald called parents “an amazing resource.”

“Many times, the parents have strategies in place for managing their children, such as a reward system or some other way to keep them engaged and compliant. So check in with [parents] beforehand and learn what that system is, because it’s good to maintain that consistency across the care spectrum. Sometimes kids are more receptive to that,” she recommended.

Building trust is particularly important for working with these patients. While healthcare professionals are no doubt pressed for time, MacFarlane and Smith suggested starting slowly.

“The child with autism may need some familiarization to the surroundings or the practitioner before they may be comfortable to be examined,” MacFarlane said. “There may need to be a desensitization routine established allowing the child to become used to touch or having orthoses in their shoes.”

Smith’s two-appointment protocol offers the child an opportunity to get used to her and the treatment process. Another advantage is that the orthotic devices she prescribes do not require casting, only measurement.

“It cuts down on the evaluation time and the amount we have to interact with the patient in a way that’s unfamiliar to them,” she said. “So, if we have a child with behavioral or sensory issues, we’ll go slow and just talk. I work with the parents while the child sits with a parent or in a lap so they start to see that it’s a safe place to be. At that first visit, we do the evaluation and measurement. They come back in for a second time for the delivery, and I’ve never had a child react in a negative way.”

Henry suggested that biomechanical experts look to what drew them to the profession in the first place—the desire to work with, and help, people.

“I don’t know that interacting with these kids is something that can be taught; rather something that probably comes naturally for someone drawn to doing this for a living,” he said. “I think the short answer lies in having patience, bringing a positive approach, and being a friend to that child and his or her family.”

Shalmali Pal is a freelance writer based in Tucson, AZ.

Quantifying the effects of hypotonia starts in the clinic

By Cary Groner

Effective management of children with hypotonia requires an understanding of how the condition affects gait. Clinicians typically rely on their professional experience when discussing the effects of hypotonia on gait in pediatric patients, partly because they trust that experience, but also because so little research has actually elucidated these effects.

“This needs to be explored from a research standpoint, because we really don’t know,” said Mark Holowka, CPO, who practices at Children’s Healthcare of Atlanta. “It’s difficult to quantify hypotonia because muscle testing isn’t reliable in such young patients. As a result, we tend to rely on what we can observe and what we hear from the parents and the physical therapists on the team.”

Pending better funding for formal studies into hypotonia and associated hypermobility, many clinicians feel they should conduct informal research as best they can.

“I think there has to be a real emphasis placed on gait analysis within clinical practice,” said Jason Henry, MSPT, LO, the practice manager at Hope Orthotics in Spring, TX. “We have to take the mindset that clinical practice is research. Many different aspects of gait can be analyzed even if you don’t have a gait lab, and that data has to be collected on a broader scale. We need more information about outcomes.”

According to Holowka, a grasp of the distinctions between pathologies associated with hypotonia is critical to clinical intervention, regardless of similarities in symptoms.

“For example, autism is more of a sensory issue, whereas in a Down syndrome patient, it’s important to focus on their joints and joint laxity,” he said. “You have to understand what is associated with each pathology, because these kids will have different developmental and physiologic concerns.”

Holowka added that performance inside the lab can differ significantly from that outside—a phenomenon he’s found more prevalent with autism patients than others. Parents are often the best source of insight about such disparities.

“The patient will walk perfectly in the lab, but then the parents say, ‘Oh no, they don’t walk like this at home,’” he said. “It’s ironic, because ideally what we want is for them to walk imperfectly in the lab so we can help them walk perfectly outside of the lab.”

Kathy Martin, PT, DHS, a professor and director of the Doctor of Physical Therapy program at the University of Indianapolis, agreed that clinicians must rely on their own observations and measurements to characterize gait in children with hypotonia. And, despite the paucity of formal studies, most clinicians seem to agree about the outlines of those characteristics.

“My opinion is that hypotonia affects gait, in that I see kids with a wider base of support and more cocontractions, who are trying to artificially create stability,” she said. “These kids are using everything but the kitchen sink to keep themselves upright because they have to work so hard to control their body against gravity.”

