SureStep TLSO

The SureStep TLSO has redefined spinal management.  The soft, flexible plastic serves well to create improved upright positioning, while still allowing for slight movement in all planes.

By more evenly distributing pressure circumferentially, this unique device creates stability without rigidity, permitting the core to continue to strengthen.  Custom fabricated from measurements, cast impressions or scans, the SureStep TLSO comes complete with a soft interface which is available in multiple thicknesses for improved comfort and compliance.

The SureStep TLSO is available with either an anterior or posterior opening and can be modified to accommodate G-tubes, baclophen pumps, etc.  The SureStep TLSO is the ideal solution for many wheelchair bound children who have difficulty in maintaining an upright sitting position.

Breathing Modifications

Anterior Window: This simple, yet effective modification facilitates anterior expansion of the ribs and abdominal areas.  The anterior window requires a posterior opening and is a perfect solution for children who tend to breathe lower.

Gill Modification: While this modification may look small, its impact will be great.  The gill modification simply allows the chest to expand laterally and enables  improved breathing within the TLSO. This modification can be added to either an anterior or posterior opening TLSO, and can be added at the time of fabrication, or anytime thereafter.

SureStep Criss Crossers

The first and only device developed to discourage w-sitting, Criss Crossers use a unique audio cue to remind children to change their position.

Available in 4 standard sizes, this innovative design can be worn under most regular clothing and will fit most infants, and small children.  The small sealed battery (similar to a watch battery) and tone generator easily unplug to allow normal cleaning and care.

Criss Crossers are a simple and effective method of discouraging w-sitting without putting any other positional limitations on children.

SureStep De-Rotation Straps

The SureStep De-Rotation Straps offer dynamic control for mild femoral or tibial rotation. The latex-free elastic hook-and-loop strap system easily attaches to shoelaces or to lower extremity orthoses. And its low-profile design easily hides under clothing.

Available in both beige and white, the universal “pediatric” size will fit patients up to 5 ft. tall.  Also available in a universal “adult” size, these devices can be custom-fitted to the patient simply and quickly.

This product gives O&P professionals and physical therapists an easy-to-use solution for flexible internal or external femoral or tibial rotation.

SureStep HEKO Custom

SureStep HEKO is the first and only pediatric hyper extension knee orthosis to incorporate a 4-axis knee hinge, for smooth, anatomically correct flexion and extension.  This exceptional device provides localized control of the knee, preventing hyperextension, valgus and varus, while allowing full flexion and extension.  With adjustable extension stops, the HEKO offers up to 30 degrees of adjustability. Each SureStep HEKO is custom fabricated to measurements using SureStep’s CAD/CAM system, but can be fabricated from a cast impression or scan. The HEKO comes complete with 2 anti-migration/suspension sleeves.

With two hinge sizes, the SureStep HEKO is ideal for patients up to 100 lbs.

SureStep HEKO PreFab

The HEKO PreFab incorporates all of the features of the original HEKO into a prefabricated version.

Integrating the same unique 4-axis knee hinge, this more flexible HEKO PreFab allows even more adjustability.

HEKO PreFab provides the physical therapist or O&P professional a prefabricated option that can create much greater knee stability and improve function dramatically.

Available in 4 prefabricated sizes, in either soft purple or black, HEKO PreFab is ideal for patients up to 50 lbs.

SureStep PullOver

The SureStep PullOver is an amazing tool that not only facilitates improved stability of the foot and ankle complex using a  SureStep SMO, but it is also the only SMO that incorporates a true dorsiflexion assist through the use of a removable proximal strut. This allows for the use of the device as a SureStep SMO, whenever proximal support or dorsiflexion assist is not necessary.

When appropriate, the proximal strut can be attached easily and the PullOver becomes a free-motion, dorsiflexion assist AFO. The PullOver is a perfect solution for many children with mild hemiplegia, or for children with hypotonia that need just a little bit of additional help into dorsiflexion.

SureStep Advanced

The SureStep Advanced AFO is the ideal device for pre-walkers. The SureStep Advanced incorporates the same concept of circumferential compression as the SureStep SMO. Plus, it extends to full AFO height, integrating increased sagittal plane stability to help children find their ideal standing position.

While the SureStep Advanced AFO prevents plantarflexion, simple changes to the proximal strapping configuration allows for varying degrees of dorsiflexion. The SureStep Advanced AFO is very adaptive. When children are ready for ambulation, it can be converted easily to a SureStep SMO. This unique device can be fit with either a full footplate or a modified SureStep footplate.

SureStep Indy 2 Stage

A uniquely designed orthosis developed to help children reach their potential. This exceptional “orthosis within an orthosis” allows for the SureStep SMO to be utilized independent of the AFO. Children can work through a variety of transitional skills without impeding normal muscle function.  When used together, the SureStep SMO locks securely into the AFO transforming this truly dynamic SMO into an AFO that provides triplanar stability without restricting normal usage of intrinsic musculature of the foot.  By doing this, children can continue to develop those intrinsic muscles, muscle strategies and movement patterns necessary for an improved gait pattern.

Product Benefits

Therapists can use the SureStep Indy 2 Stage as a tool for children as they transition through their upright gross motor skill development. The Indy 2 Stage is ideal for children who require the proximal support of an AFO to achieve independent stance, but still benefit from an SMO for proper crawling, pull-to-stand and cruising activities. It is also a great solution for children as they progress to taking independent steps with the AFO. It allows for them to continue to work on improving motor plans with the internal SureStep SMO, thus providing a smooth transition to less bracing.

Battling Fatigue

Children with disorders that cause increased fatigue throughout the day can begin their day in only the SureStep SMO.  This will serve to facilitate increased effort to maintain their functional muscle groups. As they fatigue throughout the day, the external AFO can be added to maintain a stable and functional gait pattern.

SureStep BigShot

A growing child means eventually outgrowing the original SureStep SMO. The BigShot and BigShot Lite are the perfect solutions for older children who still need the stability of SureStep.

The SureStep BigShot incorporates all of the same features of the SureStep SMO with the addition of a soft silicone-like inner boot to ensure comfort. The flexible, plastic outer frame serves to provide the compression and stability that children exceeding 80 lbs. need, but allow all of the normal, necessary flexibility for a smooth, natural gait pattern.

SureStep BigShot Lite

The BigShot also comes in a “lite” version for children between 50 lbs. and 90 lbs. The BigShot Lite offers a thinner inner boot, as well as a thinner, more flexible plastic than its stronger counterpart, the BigShot.

Want to find a clinician in your area? Email or call us with your zip code and we’ll give you a list of facilities nearest you. You can reach us at: info@surestep.net or 877-462-0711

SureStep SMO

The SureStep SMO remains the most advanced method of controlling excessive pronation and providing stability to the hypotonic population.

Through the use of extremely thin, flexible thermoplastic, the SureStep SMO compresses the soft tissue of the foot.  This compressive force stabilizes the foot and ankle complex while still allowing for the development of the intrinsic muscles, muscle strategies and movement patterns necessary to develop a normal, natural gait pattern.

The SureStep SMO has revolutionized orthotic management for children with hypotonia and has become the mandated method of treating this population in many areas of the world.

No Casting Required

Many custom orthoses require casting which is a time-consuming, messy and often a traumatic process for smaller children. SureStep SMOs require only nine easy measurements. It’s simple, it’s quick, and it’s effective.

No Adjustments, No Waiting

SureStep products are available to orthotic and prosthetic facilities nationwide and in 30 countries around the world. Once we receive an SMO order from a certified O&P clinician, the product will be fabricated and shipped within 2 business days. No returning for adjustments, no more waiting for long fabrication times. That means a child can be wearing their SureStep SMOs within a week.

Want to find a clinician in your area? Email or call us with your zip code and we’ll give you a list of facilities nearest you. You can reach us at:
info@surestep.net or 877-462-0711

Benefits of SureStep SMOs

• Improved efficiency
• Increased stability
• Enhanced alignment

About Our Trimlines

The SureStep SMOs are carefully marked for “right” and “left” and although they may look different than other orthoses, our unique patented trimlines are an integral part of the system that enhances a child’s ability to run, jump and play naturally.

The “lateral”, or outside trimline of the SureStep SMO extends further than the “medial”, or inside trimline.  This is in sharp contrast to most traditional orthoses.

Enhanced Alignment

Excessive pronation puts undue stress on the knees and hips by changing the alignment of the lower extremities, taxing the muscles of the legs greatly.

This often results in complaints of fatigue or “tired legs”. SureStep helps to stabilize the foot and ankle relieving stress and allowing  the muscles to work more efficiently.

Live outside the U.S.?

SureStep products are available in many areas of the world. Our list is growing daily. To learn more about how you can obtain our products internationally, please email us your request at info@surestep.net.

Photo courtesy of Monica Foster Jacobs.

Two recent literature reviews underscore the lack of high-level evidence to support the various treatments available for idiopathic toe walking. But new research is starting to fill that void, and is also engendering new theories about factors that may contribute to the condition.

By Larry Hand

Hard evidence continues to elude researchers investigating the origins of and treatments for idiopathic toe walking (ITW) in children, but more are now focusing on identifying factors that may contribute to the condition—which many believe may not be idiopathic at all.

Looking into what children with ITW feel when they walk, for example, may point to an underlying cause. And studies that can compare children with ITW and children whose toe walking is diagnosis-specific may identify some commonalities.

At the American Orthotic & Prosthetic Association National Assembly in September, Mark Geil, PhD, director of the Center for Pediatric Locomotion Sciences at Georgia State University (GSU) in Atlanta, presented results of a trial he conducted involving 15 children with ITW and 15 typically developing children.

The children, who had a mean age of 6.7 years, a mean height of 1.2 meters, and a mean body mass index of 27.8 kg/m², walked barefoot at a self-selected speed on three different surfaces: a rounded gravel surface, a standard pile carpet, and the smooth gait lab floor. Geil and GSU colleague Daniel Fanchiang, PhD, had the children walk 10 times on each surface and measured heel heights relative to each child. A heel marker height above baseline before 32% of the gait cycle may indicate early heel rise or toe contact.¹

The ITW children walked with significantly lower heel height (indicating less toe walking) when walking on the gravel surface than on other surfaces. The analysis also showed identical gait patterns for the ITW and typically developing children when walking on the gravel surface, probably because the gravel provided more sensory input and stability, which may be a clue that could help identify a cause of ITW, according to Geil.

In the gait lab at Georgia State University, reflective markers are attached to anatomical landmarks on the foot and ankle to monitor ankle angle at initial contact and the presence of early heel rise. (Photo by Steve Thackston, courtesy of Georgia State University.)

“The result we found wasn’t expected because the experiment we were doing was not designed to test the effectiveness of a terrain like that in this population,” he told LER. “But it was one of those happy, unexpected outcomes. What we really need to do is to design a study that, from the beginning, is focused on investigating the potential for walking barefoot on different terrains and effectiveness in controlling toe walking.”

He and his team are also considering expanding their study population base to include children with autism spectrum disorder who toe walk.

“Kids with autism have been excluded from our studies so far because we’ve been looking for individuals with no known diagnosis, something truly idiopathic. We recognize the sensory component there [in the autism population], too, and since we have what we think is an effective intervention, we could see how it works in that population, as well,” Geil said.

Vibration and vibration perception

Geil is seeking funding to expand his research into the sensory pathways involved in toe walking, with or without an underlying diagnosis.

“One of the focal areas we were really excited about is the potential for whole-body vibration as a different intervention that would address that sensory link to the need for toe walking in these kids,” he said.

But, in an article epublished in September by the Journal of Child Neurology,¹ Geil and colleagues described unexpected results when they tested vibration as a therapy in children with ITW. They were seeking to expand on work done by Cylie M. Williams, PhD, and colleagues in Australia,² who found that children who present with toe walking are more sensitive to vibrations than their typically developing peers.

“Children may be changing gait pattern to adjust to sensations they perceive during walking,” Geil said. “Our thought was, if we could flood that sensory pathway in these kids with standing for a minute on a whole-body vibration machine, it might affect their need to toe walk.”

In the study, 15 children with ITW and 15 typically developing children walked barefoot at self-selected speeds over a four-meter walkway before and after standing on a whole-body vibration machine for 60 seconds at 30 Hz, the minimum vibration frequency of the machine.

“We worked in a lot of protection for the kids in our protocol and the kids had plenty of opportunities to stop, but most of them actually really enjoyed standing on the machine barefoot,” Geil told LER.