Such patients may be less able to adapt to environmental obstacles than typically developing children, Martin explained, and have to change their ambulatory strategies when confronted with such obstacles.

“Children with Down syndrome may drop to their knees and crawl over an obstacle rather than stepping over it,” she said. “Down syndrome also includes issues related to balance and postural control, of course; it’s not just hypotonia. That raises the issue of what is the limiting factor, why they feel they can’t step over the object—but hypotonia contributes to that.”

The reading

In the scant literature that does address hypotonia in pediatric patients, a few key points are worth noting. First, though hypotonia is often thought of as muscle weakness, researchers point out that it is more accurately described as reduced resistance to passive range of motion in joints. Weakness, by contrast, is defined as a reduction in the maximum muscle power that can be generated.¹

Many neurological conditions have been associated with hypotonia; the most commonly studied connection is with Down syndrome (DS), but others include Prader-Willi syndrome (PWS) and Ehlers-Danlos syndrome (EDS). Although hypotonia does occur in children who do not have a specific neurological diagnosis, the difficulty of categorizing such children means they are less likely to be included in scientific studies.

According to Faye McNerney, PT, DPT, a pediatric physical therapist in Troy, OH, DS has become the research standard because hypotonia is more consistent in that patient group.

“There is no gold standard for describing hypotonia, and because everyone with Down syndrome has some degree of hypotonicity, they are chosen as research subjects,” she said. “Children with low muscle tone typically have joint hypermobility, which results in various biomechanical effects. Hypermobility of the foot-ankle joint allows the feet to pronate, and with time you’ll see shortening of the peroneal muscle, lengthening of the posterior tibialis, medial rotation of the tibia, genu valgum, and hip internal rotation. As a result of these issues and poor balance, patients typically use a wide base of support during stance and ambulation.”

Curt Bertram, CO, National Orthotic Specialist for Hanger Orthopedic Group who works at Children’s Hospital of Wisconsin, in Milwaukee, agreed.

“When I talk about hypotonia, I think about Down syndrome patients, because those are the quintessential hypotonic patients,” he said. “They have a hard time with advanced ambulation skills—climbing stairs, negotiating ramps, and running.”

Indeed, because DS has been studied more than other syndromes, a review of the literature can shed at least some light on hypotonia and gait. For example, early research in DS patients reported that characteristic gait deviations included increased variability, out-toeing, flat foot at initial contact, a wider base of support, and poor foot control.²

A more recent paper by Italian researchers, published in Gait & Posture in 2008, further clarified aspects of gait associated with DS. Compared with controls, 98 children with DS (mean age 11.7 years) walking at a self-selected speed had more hip flexion during the whole gait cycle, more knee flexion in stance phase, less knee range of motion (ROM), and more ankle plantar flexion at initial contact. Moreover, ankle power was limited in terminal stance and preswing, as indicated by low propulsive capacity at push-off. Children with DS also had stiffer hip joints but more lax ankle joints.³

In a 2012 study of foot-ground interaction during upright standing, some of the same researchers found that 99 children with DS (mean age 9.7 years) exhibited larger midfoot and reduced forefoot contact areas, increased arch index values, and increased average contact pressures in the midfoot and forefoot than controls.⁴ Of course, standing measures don’t necessarily predict gait mechanics, but the authors noted that the prevalence of flatfoot in DS patients, associated with higher contact pressures, suggested the importance of clinical observation and intervention to reduce balance and gait impairment. Some gait impairments, however, would appear to be less affected by foot posture. For example, Brazilian researchers compared gait in toddlers with DS with controls and reported no differences in stiffness or lower limb cocontraction indices (CCIs) during stance; however, DS patients showed greater CCIs during swing phase.⁵

In 2011, Italian researchers also evaluated gait patterns in Ehlers-Danlos and Prader-Willi syndromes. Although this research was conducted in adults, the rare conditions have been so infrequently studied that results are worth noting here.

The two conditions were associated with different gait strategies. Patients with PWS showed some functional limitations at every level of the lower limb joints, whereas those with EDS had greater limitations that were nevertheless found mainly at the distal joints.

The authors recommended that PWS patients be encouraged to walk to improve muscle mass, strength, and energy balance, while rehabilitation for EDS patients should focus on improvement of ankle strategy.⁶ Another Italian study comparing gait patterns in adult PWS and DS patients reported a cautious abnormal gait in both groups that was even less stable in DS patients. Those with DS also demonstrated significantly less hip flexion, knee flexion, and ROM in all joints compared to PWS patients. Both groups had significantly weaker push-off than controls.⁷

Finally, Canadian researchers reported in 2011 that children with autism had significant differences in cadence and peak hip and ankle kinematics and kinetics compared with controls. The children with autism also had reduced plantar flexor moments and increased dorsiflexion angles, which may have been associated with hypotonia.⁸

As noted, clinicians have long observed similar patterns in their patients with hypotonia.

“The disruption in neurology between the feet and the brain leads to an inefficient, dysfunctional gait,” said Jim Bauman, CO, who is in private practice at Edge Homecare Prosthetics and Orthotics in Edison, NJ. “For children with low tone, gait is more staggering and appears clumsy because of what they need to do to get their bodies to move through space.”

The role of hypermobility

As noted, hypotonia is often associated with joint hypermobility, which presents its own challenges.

“Hypotonic patients end up with hypermobility, first and foremost in the foot-ankle complex,” said Curt Bertram. “These children overpronate right at initial contact, then once they get to midstance, the foot-ankle complex can’t stabilize, so their toe lever is shortened and they lose propulsion. With time and repetition in that position, they develop bony modeling that enhances the pronatory deformity.”

According to Jason Henry, ligamentous laxity and the associated lack of coordination lead to the kind of balance deficits for which DS patients try to compensate during gait with strategies such as wider stance.

“You see delays in milestones such as cruising,” Henry said. (Cruising in this case doesn’t refer to muscle cars or girls, but rather the strategy of holding onto furniture or other stabilizing objects while learning to walk.) “You’re going to see high guard, a lot of pronation, recurvatum, genu valgum, things like that. It goes all the way up, because hypotonia is global, so you see hyperlordosis, an anterior center of mass, and so forth.”

Researchers and clinicians acknowledge that they are somewhat baffled by the connection between hypotonia and hypermobility. Muscles contain motor neurons but ligaments don’t—they provide primarily proprioceptive feedback—so the physiological correlation between the conditions isn’t clear. Nevertheless, the connection is obvious in the clinic.

“Either problem can affect the development of motor skills, but it’s even worse if you put them together,” said Kathy Martin. “The ligaments are the primary stabilizers of your joints, and if they aren’t working well, your muscles are the second line of defense. Kids with low tone don’t have either, and I think that’s what leads to the delay in the acquisition of motor skills and the gait deviations we see.”

A few researchers have investigated hypermobility and gait. In a 2011 study, 29 pediatric patients with hypermobility syndrome had greater passive knee ROM but less peak knee flexion during walking than 37 healthy controls (mean age of participants was 11.5 years). Midstance knee extension during walking was increased in the hypermobile children, but gait speed was not significantly different between groups.⁹ Another study found decreased lateral trunk stability during walking in hypermobile children and adults.¹⁰ Research into the hypermobile type of EDS (in this case, in adults), moreover, reported significantly impaired balance and gait; 95% of them fell during the course of a year.¹¹ And British researchers have reported that, when joint hypermobility syndrome overlaps with genetic disorders such as EDS and Marfan syndrome, children report relatively high levels of neuromuscular and motor development problems.¹²

Further complicating the picture, however, is that hypermobility can exist by itself, absent the neurological conditions associated with hypotonia. In a study of 8-year-old schoolchildren with generalized joint hypermobility or benign joint hypermobility syndrome, but without other problems, Danish researchers reported that neither condition reduced motor competence or physical activity.¹³ A study from the Netherlands reached a similar conclusion.¹⁴

Even though research into pediatric flexible flatfoot (PFF) is sometimes used as a proxy given the scarcity of data about hypotonia and hypermobility, the clinicians LER spoke with for this article generally downplayed the idea of a correlation. Depending on severity and factors such as patient and clinician preferences, most cases of PFF are treated with watchful waiting or simple orthotic strategies. And PFF is not, in any case, a neurological condition.