The researchers analyzed velocity, cadence, step length, and step width, as well as the timing of heel rise, which previous research has shown to be an indicator of potential gait abnormalities.³

They found no significant differences between the two groups in gait patterns, and both groups experienced similar significant changes in vibration perception after the vibration intervention.

“The outcomes weren’t what we had hoped, so we’re looking to do a new round of a study like that,” Geil said. “The machine that we used was very limited. It was all we could afford. If we can figure out a proper dose, which involves both time on the machine and the frequency and amplitude at which the machine vibrates, then it’s three variables to sort out. It still has potential. It just needs a lot of tweaking at this point.”

Geil’s results appear to contrast with what Williams, now adjunct research fellow in the Department of Physiotherapy at Monash University in Victoria, Australia, and colleagues found.² The Australians identified for the first time that otherwise healthy children with ITW exhibited motor and sensory deficits in a series of tests when compared with children who do not toe walk.

“There is a great deal of research being undertaken to better understand the challenges of sensory processing,” Williams told LER. “I think what has emerged from my body of research was that clinicians need to think more about the reason that the child may be toe walking, as current treatments have been showing limited success in long-term gait change. To better tailor treatment, sensory processing abilities should also be considered as part of the holistic approach to treating the child.”

Evidence for treatment

There is still much to be learned about ITW, Williams said.

“Given the family history, there may be a potential genetic link,” she said. “There may also be the possibility that ITW, in fact, may be a very mild form of cerebral palsy that is variable in presentation, or it may be associated with [having a] very high-functioning autistic spectrum disorder that is not able to be picked up on any current assessment. We also do not know the long-term impact of toe walking. While there are many family members who toe walk or toe walked as children who have an ITW gait, we do not know what is happening with their gait now.”

Williams and her colleagues assessed current treatments for ITW in a review published in the May/June 2014 issue of the Journal of the American Podiatric Medical Association (JAPMA).⁴ They gauged 21 published studies against levels of evidence. Briefly, they found some support in the literature for surgical interventions, serial casting, and botulinum toxin type A (BTX).

In the first of two case studies included along with the literature review in the JAPMA article, researchers placed a small arch filler in the footwear of a 7-year-old boy to increase the sensation of full foot contact. They reported a “notable” decrease in toe walking at three and six months when the child wore the footwear, with similar toe walking levels in and out of footwear. At 36 months of follow-up, the researchers reported minimal toe walking in or out of the footwear. They hypothesized that the improvement was related to an increase in plantar contact area created by the arch fillers.

In the second case study, a 7-year-old girl underwent weight-bearing serial casting and developed an allergy to the casting materials. She then received night splints and underwent an exercise regimen of heel raises on a small board. She did heel-only walking exercises with the foot dorsiflexed, which resulted in minimal improvement. Researchers injected BTX into the medial and lateral heads of the gastrocnemius muscle and casted her for seven days using a different material.

Then, researchers fitted a full-length carbon-fiber custom orthotic device with rearfoot control and instructed her to wear it as much as possible. Three years after she initially presented, she was not toe walking and she was wearing normal athletic shoes. Later, however, they observed that, though overall toe walking had been reduced, residual equinus persisted.

“There is still much to be understood about idiopathic toe walking and its relationship to sensory processing difficulties,” Williams said. “I believe this condition [ITW] is complex and the group of children presenting with this gait type is not homogenous. This makes it extremely difficult to tailor treatment and to understand the origin of the gait. There is currently no literature supporting or disproving that all children with ITW have sensory processing difficulties, only enough evidence to prompt clinicians that sensory processing abilities should be considered if providing treatment.”

Better with BTX?

Although BTX use in the child’s case described above was associated with an apparent benefit, the effectiveness of BTX in other studies has been mixed.

Pähr Engström, MD, and colleagues at the Karolinska Institute in Stockholm, Sweden, found in a randomized trial⁵ published in 2013 that adding BTX injections prior to cast treatment for ITW is not associated with better outcomes than cast-only treatment. In a trial published in 2010,⁶ the same group found that a single BTX injection in combination with an exercise program may improve walking pattern in children with ITW, but only occasionally led to cessation of toe walking.

Their later study included children evaluated at their clinic for ITW between 2005 and 2010. They randomized 26 children to receive casting only for four weeks and 21 children to receive casting plus BTX (four injections in each calf, 12 units/kg body weight) one to two weeks prior to casting for four weeks. The researchers conducted gait analysis before treatment and at three and 12 months after cast removal, as the children walked barefoot at self-selected speeds. Parents’ perceptions were part of the study’s primary endpoints, Engström said.

“When you examine a ITW child in your clinic and ask them to show how they walk, they never walk as they normally do, as the child is aware his or her walk is being examined,” he said. “Normally our walking pattern is handled on spinal level and we ‘do not use our brain’ to walk. But when you concentrate or think about your walk, then the brain overrules your normal walking pattern.”

The researchers found no difference between the groups for any gait parameter. Parents rating their children’s performances during barefoot walking before and after treatment also found no difference between groups. However, both groups showed “a marked improvement in all of these parameters after their respective treatments, at both three and twelve months.”

“Our study did not show any additional benefit with BTX compared to only casts,” Engström told LER. “If BTX is to be used, someone needs to show in a prospective randomized study that BTX has any role in the treatment of ITW. Will repeated injections of BTX have a better effect? We have no knowledge about that at present and, therefore, we believe BTX should not be used in the treatment of ITW.”

Both Engström studies were cited in a September 2014 systematic review of ITW literature published in the Journal of Rehabilitative Medicine.⁷ In that review, Annette A.A. van Kuijik, MD, and colleagues at the Rehabilitation Centre Tolbrug in the Netherlands concluded that the sustainability of beneficial effects after physical therapy or casting appears to be short, although preliminary evidence exists for beneficial effects of serial casting and surgery on passive ankle dorsiflexion. Sustainable effects lasting more than a year occur only after surgery, they wrote.

Limitations of the literature

Much of the research into ITW treatments or causes is lacking basic information, however, according to Louis J. DeCaro, DPM, a pediatric specialist in Massachusetts and president of the American College of Foot and Ankle Pediatrics, who treats at least five toe walking patients a week.

“I don’t really believe that there is a good standard of measuring dorsiflexion accurately in subtalar neutral, so I think some of the results are skewed,” he said. “I think a lot of the literature is flawed because the most critical part in what I believe in treating toe walking is being able to assess what is causing it. I don’t really believe there really is idiopathic toe walking. There’s a reason for it all, including things such as vision, autism spectrum, or undiagnosed equinus.”

Orthotic management of ITW has not been studied very well, DeCaro said. In the JAPMA review, only three of the papers analyzed discussed orthotic management, and the highest level of evidence was a case series.⁸

“You have many people making many different kinds of orthotics, but I see in practice as well as in research articles that there’s a big deficit in the type of orthotic that a child gets. It’s not controlling enough. If you don’t control whatever position you cast or ‘Botox’ in, you’re going to get failing results,” he said. “We need studies that have consistency in measuring dorsiflexion, consistency in casting techniques, consistency in orthotic treatments following any sort of successful therapy. As well, there’s no talk of referring to optometrists anywhere. It’s a huge factor in all of this. I’ve seen kids get glasses and immediately they walk on their heels.”

And when vision isn’t an issue?

“Regardless of if I cast, do surgery, or use Botox, I make sure I put a kid in a proper orthotic, no matter what age, to inevitably control the toe walking,” DeCaro said.

But research momentum may be building in a positive way.

“Longitudinal studies are very difficult to conduct due to both time and funding, especially when it is a rather benign condition,” Williams of Australia told LER. “I am really glad, though, that through the research we have done, there have been a number of research groups being established all over the world looking to better understand why some kids walk on their toes.”

Past research has focused on treating the symptoms of ITW because the cause has been considered unknown, Georgia State’s Geil added.

“That’s part of the reason why we’ve shifted our focus to investigations that might be able to get at the cause,” he said. “Then I think we can do some real good.”

Larry Hand is a writer in Massachusetts.

Photo courtesy of Fillauer

Syme, transtibial gaits are similar

By Hank Black

The relative functional benefits of transtibial and Syme amputations in adults have been discussed in the medical literature, but few studies have addressed similar issues in pediatric patients. A sizeable recent study found statistically significant kinematic and kinetic differences between the two amputation levels in children and adolescents, but the subtle nature of those differences suggests that decisions about amputation level should be decided on an individual basis.

“We found only subtle differences in gait data between Syme and transtibial-level patients, mostly due to underlying etiology, but in an earlier study we found that this does not result in increased oxygen consumption and heart rate,” said Donald Cummings, CP, a study coauthor and director of the Orthotics and Prosthetics Department at Texas Scottish Rite Hospital for Children in Dallas.

The study, which was published in the October issue of The Journal of Bone and Joint Surgery, used 3D gait analysis to assess 64 children and adolescents, aged 4.7 to 19.2 years, who had undergone unilateral below-knee amputations, with 41 patients in the Syme cohort (which also included three Boyd amputations). Patients wore their current prostheses and walked at a self-selected speed.

A total of 12 types of prosthetic feet were involved, with each categorized as permitting a high, medium, or low activity level as defined by Medicare for prosthesis prescription. Six patients wore high-performance dynamic response feet, and five of those were transtibial amputees; 59% of the study population wore medium-performance feet, and 31% wore low-performance feet. The PODCI (pediatric outcomes data collection instrument) was completed by the accompanying parents.

Total ankle excursion and peak power of the prosthetic ankle were significantly greater in the transtibial patients than the Syme patients. Patients in the Syme group walked with more external hip rotation during stance phase than those in the transtibial group, and also had greater peak coronal-plane hip abductor power. However, the authors noted that the kinematic differences between groups, despite being statistically significant, were small enough that they were unlikely to be clinically relevant. Rather than amputation level, they hypothesized, the kinematic differences at the hip probably were related to the fact that more than half the Syme patients had fibular hemimelia, which is associated with femoral external rotation, and the differences at the ankle probably resulted from the foot types used. PODCI measures did not differ significantly between groups.

Prosthetic ankle range of motion was significantly greater in the patients with high-performance, dynamic response feet than in those with medium- and low-performance feet. Lower-tech feet were actually associated with greater happiness or satisfaction scores than more advanced feet, possibly because the low-tech versions were more likely to be worn by younger children.

“A major challenge facing the clinical team and the parents is coming up with criteria about when we should be fitting higher-end feet, which require more space between the ground and the distal tibia,” Cummings said. “Parents want their kids to develop as normally as possible in their peer group, not necessarily expecting the child to become a top athlete, but at least to be on par with peers in active play and sports. With T-ball, soccer, and other active sports and play that involve running, the controversy starts within a year or two of the first fitting, if not at the start.”

Of course, the space available between the end of the residual limb and the floor, patient size, and available pediatric components often determine prosthesis prescription. Growth modulation or revision surgery can provide patients with a shorter limb and greater choices, but surgical shortening of the residual limb, which allows more choices of high-tech components, remains controversial, Cummings said. A Syme amputation, in addition to having structural advantages in growing children, also offers the potential ability to walk at least very short distances without a prosthesis, which 18 of the study’s Syme patients said they could do.

In a commentary accompanying the online version of the article, orthopedic surgeon Michael Aiona, MD, chief of staff at Shriners Hospital for Children in Portland, OR, suggested the Dallas group’s findings could be used to outline a management program for the child with a limb deficiency. Specifically, he wrote, the data support the current gold standard of an amputation that maximizes limb length; they also suggest that low-cost SACH (solid ankle cushion heel) feet can be worn by young children without compromising their gait, function, or satisfaction, and that more expensive, high-performance feet can wait until a child has reached adolescence.

Hank Black is a medical writer in Birmingham, AL.

Sources:

Jeans KA, Karol LA, Cummings D, Singhal K. Comparison of gait after Syme and transtibial amputation in children. Factors that may play a role in function. J Bone Joint Surg Am 2014;96(19):1641-1647.

Aiona M. See you in the Paralympics in 2022. Commentary on an article by Kelly A. Jeans, MS, et al.: “Comparison of gait after Syme and transtibial amputation in children. Factors that may play a role in function.” J Bone Joint Surg Am 2014;96(19): e173(1-2).

Location of shortening is key factor

By Larry Hand

Contrary to popular perceptions, children with limb length discrepancy (LLD) may each use multiple compensatory strategies when they are walking, depending on where their discrepancy is, and those gait patterns may present a dilemma for practitioners.

In a recent study published in the Journal of Pediatric Orthopedics, Michael Aiona, MD, chief of staff at the Shriners Hospital for Children in Portland, OR, and colleagues found that children can compensate for LLD with multiple gait patterns, depending on which aspect of the limb is shortened.

The researchers used a motion capture system to analyze 45 children (24 girls) with an average age of 12.8 years and an LLD of more than 2 cm and 20 typically developing controls as they walked at a self-selected speed along a two-meter walkway.

Of the 45 children with LLD, 18 used multiple strategies to compensate for the shortening.

These included:

• Pelvic obliquity with the short side lower;
• Knee flexion in the longer leg during stance;
• Ankle plantar flexion in the shorter leg through the gait cycle; and
• Vaulting.

“We looked at a bunch of different kids with a bunch of different causes for differences,” Aiona told LER. “Basically, in some areas they were putting a bit more pressure on certain joints and doing more work in certain areas. They also took different strategies.”

Of the 45 children, four had a diagnosis of Legg-Calve-Perthes, nine had a diagnosis of developmental hip dysplasia, six had growth plate damage due to infection or trauma, five had shortening of the femur, seven had shortening of the tibia, five had syndromes creating limb shortening, and nine had other diagnoses.

Children with a length discrepancy in the femur used compensations at the ankle, which led to more work at the ankle joint on the short limb compared with those in the control group.

Children with tibia shortening demonstrated pelvic obliquity (when the pelvis is lower on the short side throughout the gait cycle), and a few also compensated with knee flexion, which led to more work at the hip on the short limb compared with normal parameters. These findings suggest that underlying factors related to tibial shortening, whether specific to muscles or joints, limit the ankle’s ability to perform increased work, the authors wrote.

Because many clinicians believe that children with LLD who are otherwise healthy develop compensatory strategies specifically to maintain a level pelvis during gait, Aiona and colleagues were surprised to find that 24 children in their study had persistent pelvic obliquity.

“A lot of the kids actually walked with their pelvis uneven, which is a little bit surprising to us,” Aiona said. “There’s some concern whether that has any effects on long-term hip joint development. You could argue that, if hip joint development is affected, then you need to more aggressively manage kids earlier on to get things a bit more equal.”

A lift or surgery may be in order, he said, depending in part on the extent of the discrepancy.

“You could use a lift to equalize, but it’s hard for the kid to play, and you can’t modify every shoe,” Aiona said. “It’s the practicality and the cosmetic things that are involved in it that make it tricky.”

Options for intervention also may be less clear in children who use multiple compensation strategies, which also appears to be associated with the magnitude of the discrepancy. The Shriners study found that all children with shortening of more than 7 cm used multiple compensation strategies. By comparison, only three of 11 children with discrepancies between 4 cm and 7 cm used multiple gait strategies.

“For me, the take-home message is, when the discrepancy becomes greater, that’s when you have to worry more about different ways of how they walk to compensate,” Aiona said.

Mark Geil, PhD, director of the Center for Pediatric Locomotion Sciences at Georgia State University in Atlanta, said the study definitely adds to the evidence base.

“I’ve often taught in my classes that it’s very difficult to predict which strategy an individual will employ given a leg length discrepancy, and in my own lab I’ve found that, when introduced with an acute, artificial leg length discrepancy [eg, shortening a prosthetic pylon], some subjects will immediately adopt multiple strategies before paring down to one over just a few dozen steps,” Geil told LER.

The Shriners findings may provide a new perspective on those observations, Geil said.

“I honestly had not thought of the possibility this paper raises, that LLD etiology might predict gait strategy,” Geil said. “But it makes sense.”

Larry Hand is a writer in Massachusetts.

Sources:

Aiona M, Patrick K, Emara K, et al. Gait patterns in children with limb length discrepancy. J Pediatr Orthop 2014 Jul 29. [Epub ahead of print]

Geil M, Coulter C. Analysis of locomotor adaptations in young children with limb loss in an early prosthetic knee prescription protocol. Prosthet Orthot Int 2014;38(1):54-61.

Experts push for better sprain rehab

By P.K. Daniel

Primary ankle sprains often occur before adulthood, and a recent literature review from Australia suggests that some pediatric populations exhibit a high rate of ankle injury recurrence and chronic ankle instability (CAI). This finding, which echoes what has long been observed in adults, has raised concerns that ankle sprains in children are not being managed properly.

A systematic review of chronic ankle instability in children by University of Sydney researchers found that as many as 71% of children with a history of ankle sprain had perceived instability and as many as 47% had mechanical instability.

“We still don’t know exactly what percentage of children will go on to develop CAI, as no prospective study has been done. However, it is an outcome that is under-recognized,” said study coauthor Claire Hiller, PhD, a postdoctoral fellow in the university’s Arthritis and Musculo­skeletal Research Group.

More than half (53.5%) of all ankle sprains occur in individuals aged between 10 and 24 years, according to a 2010 study. That study also found that teenagers and young adults have the highest rates of ankle sprain, with a peak incidence of 7.2 per 1000 person-years for those aged 15 to 19 years.

“As currently the only known predictor of a sprain is a previous sprain, having an ankle sprain early in life gives you a much greater opportunity to have another one and develop long-term problems,” Hiller said.

And yet, adults have been the focus of chronic ankle instability research.

The Australian review, which was published in March by the Journal of Foot and Ankle Research, analyzed nine studies on CAI focused on children and included ages up to 18 years. Many of the studies looked at specific youth populations, ie, dancers, soccer players, and those who had experienced “severe ankle trauma.” The review found that the prevalence of CAI was equal to or higher than that of adult populations. However, the overall shortage of studies on CAI in children led the review article authors to determine that more research was required.

Prevalence of perceived instability ranged from 31% in children with severe ankle injuries to 71% of children who were dancers. Nearly half (47%) of the dancers had mechanical instability.

“If our younger populations are exhibiting such high rates of ankle injury recurrence, it raises concerns that ankle sprains in this age group are not being managed properly,” said Phillip Gribble, PhD, ATC, associate professor in the Division of Athletic Training at the University of Kentucky in Lexington. “This is likely [because of a] lack of recognition for the need for proper immediate care and evaluation, as well as a lack of thorough rehabilitation before returning back to activity.”

Tricia Turner, PhD, ATC, an associate professor in the Department of Kinesiology at the University of North Carolina at Charlotte, said part of the problem is that children are being seen by practitioners who may not be aware that chronic ankle instability is a potential issue in children.

“Ankle sprains in children need to be treated and taken more seriously,” Turner said.

Balance training exercises, taping, and prophylactic bracing can minimize the risk of future instability, but access to healthcare professionals who can effectively implement these practices is limited in the adolescent population.

“And typically nonexistent for the prepubescent populations participating in sports,” Gribble said. “The treatment decision then rests with the parents. They are likely not to seek formal management and rehabilitation for their child’s ankle sprain, leaving the injured ankle at a higher level of susceptibility for reinjury.”

Fereshteh Pourkazemi, PhD, a physiotherapy lecturer in the School of Science and Health at the University of Western Sydney and coauthor of the Australian review article, said less developed patterns of motor and postural control, combined with higher levels of activity, may increase the risk of ankle sprains and development of chronic ankle instability in children.

“We should really be cautious, go slow, and be very, very conservative with the treatment,” said Thomas W. Kaminski, PhD, ATC, director of athletic training education and professor in the Department of Kinesiology and Applied Physiology at the University of Delaware in Newark.

Patrick McKeon, PhD, ATC, an ankle researcher with Ithaca College’s School of Health Sciences and Human Performance in Ithaca, NY, echoed that approach, also indicating that the return-to-play timeline probably needs to be longer for children than adults.

“Joint deficit influences coordination, and their coordination is in the development stage, as opposed to the refinement stage,” McKeon said. “We need to allow them more time to heal.”

Sources:

Mandarakas M, Pourkazemi F, Sman A, et al. Systematic review of chronic ankle instability in children. J Foot Ankle Res 2014;7(1):21.

Waterman BR, Owens BD, Davey S, et al. The epidemiology of ankle sprains in the United States. J Bone Joint Surg Am 2010;92(13):2279-2284.

While the patient is always the practitionerʼs first priority, establishing a convivial relationship with a childʼs parents can mean the difference between victory and failure when it comes to diagnosing, treating, and rehabilitating lower extremity problems in pediatric patients.

By Shalmali Pal

These days, there as many labels for parenting styles as there are names for mother and father: There’s kangaroo mom versus stroller mom; strict tiger parents versus permissive parents; helicopter (aka anxious) dad versus secure dad.

But when a child has special needs that include lower extremity impairments, parenting reaches another level, as mom, dad, and the rest of the family adopt and adapt to a unique way of life. In turn, the lower extremity professionals who work with these pediatric patients, and their parents, have to be comfortable with many different labels: healthcare practitioner, counselor, cheerleader, teacher, and diplomat, to name a few.

While the patient is always the first priority, establishing a convivial relationship with the parents (or even one parent) can make the difference between victory and failure when it comes to diagnosing, treating, and rehabilitating lower extremity problems.

“The success of the patient really is tied to the interaction with the parents,” said Tyler Sexton, MD, a pediatrician who works with kids with disabilities including cerebral palsy (CP). “Sometimes, parents are the only advocates for these children. As practitioners, sometimes we only see these kids in fifteen- to thirty-minute segments, so we really have to rely on the parents to find out how things are going with the child, what the potential problems are. In that sense, we are treating the whole family.”

And, as in any family, it’s best if everyone gets along. Sexton and other experts shared their advice for creating successful working relationships with parents and what to do when those relationships prove challenging.

Photo courtesy of SureStep

First impressions

The introductory meeting between the practitioner, the patient, and the parents is crucial, practitioners agree, as it will lay the foundation for future interaction.

Chad Brown, a BOC (Board of Certification/Accreditation) pedorthist at Metro Orthotics in St. Louis, MO, likes to jump in with both feet.

“It’s very important to me to learn as much as possible about the family and the environment that the patient is living in, so I ask a fair amount of questions,” he said. “What kinds of activities does the patient participate in? What kind of progress would [the parents] like to see? What have they tried in the past [with regard to the lower extremity problems] and what hasn’t worked out? The more information I have about the entire situation puts me in a better position to meet, or hopefully exceed, their expectations.”

The first meeting is also a time for Brown to get a sense of the family dynamics: Does one parent tend to dominate the conversation? Does one seem more protective of the child? Is the child able to communicate on his or her own at any level?

Sexton takes a slightly different approach during that first meeting: He does a quick intake of the child based on pediatric behavioral classifications: The easy-going child, the slow-to-warm child, and the difficult child. From there, he begins to paint a picture of the parents.

“The easy child will adapt pretty easily. The child is willing to try anything, whether it’s a new type of therapy or a new device, and the parents have that same attitude,” explained Sexton, who is president and chief executive officer of Caribbean Hyperbaric Medicine in Zephyrhills, FL.

“A slow-to-warm child will take a bit longer to get started. There will be some wariness at first for the child and the parents, but they will eventually warm up, and work with you,” he continued. “Now the difficult child, no matter what you do, it’s hard to get them motivated, it’s hard to show them rewards. Those parents are going to have a different mindset; they may need additional encouragement along with the child. But they may also have a system of tips and tricks to get the child to comply, so you’ll want to learn those as soon as possible.”

Lisa Swenson, PT, C/NDT, opts for an unhurried approach. She is a senior instructor at the ENRICH/JFK Partners program for developmental disabilities at the University of Colorado School of Medicine in Denver, and is the primary physical therapist at The Rise School of Denver, an inclusive preschool for children with and without special needs.

“You can’t really obtain all this information at once,” she said. “We work to develop a relationship with the family.”

Swenson consults with families in their homes as part of the ENRICH/JFK Partners program and also at the school.

At the home, Swenson does an environmental intake: Are there siblings or extended family present? What kind of support system do the parents have? How busy is their typical day?

Phto courtesy of Cascade Dafo.

“I can get a real sense of what home life is like,” she explained. “I have a questionnaire that I use to find out more about the daily routine. I might ask questions like ‘How is it getting your child into the car seat? How is it helping her get dressed?’ I’m trying to find out what’s working well at home in terms of the daily routine.”

At the school, the child undergoes an intake evaluation by the occupational or speech therapist. The child’s teacher, along with Swenson and the other therapists, then develop a unified plan for working with the child, whether it’s on behavioral issues or mobility problems.

While parent-teacher conferences are held semiregularly, Swenson’s interaction with parents often happens more casually, such as an introduction in the hallway or at a school event, she said.

Whatever approach is taken, a key point to remember during this inquiry phase is that “whether the patient has a more serious condition related to Down syndrome or CP, or a more common ailment like Severʼs disease or heel pain, the dominant category that parents fall into is that they want the best outcome possible for their child,” Brown said.