“The problem with Down syndrome children is that they have hypermobility associated with hypotonia, and they end up in a severely overpronated position,” explained Curt Bertram. “It’s a rotary deformity and very difficult to correct with mild treatment like a foot orthosis. Neither hypotonia nor the associated hypermobility is going to go away in kids with Down syndrome or Prader-Willi syndrome, whereas flexible flatfoot will typically resolve as the child develops and matures.”

Clinicians will continue to recognize and treat the manifestations of hypotonia and hypermobility regardless of etiology. That’s good news for patients and their parents.

Cary Groner is a freelance writer in the San Francisco Bay Area.

Diagnostic challenges should not delay clinical intervention

By Christina Hall Nettles

Hypotonia, or abnormally low muscle tone, is by itself not a disorder but a symptom of an enormous array of issues—many of which can be difficult to diagnose accurately. Even in the absence of a specific underlying diagnosis, however, children with hypotonia can benefit from clinical intervention.

Hypotonia can result from damage to the brain, spinal cord, nerves, or muscles, or may be a result of genetic, muscular, or central nervous system (CNS) disorders. The condition appears independently from muscle weakness, although the two may coexist in some disorders, such as motor neuron disease or multiple sclerosis. Young children with hypotonia appear “floppy” and may present with inappropriate head lag, astasis, hypermobility, decreased deep tendon reflexes, and problems sucking or swallowing. Older children with hypotonia may exhibit delays in gross motor skills or coordination or problems with ligament and joint laxity, respiratory control, posture, or speech.^1,2

Demographically, hypotonia affects both genders equally and is no more likely to occur in one particular ethnic or racial group than another. It is one of the most common muscular abnormalities diagnosed in newborns with disorders such as Down syndrome, cerebral palsy, Prader-Willi syndrome, and Tay-Sachs disease yet, in some connective tissue disorders or certain muscular dystrophies, low muscle tone may not be revealed until later in life. Hypotonia is an associated symptom among many children with autism spectrum disorders,^3,4 but typically improves over time and responds to clinical intervention.

Neuromuscular specialist for children Thomas O. Crawford, MD, who treats patients at Johns Hopkins Children’s Center in Baltimore, MD, estimates more than 95% of patients he sees for hypotonia can be evaluated with a comprehensive history and physical examination.

“In central issues, muscle tone may be diminished, but there is a discrepancy between tone during power of motion and the resting tone. That’s an important distinction. If I find a child has more vigorous muscle power than tone, that leads me to concentrate on CNS issues,” Crawford explained.

Expertise matters in both performing diagnostic studies on children, such as EMG, and assessing individual abilities, he said.

“If a child is weak as well as hypotonic from a central cause, his face is less animated than, for example, a child with spinal muscular atrophy, but in children with congenital myopathies, their faces may look unresponsive but the children are not,” Crawford said.

In the 1970s, neurologists began classifying types of hypotonia, but “benign congenital hypotonia” remained a diagnosis when no cause could be found. Improvements in identifying genetic, neuromuscular, and connective tissue disorders now provide more detailed answers, opening the door to appropriate treatment responses. Still, some families live with the frustration of knowing hypotonia exists without discovering its underlying cause.

In some cases, idiopathic hypotonia resolves within the first few years of childhood, though minor cognitive impairments or developmental delays may persist.⁵ Hypotonia caused by hormonal or metabolic disorders, such as rickets or congenital hypothyroidism, must be specifically screened for but can be easily treated.