Motivation, communication, compliance

Having open lines of communication with parents is vital, whether that’s because they are the child’s main spokesperson or because they are paying the bills. But establishing those lines will require a practitioner to get a handle on the parents’ motivation, as that can play a large part in the treatment decision-making and compliance.

There are two extremes when it comes to parents: There are the highly motivated ones who are determined to see their children succeed as soon as possible. They can sometimes be aggressive communicators, with unrealistic expectations and demands for nearly immediate results with a treatment course.

At the other end are the permissive, less-driven parents who won’t follow through with any home-based PT exercises, or don’t want to struggle with getting the child to wear a device daily. These parents can sometimes be defensive about their lack of motivation, although they may not necessarily be any more realistic about treatment progress than their aggressive counterparts.

Photo courtesy of Allard USA.

The majority of parents fall in the middle of the spectrum, so developing tools to deal with parents at either extreme will give practitioners an edge.

The experts agreed that sometimes an enthusiastic-to-the-point-of-pushy parent can be easier to manage than a resistant one, simply because the former has a vested interest in seeing the child improve.

“With a motivated parent who has very high expectations, I break those expectations down into smaller, more attainable goals,” Sexton explained. “That’s the key: Coming up with a realistic treatment plan and then getting everyone to focus on implementing that plan, step by step.”

For example, therapy for a child with autism spectrum disorder (ASD) and severe hypotonia may never put that child in a position to join other children on the playground. Sexton will set more realistic goals, such as helping the child gain confidence so he has fewer outbursts or improving her strength so she can help dress herself.

“My motto is: under-promise and over-deliver,” Sexton said. “Set those small, realistic goals that you know the child can achieve. And, when the aggressive parent sees the child succeeding, it’ll make them happy, and they’ll see the benefit of moving forward with treatment in a planned, methodical way.”

Ryan Hines, CO, from Park Nicollet Health Partners in St. Louis Park, MN, manages expectations by making sure that parents understand the logic behind a treat­ment course.

“Let’s say you fit a pair of [ankle foot orthoses (AFOs)] for toe walking,” he said. “The more motivated parents might want to come back in six months to see if there’s been any improvement, but that may be too soon to see changes, depending on the age of the child. Then you’ll have parents who don’t want to have to come back for another year, and that may be too long. So we make it a point to discuss the logical progression of when and why the child needs to be seen again, because kids will outgrow a device.”

Brown said he likes to give parents two different options, covering the pros and cons of both, make his recommendation, and then let the parents decide because that gives them a sense of ownership in the treatment process.

For example, for a child with heel pain, “one course of action would be trying an over-the-counter, mass-produced insole. I’d explain that the price point is lower on that, and it’s something we can try for a couple of weeks without a huge financial commitment,” Brown said. “If that doesn’t work out, then we move on to the option of fabricating a device, and again, go through the same decision-making process, with the parents having the final say. Making them partially responsible for the outcomes can help with compliance.”

Sometimes noncompliant parents are really just frustrated parents, Swenson pointed out. If getting a child into her AFOs every morning to get to school on time is an ordeal, the parent may be more likely to give up on the devices.

“We’ll say to the parent ‘There’s no point in all of you struggling in the morning under pressure. Bring the AFOs with you when you drop the child at school, and we’ll work on it there,’” she said. “We’ll make donning and doffing the AFOs part of the daily routine at school, so then the family can start implementing that routine at home. It becomes very matter of fact. It also takes away that sense that the parent is forcing the child to do something.”

Hines emphasized that showing parents the logical progression of treatment can act as an incentive toward compliance. For instance, when treating hypotonia, he’ll tell parents that they may be looking at two sets of supramalleolar orthoses (SMOs) before the child stops growing, based on a child’s age at the time of the initial prescription and his or her projected growth rate.

“We’ll let them know that we’ll examine the feet and legs at every visit and that, if we see progressive improvement with the SMOs, there’s the possibility that a new device will be necessary—maybe a set of UCBL [University of California Biomechanics Laboratory] foot orthoses—which are less expensive and less of a process to put on,” he explained.

It takes a village

The concept of a healthcare team has gained popularity in modern medicine, and it’s especially important when consulting with pediatric patients and their families.

For Swenson, the team approach is already built in at her school, but even during home visits, she emphasizes that she, the family, and other healthcare providers need to work as one unit.

“My job is to be the specialist who gives my knowledge to the family. I want to use my experience to help parents make decisions…. You’ve got to have them see that they are part of the team, and the team includes any other therapists they see, physicians they consult with, and the orthotists that I may refer them to. I make it a point to keep open lines of communications with everyone.”

So when a father stops Swenson in the hallway and asks her to check if his child is outgrowing an AFO, Swenson can relay that information to the orthotist.

Sexton also tells parents that, “It’s not just about coming to see me. You must see the PT, you must see the speech therapist, you must maintain visits with the orthotist or prosthetist if that’s appropriate. There’s no quick fix. We are all creating a huge road map that everyone needs to contribute to.”

To that end, parents should be encouraged to speak up about any concerns they have, especially if those concerns have to do with a device that the child is using. Hines reassures cost-conscious parents that, “we don’t charge for adjustment. We bill for the delivery of the device, and that includes the follow-up. So no news to us means the device is working out fine. Parents should always feel free to let us know that there may be a problem.”

Hines also pointed out that an interpreter is part of the healthcare team for non-English speaking parents (although the child may be quite comfortable in the language).

“The language barrier is an issue that we deal with a lot. You still have to communicate and answer all the questions, but it’s more challenging because the process is much slower,” he said.

Ultimately, practitioners need to keep in mind two major points when navigating the patient-parent-provider pathway, Sexton stated.

“First, all parents want the best quality of life for their child; that’s universal,” he said. “Second, with a special needs child, when parents get involved, the child is going to do better. This is a long-term commitment, and I think it’s important to everyone, including us as healthcare providers, to make that commitment.”

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

By Shalmali Pal

Knee pain is common in children with CP, but given the many health challenges facing this patient population, knee symptoms may not be given high priority. Proper diagnosis and treatment of knee pain, however, can be key to maximizing a childʼs mobility and quality of life.

Knee pain affects one in five ambulatory children with cerebral palsy (CP).¹ But these patients have a range of health concerns—behavioral, cognitive, psychological, emotional, and biomechanical—and all require different levels of management. That’s why practitioners may not be aware of knee pain in children with CP unless they go looking for it.

“Clinicians are usually focusing on the bigger issues, so knee pain kind of falls down their checklist,” explained Frances Gavelli, PhD, lab chief of the National Institute of Biomedical Imaging and Bioengineering at the National Institutes of Health (NIH) in Bethesda, MD. “The child may not even mention it because what’s a little knee pain when they have more complex issues to deal with?”

Katharine Alter, MD, medical director of the NIH’s Gait Lab, agreed that if clinicians don’t ask about knee pain in this population, they won’t hear about it.

“A lot of practitioners don’t ask children with CP specifically about musculoskeletal pain, especially if they are under the age of twelve,” Alter said. “Of course, part of the problem is communication—how accurately can a three-year-old answer the question, ‘Are you having knee pain?’”

And yet, a recent study from the Children’s Orthopaedic Center in Los Angeles found a 21% prevalence of knee pain in ambulatory kids with CP. Susan Rethlefsen, PT, DPT, and colleagues retrospectively reviewed the records of 121 children with CP. Rethlefsen is the lead physical therapist (PT) at the center’s John C. Wilson Motion Analysis Lab.

A patient at Gillette Children’s Specialty Healthcare undergoes gait and motion analysis, which is also used to assess gait in children with cerebral palsy and knee pain. (Photo courtesy of the James R. Gage Center for Gait and Motion at Gillette Chidren’s Specialty Healthcare in St. Paul, MN.)

The authors noted the following:

• The likelihood of knee pain was almost five times higher in girls than boys.
• The likelihood of knee pain increased with age by about 13% per year.
• The presence of malignant malalignment syndrome showed an association with knee pain severity, which trended toward statistical significance.
• The prevalence of knee pain was equal for Gross Motor Functional Classification System (GMFCS) levels I to III.

Finally, the group found that the eight children who walked with the greatest stance knee flexion (GMFCS level IV) did not report knee pain. The finding may seem counterintuitive because a more severe crouch is generally associated with patellofemoral pain (PFP). But the result did not come as a surprise to the authors.

In a joint email to LER, Rethlefsen, along with coauthor Robert Kay, MD, vice chief of the center, explained that the patients who walked with the greatest amount of knee flexion also walked the least.

“So it makes sense that they put less stress on their patellofemoral joint structures than their more ambulatory peers, and did not experience knee pain,” they wrote.

But that doesn’t mean clinicians can skip asking about knee pain in patients who are mainly sedentary, Rethlefsen and Kay pointed out.

“It is important that practitioners ask about leg and knee pain in children with CP,” they told LER. “Sometimes, the pain will be worsened by specific activities and/or positions, such as going up and down stairs, squatting, or sitting for prolonged times with flexed knees.”

Alter agreed that nonambulatory or less ambulatory patients can experience PFP just as often as ambulatory patients. She said that she’d like to see how the patients in the study by Rethlefsen’s group are doing in 10 years—for example, if a CP patient who continues to walk in crouch all day at school has a higher incidence of pain than those who do only household walking.

“There are a lot of different subpopulation studies within the larger population of children with CP that could…fine-tune our knowledge in regard to pain, crouch, and pain markers,” Alter wrote.

Rethlefsen said her group’s next step would be to investigate whether or not radiographic factors (such as patella alta, patellar pole abnormalities, and tibial tubercle abnormalities) are related to knee pain in children with CP.

Diagnosis

In 2012, Gavelli, Alter, and colleagues conducted a case-controlled study in 20 CP patients, many of whom were in their teens, to quantify the role of patellofemoral kinematics in the development of anterior knee pain (AKP).²

Patients were assessed using a variety of physical tests and then underwent 3D magnetic resonance imaging (MRI), with images acquired during active leg extension and under volitional control. Kinematic markers associated with AKP included greater patellofemoral extension, valgus rotation, and superior and posterior patellar displacement relative to controls and to the subgroup of participants with CP and no AKP.

While Gavelli and Alter continue their research in this area, they suggest that practitioners be aware of those three markers when trying to get a handle on knee pain in younger CP patients sooner rather than later. That information can be incorporated into the physical exam and shared with the physical therapist, the physiatrist, the orthopedist, or the general pediatrician, Alter said.

The end game with identifying these markers is implementing early intervention, they explained. Regardless of the child’s level of ambulation, knee pain needs to be evaluated and managed to keep the patient as mobile as possible.

“The consequences of inactivity and/or a cessation of walking are a cascade that includes atrophy of muscles, deconditioning, increased weakness, joint problems, and loss of function in other areas. So whatever we can do to keep kids active, including walking, is great,” Alter wrote.

Tom Novacheck, MD, a pediatric orthopedic surgeon at Gillette Children’s Specialty Healthcare in St. Paul, MN, recommended a general gait analysis that includes the assessment of knee function as a reliable assessment tool that may be more accessible than MRI for most practitioners.

“You can get a measurement of the stresses on the knee joint, and that will tell you if the stresses are in the front or the inside or the outside of the knee,” explained Novacheck, who is also director of the James R. Gage Center for Gait and Motion Analysis at Gillette. “I find it more helpful than x-rays. X-rays may be abnormal, but that doesn’t necessarily tell you the cause of the pain. It’s actually more helpful to know where the stresses are, and I find that gait analysis is better for that.”

Malignant malalignment is often missed during a general physical exam, and requires computerized motion analysis to pinpoint, Rethlefsen and Kay explained, but there is a lower-tech screening option.

“An an easy way to screen for it is to place a mark on the patient’s patellas and video the patient walking from the front. If the knee points excessively in and the foot excessively out during stance phase, malignant malalignment is likely,” they wrote.

Gait analysis can also help determine if other biomechanical problems, particularly in the feet, play a role in CP-related knee pain. Novacheck pointed out that if flat foot (pes planus) is an issue for a patient, then patellofemoral pain may follow.

He added that gait analysis data can help practitioners with treatment decisions.

“Your diagnosis after the evaluation will help you decide what the goal is—is it pain management or improving gait? That determination may send your patient down different treatment pathways,” he said.

The treatment spectrum

A 12-year-old girl with developmental delays has motion capture markers in place that will be used to collect 3D joint motion and force data to help determine the cause of her knee pain. (Photo courtesy of the John C. Wilson Motion Analysis Lab, Children’s Orthopaedic Center, Los Angeles.)

Experts recommend mixing and matching from a spectrum of treatment options, starting with conservative modalities.

“For all treatment, there is a continuum where you may pick one or more treatments at different times, depending on the patient’s age and functional skills,” Alter explained.

For instance, spasticity is a major issue in the majority of patients with CP. As the leg muscles grow less and less elastic, increased pain and stiffness can lead to PFP. If an immediate goal is to diminish pain, then botulinum toxin injections are a good route. The pain reduction seen after toxin injections may be because of spasticity reduction or because of antinocioceptive effects that maintain or increase the patient’s mobility, Alter said.

Novacheck also recommended toxin injections for addressing high muscle tone and managing pain, as well as other tried-and-true methods such as ice and heat or anti-inflammatory medications.

Taping is another treatment option, the NIH specialists said, as it may reduce some of the abnormal movements of the patella during knee extension, thereby stopping it from subluxing up and over the lateral condyle (see “Patellofemoral taping: Pain relief mechanisms,” LER August 2010, page 25).

“Kids with CP tend to be hypomobile when it comes to the patella,” Gavelli explained. “There is such tension created by the spastic or shortened quadriceps that it has pulled the patella up too high and it’s like a bow string. So a small bit of extra force can offset the balance and cause dislocation. The constant wear and tear from the abnormal position of the patella and dislocation lead to joint problems and pain.”

Also on the treatment spectrum is physical therapy, which may include hip strengthening for reducing pain and increasing lower body strength or quadriceps and hamstring stretching to aid mobilization (see “Strength training improves function in children with CP,” LER: Pediatrics February 2014, page 15).

All of the experts agreed that physical therapy can work wonders for diminishing knee pain—but it also requires patient and caregiver commitment to be successful.

Alter stressed that physical therapy programs need to be based on the child’s level of engagement and, ideally, tailored to their interests. Possible barriers to physical therapy include language or communication issues, behavioral problems, or difficulty following directions.

As for knee braces and orthoses, the experts offered mixed opinions. As Novacheck pointed out earlier, orthoses may be an option if the patient has a foot health problem that is tied to the knee pain. A recent article in Current Opinion in Pediatrics recommended that orthoses be part of the treatment plan for CP-related foot deformities, noting that foot realignment may improve knee function during stance.³

Alter agreed that controlling distal biomechanical problems of the foot, ankle, or both may help the knee, but she cautioned that, when it comes to ankle-foot orthoses (AFOs), the style of the device is important.

“There are some braces that are designed to reduce crouch,” she explained. “But these may not be useful if the patient has a degree of knee flexion contracture.”

Many patients crouch through a combination of weakness and contracture, she said, and “in this subgroup of patients with crouch, these anti-crouch AFOs may not be tolerated or effective.” Alter said. She suggested that knee AFOs (KAFOs) may be useful in some patients with crouch because they can limit flexion, which in turn may alleviate knee pain.

But bracing is only valuable if the patient is compliant, and depending on the child’s age, that can be a major hurdle (see “Kids, clothes, and AFOs: Finding just the right fit,” LER: Pediatrics May 2014, page 9). Novacheck noted that it’s during adolescence, when growth spurts are occurring, that children with CP are most likely to complain about knee pain—but that is an age group that doesn’t smile on anything that makes them more different than their peers.

Finally, there is surgical intervention, which experts agree should be considered only when all other treatments have proven ineffective.

“The vast majority of these children do not require any surgery for their knee pain,” Rethlefsen and Kay stressed. “Surgery should only be considered if conservative measures have failed after a diligent nonoperative trial.”

Surgical options for addressing crouch or limited knee extension include quadriceps lengthening, patellar tendon advancement, surgery on the femur itself, or taking a wedge out of distal femur to improve the patellofemoral kinematics.

“I would say that, if the patient has a lot of bone and joint deformity—some of the common things are femoral anteversion, tibial torsion, knee contracture, foot deformity, patella alta, and patellar stress fractures—those are all things that don’t respond to conservative treatment,” Novacheck said. “Those require surgery.”

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

By P.K. Daniel

High rates of chronic ankle instability in children suggest a need for better prevention and treatment of ankle sprains in young patients, but researchers are just starting to explore whether clinical approaches designed for adults will also be effective in their younger counterparts.

Study findings of surprisingly high rates of chronic ankle instability in children suggest there is a need for increased emphasis on ankle sprain prevention and rehabilitation in the pediatric population. There is some evidence that clinical strategies designed for adults can also be effective in children, but practitioners must often improvise in determining the best approaches for younger patients.

Ankle sprains and resultant chronic ankle instability are common issues among the younger population. More than half of all ankle sprains occur in individuals aged between 10 and 24 years. Teenagers and young adults have the highest rates of ankle sprain, with a peak incidence of 7.2 per 1000 person-years for those aged 15 to 19 years.¹

The injury is most commonly seen in basketball, soccer, running, and ballet and other dance disciplines. Ankle injuries are the most common sports injury suffered by high school athletes, with more than 325,000 occurring each year.^2-4

The only known predictor of an ankle sprain is a previous ankle sprain. It is estimated that half of people who experience an ankle sprain will have a recurrence. But, because most ankle sprains appear to resolve in two to six weeks, many patients do not seek follow-up care. A more aggressive approach to follow-up care is indicated, according to a 2012 article published in American Family Physician.⁵

“One of the highest risk factors [for an ankle sprain] is indeed previous history of sprain,” said Thomas W. Kaminski, PhD, ATC, director of athletic training education and professor in the Department of Kinesiology and Applied Physiology at the University of Delaware in Newark.

With the utilization of preventive tools and rehabilitative methods, this problem could be better managed, in children in particular, researchers said.

“Kids are not often rehabilitated like adults, not because we don’t know what to do, but because they are not brought to the attention of a medical practitioner or clinician,” said Australian researcher Claire Hiller, PhD, who coauthored a study on chronic ankle instability in children in the Journal of Foot and Ankle Research.⁶

Photo courtesy of Pro-Tec Athletics

“Older children who partake of organized sports may attend clubs or schools with an athletic trainer, but in other parts of the world it is usually only people who are aware of rehabilitation and have money who will take kids to be treated.”

The numbers

In the study⁶ coauthored by Hiller, a postdoctoral fellow at University of Sydney’s Arthritis and Musculoskeletal Research Group, 71% of children with a history of ankle sprain had perceived instability and as many as 47% had mechanical instability (see “Rates of chronic ankle instability in children are surprisingly high,” LER:Pediatrics November 2014, page 5).

The ankle sprain is the most common athletic injury in the US, accounting for an estimated 2 million injuries per year and an estimated $4 billion in medical costs.⁷ And yet, ankle sprains are one of the most undertreated injuries. About half of adults do not seek initial treatment for ankle injuries.⁸

Practitioners suspect the number is even higher among children. Untreated, ankle sprains can result in chronic pain, muscular weakness, and instability. In fact, 30% of first-time sufferers develop chronic ankle instability (CAI).⁹

“It is still widely assumed by many that ankle sprains are a minor injury which will heal and don’t have long-term implications,” said Elizabeth Jean Nightingale, BAppSc(Physiotherapy), PhD, University of Sydney research fellow with the Arthritis and Musculoskeletal Research Group in Australia.

Of those who do receive initial treatment, few are referred to a certified athletic trainer or a physical therapist for rehabilitation, noted Phillip Gribble, PhD, ATC, an associate professor in athletic training at the University of Kentucky in Lexington.

“That means treatment for children falls on parents who likely don’t have adequate information to make good decisions on how to proceed with care for their child,” Gribble said.

Those who have CAI ultimately become less active because of pain, weakness, recurring sprains and episodes of “giving way.” CAI is also a known factor in development of post-traumatic ankle osteoarthritis, for which there are no effective conservative treatments.¹⁰

Lack of awareness

Lack of treatment, improper initial management, and failure to rehabilitate contribute to the risk of chronic ankle injury. Tricia Turner, PhD, ATC, associate professor in the Department of Kinesiology at the University of North Carolina at Charlotte, pointed to a lack of awareness among some practitioners as a factor contributing to potentially preventable cases of CAI. Part of the preventive process has to include the medical community, she said.

“My opinion is that most primary care or general medical physicians do not think an ankle sprain is a significant injury or that there is a need for proper rehabilitation to prevent long-term pathology [in children],” Turner said.

She attributed part of the problem to the limited numbers of studies involving children. Turner praised the study by Australian researchers but noted that it also revealed the need for further research regarding children to help practitioners understand that there is a problem.

Kaminski acknowledged practitioners are generally unaware that children are at risk for CAI.

“For the majority of us, it’s probably an afterthought,” he said. “Maybe we need to look at these kids when they’re younger and make sure that the initial ankle sprain is treated with the utmost care, and that we don’t push it and don’t get them back into competition too soon and have the risk of reinjury.”

Gribble surmises that awareness is low among the youth population because awareness is low among the adult population. He also cites a lack of access to care.

“This is probably an important cog in the problem,” he said. “Access to allied health care professionals, especially certified athletic trainers, is limited for the adolescent population in many locations.”

However, recent National Athletic Trainers Association (NATA) figures suggest this is improving. There are more than 43,000 certified athletic trainers nationally, according to the website of the Board of Certification for the Athletic Trainer. As recently as 2009, NATA estimated that only about 40% of high schools had access to a full- or part-time athletic trainer.¹¹ But, as of 2014, approximately 55% of public high school athletes nationwide had access to a full-time certified trainer, according to the NATA website. College and professional sports groups regularly use athletic trainers.

Photo courtesy of Topical Gear.

Treatment strategies

The reported levels of ankle instability in children suggest that a majority are not seeking treatment for ankle sprains, Turner said.

“Ideally, treatment would focus initially on allowing tissue healing to occur [crutch use, bracing] and then functional rehabilitation programs would focus on strengthening the ankle and doing balance exercises,” she said. “I do not think this is practiced with the majority of children who suffer from an ankle sprain. At best they may do the acute management [ice, brace], but few go through a functional rehabilitation program [motion, strength, balance exercises].”

In the 1970s, sports medicine specialist Gabe Mirkin, MD, introduced the commonly used practice of RICE (rest, ice, compression, and elevation) for treating acute ankle sprains. However, he has reversed course, asserting on his website, (DrMirkin.com) that lengthy icing of an injury inhibits healing by reducing inflammation (drmirkin.com/fitness/why-ice-delays-recovery). He cites a review summary of 22 scientific articles that found almost no evidence that ice and compression hastened healing over the use of compression alone. However, the review said ice plus exercise may provide marginal help in healing ankle sprains.¹²

Limited icing immediately following an injury is acceptable, said orthopedic surgeon Howard J. Luks, MD. Luks, who is associate professor of orthopedic surgery at New York Medical College and chief of sports medicine and arthroscopy at Westchester Medical Center in New York, recommends icing in short bursts.

“Ice for five minutes at a time and then leave it off for at least thirty minutes to allow the blood flow to return to the area,” Luks said. “After a few hours, the ice will no longer be effective in managing the swelling and should be avoided.”

The American Orthopaedic Foot and Ankle Society (AOFAS), as reported on its website (aofas.org), recommends that once the pain and swelling subsides, which is typically within five to seven days, stretching exercises should be performed. According to AOFAS, the first objective is to restore ankle range of motion, followed by ankle strengthening. Working toward comfort and stability, or proprioception, is the final stage.

Established evidence¹³ has shown that balance training exercises, taping, and prophylactic bracing can minimize the risk of future instability in adults, and a few studies conducted in adolescents suggest that similar strategies can also be effective in pediatric populations.

“If we look at the successful interventions from the literature on CAI in adults, perhaps protocols addressing deficits in neuromuscular control and balance, as well as potential restrictions in range of motion and arthrokinematics that have exhibited success in adult populations may prove useful for the prepubescent populations,” Gribble said.

Nightingale concurred.

“The treatment of primary ankle sprains is practitioner-dependent, but rehabilitation in children is as for adult sprains—with balance as a part of regular practice, and strapping tape or braces may be used,” she said.

In adults, one of the best methods to improve function in patients with chronic ankle instability is through a balance training program, Turner said. Most of these balance training protocols are four to six weeks in duration. They incorporate both static balance (balancing on one leg), as well as dynamic balance (standing on one leg while reaching or performing sport-specific skills). Studies^14,15 have demonstrated the usefulness of gaming programs (eg, Wii Fit balance platform) for balance training in adult populations, and the same technology could potentially be used in children.