Ronald D. Cohn, MD, chief of the Division of Clinical and Metabolic Genetics at The Hospital for Sick Children in Toronto, Canada, and an internationally recognized specialist in the genetics and clinical care of children with hypotonia, helped design a diagnostic algorithm to streamline hypotonia assessment for clinicians, distinguishing primary involvement of the upper motoneuron (central hypotonia) versus the lower motoneuron and motor unit (peripheral hypotonia) to indicate, for example, the need for magnetic resonance imaging.

Cohn and genetic counselor Emily C. Lisi, MS, have concluded that hypotonia can be a symptom of more than 600 genetic disorders, with still more waiting to be identified.

“A staged diagnostic approach categorizing patients as having peripheral, central, or combined hypotonia is the most efficient to providing a rational work-up. Establishing a diagnosis is crucial for prognosis, management, and treatment strategies and for ascertaining an accurate recurrence risk for future offspring,” they wrote in a 2011 study.⁶

Hypotonia in chromosomal abnormalities

“Individuals with Down syndrome are at risk for foot alignment problems due to hypotonia and ligamentous laxity. Both characteristics contribute to joint hypermobility,” said senior physical therapist Patricia C. Winders, PT, director of therapies at the Anna and John J. Sie Center for Down Syndrome at Children’s Hospital Colorado in Aurora.

“The ligaments do not hold the bones together tightly for optimal alignment and function. The joints of the foot have excessive flexibility, which causes instability and inefficient mechanics when standing, walking, running, and jumping,” she said. “The child cannot use his strength effectively because the muscles are not aligned for efficient activation. Since his strength does not generate efficient power, he uses more energy during each skill and fatigues more quickly. The consequences of faulty alignment and mechanics range from impaired performance to pain, which can result in limitations in walking. Since walking will be vital for his entire lifetime, it is very important to be proactive in promoting optimal alignment and function, beginning when he learns to walk.”

The goal of physical therapy for an individual with Down syndrome is to achieve maximal physical potential and to build a body that is fit and functional throughout his or her life, Winders said.

“Because of physical problems [hypotonia, ligamentous laxity, and decreased strength], he is prone to develop compensations, which are ways he adapts to make up for the physical problems. Some compensations, if allowed to persist, will eventually result in inefficient and painful movement patterns that will compromise his function as an adult,” she said. “Physical therapy needs to focus on minimizing the compensations that will lead to impairment of motor functioning and on building the posture, strength, and movement patterns that he will need as an adolescent and adult.”

Hypotonia is a common feature of genetic disorders associated with developmental delay. Children with Angelman syndrome have gait ataxia with tremulous limbs, but may not always have hypotonia.⁹ Neonates with Prader-Willi syndrome (PWS), however, have severe hypotonia, evidenced by lethargy and weak or no sucking. As children grow, their gross motor skills are typically delayed; they may sit at 12 months and walk at 24 months, though in some cases walking may be delayed until they are aged 4 or 5 years.

The insatiable appetite that is the hallmark of PWS does not typically present before preschool age, but obesity rates begin to soar during preschool years. Scoliosis; hip dysplasia; respiratory control issues; early risks of osteoporosis; short stature; short, wide feet that require extra care to fit shoes properly; and the stress of a restricted dietary and home environment pose challenges for the families of children with PWS and their healthcare providers.¹⁰

In 2010, Korean researchers found a high prevalence of spinal deformity, limb malalignment, and foot abnormality in PWS, regardless of age or obesity.¹¹ They urged pediatric orthopedic surgeons to evaluate PWS patients annually for these conditions because of their possible concealment by obesity.

Early intervention with occupational and physical therapy and lifelong strength training and aerobic conditioning are critical to addressing overall health status, yet therapists must be aware that many patients with PWS have decreased sensitivity to pain. Any evidence of pain should be promptly addressed, as it may suggest a serious but masked underlying problem, such as a fracture or abnormality.