Photo courtesy of MedSpec

Evidence in adolescents

Tim McGuine, PhD, ATC, a senior scientist in the Department of Orthopedics and Rehabilitation at the University of Wisconsin in Madison, coauthored a 2006 study in the American Journal of Sports Medicine (AJSM) on balance training in high school basketball players. There was a significantly lower rate of ankle sprains among 765 high school soccer and basketball players who participated in a balance training program than in a control group that performed standard conditioning exercises. In athletes with a history of ankle sprain, the risk of a recurrent sprain was cut in half in those who participated in the intervention.¹⁶

Athletes in the intervention group participated in preventive exercises for 10 minutes per day, three times per week during the season. The balance training program included five phases: (1) maintaining a single-leg stance on a flat surface with eyes open and closed; (2) performing functional sport activities such as throwing, catching, and dribbling on one leg; (3) maintaining a double-leg stance while rotating the balance board; (4) maintaining a single-leg stance on the balance board with eyes open and closed; and (5) performing functional sport activities while in single-leg stance on the board.

A 2011 AJSM study by McGuine and colleagues on high school basketball players and lace-up ankle braces also had positive results.¹⁷ The researchers found that there were 68% fewer ankle injuries in those who wore them versus those who did not. Gender differences had no bearing on the results, nor did having a previous history of ankle injury. The rate of acute ankle injury (per 1000 exposures) was .47 in the braced group and 1.41 in the control group of unbraced athletes. While braces were associated with a lower incidence of ankle injuries, bracing did not have an effect on injury severity, the study authors concluded.¹⁷

“We know now that bracing’s effective, these exercise programs are effective,” McGuine told LER.

Kaminski questioned whether the way children’s ankle injuries have been treated has been less conservative than necessary.

“Maybe we need to think in the reverse manner,” he said. “Maybe we need to be extremely cautious and extremely conservative with these young children who do suffer ankle sprains to prevent them from having recurrent instability.”

Kaminski said children should be immobilized initially and their return to activity slow and gradual. He recommended using balance training in prevention of ankle sprains, particularly in those who have had previous sprains. Kaminski also pointed to the need for preventive care and the strong evidence that prophylactic bracing, taping, and balance training can help reduce first-time ankle sprains.

Taking a conservative approach to returning to play seems to be gaining popularity among adults, as well. Minnesota Timberwolves point guard Ricky Rubio went down with a severely sprained left ankle on November 7, 2014. Rubio remained sidelined until February 2, frustrating T-Wolves fans. But Rubio’s recovery process was delayed—perceived instability in his ankle or not—because of muscle and ligament damage. Minnesota coach Flip Saunders said two medical specialists who consulted with Rubio advised that returning too soon could increase the risk of a stress fracture.

But practitioners need to remember that children are not just small versions of adults, and some aspects of ankle instability prevention may need to be managed differently for a younger population, Hiller said.

“We can certainly apply what we know about treating CAI in adults to kids, but often need to modify the regime to be age appropriate,” she said. “For example, exercises are often repetitive and adults can be assisted in compliance by logic and reason. Kids need to be given greater variety in shorter bursts. Some practitioners do not recognize this and rehabilitation may not be as successful.”

Sole stiffness affects stance time

By Erin Boutwell

A recent study published in the winter issue of Pediatric Physical Therapy found that children just learning to walk may have altered gait characteristics when wearing flexible shoes.

Whether novice walkers should wear flexible or stiff shoes is not a new question, said Melanie Buckland, DPT, at the Hospital for Special Surgery in New York City and study author, but it is a frequent question fielded by pediatricians and physical therapists.

Previous studies have associated stiff shoes with the delayed development of arches in early walkers, and arch development is often the primary focus of the flexible versus stiff shoe debate. However, Buckland and her coauthors contend that shoe flexibility may also have an important influence on children’s functional activities, especially walking and balance.

Buckland’s study investigated the gait and stability of 25 early walkers, children aged 9 to 24 months who had been walking for less than five months. Four shoes with different torsional flexibilities (from
most flexible to least: UltraFlex, MedFlex, LowFlex, and Stiff) and a barefoot condition were compared during level walking.

While walking speed and step length were not affected by shoe flexibility, stance time was significantly lower for the most flexible shoe (UltraFlex) than the LowFlex shoe. Additionally, the barefoot condition was associated with a significantly shorter stance time than any shod condition.

Buckland attributed the shorter stance time in the UltraFlex condition to improved sensory feedback, saying, “We believe they were able to feel the ground better with the more flexible shoe…and they were able to accommodate and go ahead and take another step.”

Elaine Owen, MSc, superintendent physiotherapist at the Child Development Center in Bangor, North Wales, UK, proposed an alternative explanation: Reduced stance time could be a result of biomechanical effects of the footwear.

“The stiffer the material…the longer it will take for the MTPJs [metatarsophalangeal joints] to extend to the amount they need to by end of stance,” Owen explained.

In addition to gait, Buckland and her coinves­tigators designed an obstacle course to assess the stability of these early walkers by counting the number of falls and stumbles. No significant differences were found among the various shoes, nor did children perform better when barefoot than when shod.

That stability was unaffected by shoe flexibility is a surprising result, as work published in 2013 by researchers at the Hospital for Special Surgery in the Journal of the American Podiatric Medical Association demonstrated that increasingly flexible shoes were associated with greater plantar loading in the same group of early walkers.

The authors of that paper, Buckland among them, identified plantar loading as a mechanical feedback mechanism that could improve these early walkers’ proprioception. The authors also anticipated that stability might be improved in a more flexible shoe if the child was able to take advantage of the intrinsic structures of the foot.

“The foot is made up of many small bones and joints for mobility,” Buckland explained, “so the foot needs to be very mobile, flexible, able to accommodate to the ground for balance.”

In the current study, stability—as evaluated through stumbles and falls—was not related to shoe flexibility. However, step width was larger in the UltraFlex shoe compared with the MidFlex and LowFlex shoes. According to the study authors, this finding is a possible indication of reduced gait stability with the most flexible shoe.

Increased step width is often associated with a reduction in stability, but other explanations are possible. Coronal plane curvature in the shoes themselves may have contributed to an unstable base in the mediolateral direction, Owen suggested.

Three of the four tested shoes were made by the same manufacturer, while the Stiff shoe was produced by a different company. Buckland acknowledged that factors such as heel height, position of the toe break, and insole materials—none of which were controlled in the current study—could influence how children walk in these shoes.

Owen asserted that the shoe profile is a primary consideration when considering gait biomechanics.

“You can walk very well in a stiff shoe as long as you have the right profile on the shoe,” she said. “A lot of stiff shoes actually have a rounded profile.”

Nonetheless, Owen affirmed that flexibility is a significant factor in shoe design.

“I feel that the footwear should be very flexible, the most important reason being to use the windlass mechanism [critical in arch development]…if stiffness is introduced, this does not occur,” she said.

Similarly, Buckland emphasized the importance of continuing to explore the effects of shoe flexibility.

“With further research, we can really help these kids promote their development and their gross motor skills,” she said.

Erin Boutwell is a freelance writer based in Chicago, IL.

Sources:

Buckland MA, Slevin CM, Hafer JF, et al. The effect of torsional shoe flexibility on gait and stability in children learning to walk. Pediatr Phys Ther 2014;26(4): 411-417.

Hillstrom HJ, Buckland MA, Slevin CM, et al. Effect of shoe flexibility on plantar loading in children learning to walk. J Am Podiatr Med Assoc 2013; 103(4):297-305.

Experts call for age-specific options

By Chris Klingenberg

With head trauma becoming increasingly worrisome in sports these days, a significant concern is whether the same protocols for clearing an athlete to return to sports should be used in both adults and children. Research from Cincinnati Children’s Hospital Medical Center suggests that the Balance Error Scoring System (BESS) may not be as useful for assessing postural sway after concussion in children as it is in the college athletes for whom it was originally developed.

“The BESS has a number of limitations when being applied to the pediatric population,” said Catherine Quatman-Yates, DPT, PhD, an assistant professor in the Department of Pediatrics and first author of the study. “First, it was designed as a sideline test and is best when you have preinjury baseline scores to compare to a postinjury performance. These are rarely available for children. Second, the balance challenges that the BESS utilizes are not specific to postinjury changes. Age- and maturation-related factors can also significantly affect a child or adolescent’s performance on the BESS, so it can be difficult to know if the errors are related to an injury or to other factors, even when baseline scores are available.”

Quatman-Yates and colleagues assessed the reliability and validity of the BESS for use with children and adolescents with a recent diagnosis of mild traumatic brain injury and a cohort of age-, sex-, and activity-matched healthy peers. There were 20 children (13 boys) in each cohort; the mean age was 13.24 years. Participants from the injured cohort were assessed within 14 days of injury (mean, 7.42 days).

The researchers found significant differences between the injured and control participants for single-leg firm stance, tandem firm stance, single-leg foam stance, and total BESS score. Injury status uniquely explained 18.9% of the variance for single-leg firm stance, 20.7% of the variance for single-leg foam stance, and 19.5% of the variance for total BESS score.

Actual between-group differences for the corresponding mean BESS scores were small, however, ranging from 1.1 (out of 10) for single-leg foam stance to 3.45 (out of 60) for total score. The differences were not great enough to fall outside the within-rater and between-rater minimal detectable change estimates of 7.3 and 9.4 points, respectively, for youth athletes. The findings were published in the September 2014 issue of The Physician & Sportsmedicine.

The Cincinnati researchers found that, within the injured cohort, younger children were more likely than older children to commit errors during the BESS test, which raises another potential concern specific to youth athletes: A greater potential for errors also means a greater potential for rater mistakes in observing and recording errors correctly.

Typically in college athletes, using the BESS as part of a postconcussion assessment is more valuable when an athlete’s scores can be compared with a previously performed baseline test. In youth athletes, however, that isn’t always possible, Quatman-Yates said.

“A lot of times with kids the problem is there is no baseline,” she said. “Another problem is that when the kids go through puberty it is harder to hold a baseline. Plus, all kids go through changes at different times.”

Tamara McLeod, PhD, ATC, professor and director of the Athletic Training Program at A.T. Still University in Mesa, AZ, agreed that postconcussion assessment protocols for children should indeed be different from adult protocols.

“Yes, I think postural control is an important part of the assessment piece and should be done in all athletes,” McLeod said. “We do know that postural control is usually worse in younger children as they have not fully developed their postural control systems, so getting a baseline for them is important to have an individualized healthy comparison. We have used the Balance Error Scoring System in children as young as eight years. I think balance tests need to be developed for children that are age-specific.”

Researchers and clinicians are starting to explore other options for balance testing in children. Quatman-Yates said she is seeing increased use of the Force Play Test in children, in which postural control is assessed while the kids stand with two feet together and have their eyes open and closed for two minutes. And McLeod and colleagues have submitted an abstract to the upcoming 2015 annual meeting of the National Athletic Trainers Association that shows even children have good reliability in postural control when measured using Sway, an app that evaluates stability and reaction time, she said.

Chris Klingenberg is a freelance writer based in Massachusetts.

Source:

Quatman-Yates C, Hugentobler J, Ammon R, et al. The utility of the balance error scoring system for mild brain injury assessments in children and adolescents. Phys Sportsmed 2014;42(3):32-38. 

MRI images show the lower legs of a child with bi- lateral clubfoot. The smaller leg is the affected side, showing smaller muscles. (Image courtesy of Matthew Dobbs, MD.)

Analysis focuses on muscle deficits

By Erin Boutwell

Children with idiopathic clubfoot who have relapses after treatment with the Ponseti method demonstrate different soft tissue abnormalities than children whose clubfoot is permanently corrected, according to research published in the August issue of the Journal of Bone & Joint Surgery.

“Once we gain correction with the Ponseti method, we still have a number of kids who go on to have trouble with the deformity recurring,” according to Matthew Dobbs, MD, professor of orthopedic surgery at Washington University in St. Louis, MO, and study author.

Dobbs and colleagues recruited 20 patients with clubfoot who had previously undergone treatment using the Ponseti method of serial manipulation and casting. Patients were categorized as “treatment-responsive” (no relapses) or “treatment-resistant” (relapses that did not require extensive surgery), and had completed their final casting a minimum of one year prior to the study. The soft tissue composition of both limbs was evaluated using magnetic resonance imaging (MRI). Researchers measured the total cross-sectional area of the limb, as well as the relative amounts of muscle and fat, and, in unilateral patients, compared the affected and unaffected limbs.

In the six patients with unilateral treatment-responsive clubfoot, the affected limb had a 15.3% smaller mean cross-sectional area and 26.6% less mean muscle tissue than the unaffected limb. The five unilateral patients in the treatment-resistant group demonstrated a larger between-limb difference in intracompartment adiposity index (IAI, the ratio of fat tissue to overall soft tissue) than the treatment-responsive group. The treatment-resistant limbs were also characterized by more intracompartment fat tissue than the unaffected limbs. Further, these patients exhibited an even more pronounced between-limb imbalance in muscle tissue (47.8% less muscle tissue on the affected side than the unaffected side) than the treatment-responsive patients (26.6%).

Dobbs suggested that detecting soft tissue abnormalities such as muscle weakness or absence through physical clinical examination may permit more individualized prescriptions for children with clubfoot, in which casting and manipulation procedures could be personalized based on features of their individual presentation. Specifically, the findings of this MRI study may assist in identifying abnormalities associated with clubfoot presentations that are less responsive to the standard Ponseti method.

Jose Morcuende, MD, PhD, chief medical director of the Ponseti International Association, challenged the “treatment-resistant” label used within this study for cases of clubfoot that did not respond to the usual Ponseti method. Morcuende noted that, in an earlier study, co-authored by Morcuende and Dobbs and published in the October 2006 issue of Clinical Orthopaedics and Related Research, all but two of 50 “complex” idiopathic cases were successfully treated using the Ponseti method. The distinction between “resistant” and “complex,” Morcuende asserted, has more to do with the skill of the practitioner than differences in soft tissue characteristics: “It’s not the foot per se, but the hands of the doctors.”

Still, identification of children who are less likely to be treated successfully with the standard Ponseti method could be another advantage of detecting soft tissue differences.

“Knowledge of abnormalities in certain muscle groups…may lead one to increase the time in the post-corrective brace or, more likely, recommend early anterior tibial tendon transfer,” said Lewis Zionts, MD, a clinical professor of orthopaedic surgery at the David Geffen School of Medicine at UCLA.

Morcuende cautioned against using the MRI findings to recommend premature surgery for children whose soft tissue composition seems to predispose them to be resistant to Ponseti treatment.

“It will take you ten casts or twelve casts, but you can get those feet corrected,” he said.

A third possible benefit of knowing the soft tissue characteristics associated with clubfoot is a more reliable classification system to improve the prediction of treatment responsiveness, the need for which was mentioned by both Dobbs and Zionts.

“Unfortunately, current clinical examination techniques do not accurately predict the likelihood of the response to Ponseti treatment,” Zionts said.

Dobbs and his fellow authors anticipate that these MRI studies of soft tissue abnormalities may be used in the future development of a new clinical classification method for clubfoot, in which functional and structural characteristics of the affected limb are assessed.

“Our ultimate goal with this MRI study is to take it back to the physical exam, and turn this into a new classification system for clubfoot that’s going to be prognostic,” Dobbs explained, “because it shouldn’t be one global treatment for all clubfeet when all clubfeet are not alike.”

Erin Boutwell is a freelance writer based in Chicago, IL.

Sources:

Moon DK, Gurnett CA, Aferol H, et al. Soft-tissue abnormalities associated with treatment-resistant and treatment-responsive clubfoot: Findings of MRI analysis. J Bone Joint Surg Am 2014;96(15):1249-1256.

Ponseti IV, Zhivkov M, Davis N, et al. Treatment of the complex idiopathic clubfoot. Clin Orthop Relat Res 2006;451:171-176.

Photo by Joshua Albanese (joshuaalbanese.com)

Orthotic devices, tailored to each patient and adjusted for disease progression, can improve mvobility by addressing gait impairments and maintaining stability.

By Cary Groner

Orthotic management of the muscle imbalances associated with Charcot-Marie-Tooth (CMT) disease is a critical part of preventing or delaying later complications, which may include foot deformities and severely impaired gait. Because CMT is caused by genetic mutations that affect the nerves, such interventions don’t affect the progression of the disease itself; they can, however, help ameliorate its worst manifestations.

Clinicians wrestle with balancing correction and accommodation when prescribing orthotic devices for patients with CMT, and such decisions depend partly on the patient’s condition and wishes, as well as on the progression of the disease.¹

What’s most important to patients isn’t always at the top of the list for practitioners, however.

“I ask my patients to prioritize,” said Ken Cornell, CO, who practices with Cornell Orthotics & Prosthetics in the Boston area. “Usually they complain about lateral ankle instability, painful calluses, foot drop, or claw toes. Only rarely do they mention balance problems, but I’ve found that, when you restore their balance, they suddenly realize what a big problem it was.”

Cornell agreed that orthotic intervention doesn’t address the underlying pathologies associated with CMT—an ankle foot orthosis (AFO) doesn’t give patients back their lost muscle strength or proprioception—but it can do other things.

“What’s challenging and exciting is restoring both static and dynamic balance by derotating the foot and trying to restore its alignment,” he said. “That changes the path of the center of pressure as it tracks over the foot, and that improves patients’ functional balance.”

Photo by Vincent Giordano
Trinacria Photography (trinacriaphotography.com).

According to Cornell, skilled orthotists can also address the deterioration that may lead to a “slapping” foot during the stance phase of gait.

“Particularly with dynamic carbon bracing, we can restore those three stance-phase rockers to normalize someone’s gait pattern,” he explained. “They have a heel strike, then controlled plantar flexion as the foot comes down to the ground. They’re getting dynamic resistance from the carbon so it’s giving them a ground reaction that tells them where their center of gravity is over their base. Then the tibia advances over the foot until it gets resistance in dorsiflexion, and then it raises the heel, which is the normal third rocker.”

Research supports the efficacy of AFOs for treating gait issues in CMT patients. One 2012 study from the UK, for example, noted that CMT patients often compensated for foot drop by increasing hip flexion during swing. In 14 individuals, three types of AFOs designed to address foot drop improved both proximal and distal leg control, decreased hip flexion amplitude during swing, and increased both ankle dorsiflexion and foot clearance.²

In a study noted in LER last year,^3,4 researchers fitted eight CMT patients with custom carbon-fiber braces and reported that participants walked faster with the braces, particularly if they were relatively weak to begin with. As velocity increased, moreover, maximum joint moments during loading response shifted from the hip to the ankle and knee joints; the hip joint dominated during propulsion.

Clinical experience

Overall, however, the relative paucity of research on orthotic treatment of CMT means that clinicians often develop approaches based on their own experience and that of their colleagues. Collaboration is often a key element of success.

“The literature as to when to use orthotics or AFOs, or when to perform surgery, is very poor,” said Michael Shy, MD, a professor of neurology, pediatrics, and physiology at the University of Iowa’s Carver College of Medicine in Iowa City. “It’s almost always based on a person’s clinical judgment. I’ve been fortunate enough to work with many talented orthotists who’ve all seen a lot of patients with CMT. They understand the foot structures and disease progression, and they’ve seen people respond over time. They’re partners in providing care, and they know more about their field than I do.”

Photo by Susan Ruediger

Although genetic screening has led to the identification of at least 80 subtypes of CMT, when making treatment decisions orthotists are more concerned with how the condition presents.

“It’s not really diagnosis-specific, because as an orthotist you have to deal with the biomechanics that are present,” said Sean McKale, CO, LO, practice manager at Midwest Orthotic and Technology Center in Chicago. “People with the same type of CMT may be at different stages of progression, or just affected very differently, so you have to evaluate them as individuals and assess their symptoms to address their needs.”

One of the first factors McKale evaluates is a patient’s strength—or, more precisely, how much of it they’ve lost.

“Muscle weakness plays a big role in terms of decisions about level of support and the dynamics of the orthotic device,” he said.

Other concerns are the position of the foot, loss of range of motion (ROM), triplanar deformity, and proprioception.

“You use these clinical findings to help guide you, to show you the path to take with that patient,” McKale said.

McKale believes functional alignment correction is more important than accommodation in most cases.

“I think that with accommodation you end up allowing the foot to become more deformed,” he said. “The exception is when there’s been surgical fixation or fusions, which limit range of motion so that you can’t gain functional corrections.” In such cases McKale prefers a stable nondynamic device to maintain an aligned position.

In more typical cases, however, his goal is to balance the foot as well as possible to prevent further deformity.

“Someone with better proprioception can deal with increased dynamics,” he said. “You have to vary the device you select, its stiffness and trim lines, based on the dynamic needs of the patient.”

According to Geza Kogler, PhD, CO, director of the Clinical Biomechanics Laboratory in the School of Applied Physiology at the Georgia Institute of Technology in Atlanta, addressing common gait issues with orthotic devices can make important contributions to patients’ quality of life.

“The ability for someone to lift their toe during swing phase has a dramatic impact on their ability to walk efficiently,” Kogler said. “That’s the primary benefit of the orthosis; a secondary benefit is that it slows down the foot during heel strike and stance phase, to help minimize foot slap. It also helps with stability during standing, and these things together have a profound impact on patients.”

Kogler agrees with McKale that alignment should be maximized. In some cases, however, it isn’t possible.

“Over time, with the loss of muscle, there can be contractures of the calf musculature, and that can lead to permanent deformity,” Kogler said. “That can change the alignment so that you can’t get the foot into a neutral position during standing, and in those cases you have to accommodate that alignment. You might have to put a lift under the heel to reach neutral, for example.”

Allowing the muscles to work

Photo by Vincent Giordano Trinacria Photography (trinacriaphotography.com).

According to David Misener, CPO, who practices with Clinical Prosthetics and Orthotics in Albany, NY, orthotists typically strive to find the best balance between correction and accommodation. Misener has a deeper experience of CMT than most clinicians because the disease runs in his family and he has it himself.

“You have to be careful not to overbrace someone,” he said. “The muscles need to work. Orthoses do need to support and align the body, though, and that can be as simple as an in-shoe orthosis. You can start with a very low profile device, inside the shoe, and then as the disease progresses, start working your way up the chain.”

In his practice, however, Misener frequently sees patients who haven’t been diagnosed early enough to allow for more minimal interventions.

“Ideally you want preventive orthotic management to maintain range of motion around specific joints,” he said. “To balance correction and accommodation, I think we have to be more proactive in getting functional corrections of alignment around joints, because bracing becomes exponentially more difficult when more range of motion is lost.”

According to Misener, patients should never be made physically uncomfortable by their devices, but other factors can also affect compliance. His experience is that CMT patients resist wearing corrective devices because they want to feel as “normal” as possible. This tends to backfire, however, as they start to lose range of motion.

“Ideally, you’ll get a diagnosis early and start with simple corrective orthoses,” Misener said. “Then, if you’re starting to get muscle weakness around the ankle, or foot drop, a carbon brace will still allow range of motion and allow the muscles to work without over-supporting them. I think having some fatigue throughout the day is good, because it means you’re exercising and strengthening your muscles.”

Maintaining range of motion is paramount as CMT progresses, Misener said.

“The key is to get the foot in subtalar neutral so you’re really stretching what you need to be stretching,” he said. “Night splinting [for Achilles tightness] can help, particularly with kids. They don’t want to wear braces, but if they do they’ll often gain range of motion.”

Disease progression

As Misener evaluates disease progression in his patients, he tries to address problems with the least drastic intervention possible.

“I think dynamic braces are a solid way to go,” he said. “Carbon systems are great because they’re lightweight and reduce fatigue, but you have to get the alignment correct. I like to be a minimalist whenever possible, but if I have to build something to overpower a muscle imbalance, then I have to go stronger. But I try to go stronger in a more dynamic way; you’d like to have the individual control their body as much as they can.”

Geza Kogler agreed that it’s crucial for the clinician to adapt to disease progression. In his experience, orthotic treatment and stretching should work together.

“A patient may go for years wearing an AFO that sets an alignment, but if they don’t keep up their stretching routine, they can end up with significant foot and ankle deformities that can complicate the fit of that AFO,” he said.

Kogler sees patterns in adjusting orthotic devices to disease progression.

“Early on, if someone has drop foot, a dorsiflexion-assist AFO might be enough, so the ankle can still move. But with time, if they develop more of a contracture, that assist will be overcome by the patient’s calf muscles, and they’ll have to switch to a solid-ankle AFO. But if they’ve stuck to a good stretching regimen, they may be able to avoid that step. It can’t always be avoided, but it can be delayed,” he said.

Ken Cornell agreed that a primary goal of orthotic intervention is to prevent deformity as CMT progresses, but that strategies depend on the individual patient.