Missed diagnoses

Cohn suggests children with mild hypotonia and features such as joint hypermobility, pectus excavatum, pes planus, or cardiac abnormalities be carefully evaluated, as he believes patients with connective tissue disorders who have less severe forms of hypotonia remain dramatically underdiagnosed.

Crawford, too, cautions that beyond the parameters of classic presentations of obvious congenital disorders of infancy, hypotonia may be missed.

“Children with classic Duchenne muscular dystrophy are not hypotonic as infants and, as a consequence, that diagnosis may not be considered until later. Some pediatricians might not think of Duchenne dystrophy for a boy who at 15 months of age manifests motor and cognitive delay. But weakness doesn’t show up until later. In many of those cases, we are missing diagnoses,” Crawford said. “Any boy not walking by 16 months should have a CK [creatine kinase] screening. In the case of Duchenne, the result will be five digits even though the child may show no signs of weakness.”

Congenital hypotonia may be seen in tandem with joint hypermobility or ligamentous laxity, but, excluding obvious dysmorphic presentations, hypermobility may not be diagnosed until children are school-aged and present with arthralgia, back pain, abnormal gait, or joint deformity. Knees, elbows, wrists, metacarpophalangeal joints, and ankles are most commonly involved, according to British arthritis researchers, who collected data during a three-year period from pediatric rheumatology and hypermobility clinics.¹² Nearly half of the study participants were described in their clinical history as “clumsy,” and more than a third showed signs of poor coordination in early childhood.

Dutch pediatric physical therapists performing a retrospective study concluded that one-third of children with generalized joint hypermobility presented with severe delays in motor development, though there was no significant association between the number of hypermobile joints and the age of independent walking.¹³

Joint hypermobility, if associated with hypotonia, may be indicative of Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis imperfecta, or other mild variants of these and other musculoskeletal or connective tissue disorders.

Objective assessments

In a 2005 study, physical therapists and occupational therapists found that the specific characteristics of hypotonia included increased flexion, hypermobile joints, round shoulder posture, decreased strength, low activity tolerance, delays in motor development, and poor attention and motivation.¹⁴

Some studies acknowledge that objective measurement of the degree of hypotonia in an individual poses a challenge because, historically, therapists have defined resistance subjectively based on their methods and areas of expertise. Reliable tools, such as the 2011 Segmental Assessment of Trunk Control (SATCo),¹⁵ which measures discrete levels of trunk control in children with motor disabilities, are being developed.

Billi Cusick, PT, MS, C/NDT, COF, of Telluride, CO, uses SATCo in her practice.

“In my experience managing the alignment and movement disorders that are common in children with hypotonia, the functioning alignment of the joints and the somatosensory system are critical areas of concern,” Cusick told LER.

She pointed to a recent systematic review that determined children with benign joint hypermobility syndrome demonstrated significantly poorer proprioception compared to children without the disorder.¹⁶

“Mechanoreceptors in load-bearing joints and the skin on the plantar foot deliver sensory information about limb position and weight bearing when they are stimulated,” Cusick explained. “When joint surfaces are malaligned due to laxity in supporting ligaments, those receptors lose appropriate contact, and, presumably, their messages to the central nervous system are compromised. Lax joints fall to end range when loaded, where it appears that the sensory receptors finally detect the functioning positions. This lack of adequate and timely sensory information is evident in postural deviations, such as a wide-based stance and gait, excessive spinal lordosis, anterior pelvic tilt, knee hyperextension, and foot pronation seen commonly in children with hypotonia. For these children, such postural deviations are normal, and they persist without intervention.”

Intervention with physical therapy and orthoses in childhood is key to preventing or managing pain in adolescence and adulthood commonly associated with joint hypermobilility and hypotonia, even if an underlying diagnosis is not determined. Persistent postural deviations and foot joint laxity typically deform the feet and interfere with independent mobility and endurance as body size and weight increase, Cusick said, citing a 2011 study by Wolf et al that recommended managing the pain with prolonged therapy and general conditioning, with special emphasis on improving strength and proprioception.¹⁷

Cusick not only has decades of experience treating children clinically, she has used her expertise with her own daughter, Ting, who arrived from China aged 12 months and unable to assume an all-fours position, crawl, get into and out of a sitting position, or pull to kneeling or standing.

“Her hips were very weak,” Cusick said. “She had never taken weight on her knees or feet before, so I began to help her put weight through them in small increments and in a variety of postures. I used a jumper seat suspended on a spring and later fitted her in a pair of support shorts to keep her hips from sliding into full abduction when she attempted to assume all fours.”

Ting was soon crawling and pulling to stand, but her feet were profoundly pronated, her foot ligaments were lax, and her wide stance imposed further pronatory forces on her forefeet. Cusick fitted her with heel cups and sturdy flat-soled sneakers, and Ting was soon cruising. At age 5 years, she began gymnastics; at 6, she started soccer; and at 15, Ting became a competitive cheerleader.

“Although Ting’s feet are aligned and competent, because she has a tendency toward joint laxity that is typical of the Chinese population, she continues to wear plantar orthotic inserts that protect her feet and knees from the wear that commonly occurs with excessive pronatory strain,” Cusick said.

In caring for patients with low muscle tone or ligament laxity, Cusick relies on management strategies that improve functioning joint alignment and raise the level of—and improve the quality of—sensory input in daily life. To support these goals, she developed TheraTogs, a live-in orthotic undergarment and strapping system for children with hypotonia and other issues that is designed to deliver enhanced sensory input and improve postural alignment, and can be used in conjunction with orthotic devices that specifically target the foot and ankle. She also selects play activities that build balancing skills, muscle strength, and muscle tone, while maintaining the feet, trunk, and hips in optimum alignment.

As Cohn and Lisi underscored, an inability to define an underlying diagnosis for low muscle tone should not interfere with the ability to manage hypotonia in patients of any age. Symptomatic treatment can and needs to be tailored, they wrote, to create lifelong strategies vital to maintaining strength, reducing pain, and fostering independence.

Christina Hall Nettles is a freelance writer based in Monroeville, AL.

“Enriching and transforming lives through compassionate care and innovation” is our company mission statement. As our focus is on the pediatric special needs population, it’s pretty easy to stay attentive to that mission. Still, we know we can only make a difference in the lives of our patients with the dedication of our employees to our mission and the culture we strive to achieve.  Our culture is the “compass” that guides us.

While every organization has its own way of sharing its philosophy, we at SureStep define ourselves—our culture—in the following ways:

Compassionate care
Unparalleled service
Life changing outcomes
Tremendous team spirit
Unrelenting drive
Remarkable quality
Extreme innovation

Even though a company’s culture can be difficult to capture or define, we believe it can elicit a powerful energy in any organization. At the National Down Syndrome Congress Annual Convention in Indianapolis where I, as the founder of SureStep, was presented with the “Exceptional Meritorious Service Award,” that energy enveloped me. Since 1978, this award has honored and recognized individuals whose service and significant contributions to persons with Down syndrome and their families have had national or international significance.

The experience was humbling to say the least. It caused me to reflect on how what started as a simple idea has grown into a company filled with amazing people who have joined together to change the lives of children worldwide. It comes back to culture. Our team is made up of people with shared values, beliefs, and behaviors. This culture guides individual decisions and actions at the unconscious level. As a result, it has had a profound effect on SureStep’s well-being and success—a success that has allowed us to enrich and transform lives.

I hope that as parents and practitioners, you will join our extended family and experience the culture we have worked so hard to create.

Sincerely,

Bernie Veldman, CO
CEO, SureStep

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SureStep Children’s Footwear

SureStep shoes have been custom designed specifically for children who wear orthoses. Their wider, deeper heel, toe box and instep allow for adequate room and a comfortable fit. The unique tread promotes intrinsic movement and flexibility while a special “cut-line” allows for easy shoe modifications.