“There is no one CMT brace, because everyone has a different level of gadget tolerance,” Cornell said. “You really have to make the patient part of the plan. If I apply too much pressure, to the point that the brace is uncomfortable and breaking down skin, no one is going to wear that. You have to understand the patient’s priorities.”

The role of physical therapy

Photo by Jordana Bieze Foster

As noted, physicians and orthotists play a big part in determining how patients respond to orthotic interventions. But physical therapists often play an important role, too, particularly because they often work with patients who have just received an orthotic device.

“Walking is an automatic task for most of us, but when people develop weakness or sensory changes, it requires much more cognitive attention,” said Katy Eichinger, DPT, CS, who practices in the neuromuscular division of the Department of Neurology at the University of Rochester in New York. “In slowly progressive conditions such as CMT, the body adapts and gait changes over time. We can be instrumental in helping patients relearn walking, by using different strategies and gait-training techniques to accommodate assistive or orthotic devices.”

For example, as part of these overall goals, physical therapists can help patients compensate for sensory loss, according to Eichinger. As clinicians have noted above, however, the success of such strategies has partly to do with getting the patient on board.

“Some people don’t like to use a cane, but they’ll use a hiking pole,” Eichinger said. “That may be all they need; you can see their gait improve because the pole provides increased sensory input about their position in space. It’s just a matter of teaching them to accommodate assistive and orthotic devices to maximize their functional abilities and gait techniques so they’re as energy efficient as possible.”

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

Photo by Joshua Albanese (joshuaalbanese.com)

Early diagnosis and early intervention in people with Charcot-Marie-Tooth disease give clinicians and patients a valuable head start on keeping symptom progression in check.

By Cary Groner

David Misener, CPO, understands Charcot-Marie-Tooth (CMT) disease firsthand because he has it himself, as does his son. Misener, who practices with Clinical Prosthetics and Orthotics in Albany, NY, said his case was difficult to diagnose at first because he and those in his family don’t carry the genetic mutation usually associated with the disease.

“They first ran the test on CMT1A, because that’s the most common, but they didn’t find the error,” he told LER.

As it happens, his family carries a different genetic mutation, CMT1B, which has the same symptoms but originates in a different gene.

“If you’re not one of those with type one-A, they have to keep looking, and it can become expensive,” Misener said.

Closing the information gap

CMT, a sensorimotor neuropathy marked by progressive muscular atrophy, was first described in 1886 by physicians from France and England. The disease typically begins in the intrinsic muscles of the foot, then extends to the peroneus brevis and longus, the tibialis anterior, the extensor digitorum longus, and the extensor hallucis longus. It may eventually involve the hands, as well, and it’s even been associated with seemingly unrelated conditions such as scoliosis.^1,2 Roughly one in 2500 people in the US has CMT, according to the National Institute of Neurological Diseases and Stroke (NINDS), and men are affected more often than women, in approximately a 5:3 ratio.³

David Misener’s diagnostic challenges occurred 20 years ago, and since then researchers have continued to refine their understanding of the genetic mutations that cause CMT. Research has revealed ever-expanding subclasses of the disease, in fact, which is usually inherited in an autosomal dominant pattern (ie, if one parent has CMT, each child has a 50/50 chance of inheriting it). However, some CMT subtypes may be passed on in an X-linked, or recessive, pattern, and the disease may also arise due to spontaneous or de novo mutations.⁴

“The most common subtype, CMT1A, affects about half of CMT patients,” said Michael Shy, MD, a professor of neurology, pediatrics, and physiology at the University of Iowa’s Carver College of Medicine in Iowa City. “As of now, four subtypes account for ninety percent of genetically identifiable CMT: CMT1A, CMT1B, CMTX, and CMT2A.”

According to Shy, CMT1A is considered the classic phenotype.

“These patients are likely to have been slow runners and clumsy as children, and as they reach adulthood many will need foot orthoses or ankle-foot orthoses (AFOs) to help them ambulate,” he continued. “Only a small percentage will ever need a walker or a wheelchair.”

Other subtypes exhibit different characteristics, Shy said. For example, some CMT1B patients don’t begin to walk until two or three years of age and are severely affected in the first two decades of life, as is also the case with many CMT2A patients.

“It just depends on the particular mutation within the subtype,” he said. “It can range from severe early on to very mild, though as a rule the recessive types—such as CMT4—are often fairly severe.”

Photo by Jordana Bieze Foster.

Pathophysiology

CMT pathophysiology has traditionally been classified as either predominantly demyelinating or axonal, but clinicians and researchers disagree about the relative importance of these categories with regard to manifestation and progression of the disease.² For example, although axonal degeneration is a predictor of disability—suggesting that axonal damage may be the root cause of the neuropathy—the mutations responsible for the different forms of CMT1 typically occur in myelin genes, and myelin disturbances lead to axonal damage. Further muddying the waters is that axonal damage can result in secondary demyelination.²

Genes cause CMT by disrupting particular proteins, Shy explained, and if those proteins form a constituent of myelin, the myelin will be damaged.

“You will see axonal damage in CMT1,” he said, “but the primary problem is the myelin.”

Some aspects of CMT remain perplexing, however.

“Many of the genes that cause CMT2—the axonal form of the disease—are expressed widely throughout the body, not just in neurons,” Shy said. “So why other organ systems aren’t affected is a mystery. It probably has something to do with the biology of the neurons, since they don’t divide, and have long axons that have to be maintained throughout the person’s lifetime.”

Axon length likely explains why distal parts of the body, starting with the feet, are affected first. And, because CMT affects both motor and sensory nerves, patients can experience not only muscle weakening and imbalance, but also problems with proprioception, Shy said.

Some muscles weaken more quickly than others, moreover, which leads to imbalances between dorsiflexors and plantar flexors. These imbalances, in turn, contribute to contracture of the Achilles tendon and problems such as cavus feet, calcaneal inversion, forefoot adduction, and claw toes.⁵ Other CMT-related problems include skinny calves, scoliosis, foot drop, “slapping” gait, foot numbness, muscle weakness, balance difficulties, and multiple non-neuropathic pain symptoms.^2,4

Diagnostic challenges

The heterogeneity of symptoms associated with CMT, and the subtlety with which they often present, may lead to delayed diagnosis in some patients.

“Many times people get diagnosed well after the age of onset,” David Misener said. “Those with mild cases may not realize they have the disease until they’re in their sixties and develop a drop foot.”

Because more than 80 CMT subtypes have been identified, the broad range of severity and age of onset can also contribute to missed diagnoses.

Photo by Vincent Giordano
Trinacria Photography (trinacriaphotography.com)

“In many families with CMT, you sometimes reach a tipping point where something gets your attention, such as stumbling,” Misener continued. “I think the disease probably progresses at an even rate, but certain events make you think it may have advanced more suddenly.”

Michael Shy agreed.

“In CMT1A patients, the disease tends to develop slowly through the first two decades of life,” he said. “Some CMT1B can present early, others don’t present until adulthood. It depends on the particular mutation.”

In making a diagnosis, Shy typically looks for length-dependent weakness and sensory loss, particularly in the hands and feet.

“Usually we look for absent ankle reflexes,” he said. “In some cases, all the deep tendon reflexes can be absent, and then we use nerve conduction testing to determine whether or not it’s primarily a problem with myelin or the axons.”

Although Shy and other specialists are attuned to CMT, many clinicians are not. The result, he said, is that the initial diagnosis is sometimes made by a foot specialist rather than a primary care physician.

“I think podiatrists are often the first people to recognize that someone may have CMT, because they see pes cavus or other foot abnormalities,” Shy said. “Length-dependent weakness and atrophy, and decreased deep-tendon reflexes are things that primary care physicians may pick up, though.”

Ken Cornell, CO, who practices with Cornell Orthotics & Prosthetics in the Boston area, finds himself making the initial diagnosis less often than he used to.

“Twenty or twenty-five years ago I was getting a lot of these patients, and I’d call their doctors to discuss CMT, and they wouldn’t even know what I was talking about,” he said. “In the last few years that’s started to change, I think partly due to outreach from the CMT Association.”

Cornell said his patients usually present with one or more of five key symptoms: poor balance, foot drop, lateral ankle instability, sensory loss, or painful calluses. And, although nerve conduction studies have typically been considered definitive, increasing knowledge about the genetic basis of CMT has elevated demand for genetic testing.

Photo by Vincent Giordano
Trinacria Photography (trinacriaphotography.com)

Disease progression

Once the diagnosis of CMT is made, through either traditional means or genetic testing, clinicians and patients must monitor the disease’s progression to determine the most appropriate interventions. As noted previously, the disparity in weakness between opposing muscles may ultimately lead not just to imbalances but to deformities.

“The longest nerves in the body are affected first,” reiterated Ken Cornell. “In a man who’s six feet tall, one single nerve cell going from the spine to the foot can be three feet long. That cell is affected first, and that in turn has effects on the muscles that extend the toes. But the muscles that flex the foot are higher up in the leg; they’re shorter nerves, affected later, so you wind up with unopposed toe flexion. That’s how you get claw toe deformity.”

“The nerves are not feeding the muscles, so they start wasting away, and this creates muscle imbalances over specific joints,” added David Misener. “As one muscle pulls harder over that joint, it can affect the shape of the foot, which becomes cavus. Drop foot arises because the anterior muscle group weakens before you reach that tipping point in the calf muscle, and those anterior muscles lift the foot during swing phase and decelerate it at heel strike. These things start with the small intrinsic muscles in the feet, then affect the ankles, then the knees, and in some people, the hips.”

Cornell added that these disease traits make progression reasonably straightforward to predict.

“People are typically focused on the motor loss first, which usually manifests first with foot drop, then with everter paresis,” he explained. “The peroneus longus—the plantar flexor of the first ray—outlasts its antagonist, the anterior tibialis, so you wind up with a plantar flexed first ray and a tripod effect. This causes an inversion twist to the rearfoot, which sets you up for lateral ankle instability and forefoot adduction. All these muscle imbalances are progressive, and they set up the conditions for deformities.”

Michael Shy noted that certain types of nerves seem to develop problems more quickly than others, and it may not always have to do with nerve length.

“For reasons that are unclear, the nerves that cause dorsiflexion and eversion seem to get damaged earlier than those that cause plantar flexion and inversion, so you get a lot of anterior calf wasting,” he said.

With all the attention to motor nerve deficits, Shy added, CMT’s effect on sensory nerves shouldn’t be downplayed.

“Most CMT types affect both motor nerves and the large-fiber sensory peripheral nerves, which tell our brain where we are in space,” he said. “This can cause problems with balance. Some forms of CMT affect only motor nerves, others affect primarily sensory nerves, and why that difference exists isn’t completely understood yet.”

Interventions

Although CMT isn’t curable, interventions such as physical therapy, exercise, and orthotic device use can ameliorate its effects.

“We recommend stretching and moderate-intensity exercise for CMT patients,” said Katy Eichinger, DPT, CS, who practices in the neuromuscular division of the Department of Neurology at the University of Rochester in New York. “Patients should be evaluated by a physical therapist, who can guide them to a program that’s best for them. Assessing a person’s strengths, weaknesses, age, disease progression, and personal desires can make an individualized program much more successful.”

Photo by Joshua Albanese (joshuaalbanese.com)

In terms of stretching, Eichinger emphasizes the ankle plantar flexors (a calf stretch, facing the wall with the leg extended backward and the heel on the ground, is a typical approach). For strengthening, she works with patients’ likes and dislikes to find something they can stick with.

“You can optimize patients’ functional ability and make sure no weakness occurs due to disuse or aging,” she explained. “If someone doesn’t like gyms, they may be willing to do aquatic exercises or ride an exercise bike at home.”

Because CMT patients have distal weakness, physical therapy should focus exercise programs more proximally, according to Eichinger. This means an emphasis on the quadriceps, the hip muscles, and the core. It also may mean balance training, particular in those whose neuropathy forces them to rely more on their vision and vestibular systems to maintain an even keel.

Where we go from here

With improved genetic sequencing techniques and increased understanding of proteins and myelination, researchers are moving closer to finding a cure for CMT. Today, the best treatments for CMT include regular exercise, maintaining a healthy body weight, and, when necessary, the use of orthotic devices. Approaches to orthotic management are discussed in detail in the article that follows.

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

Once pain and inflammation have been addressed, clinicians can implement interventions—including orthotic devices, stretch­ing, and strengthening—to address the biomechanical factors that are believed to contribute to heel pain and other symptoms in this population.

By Erin Boutwell

Sever disease, also known as calcaneal apophysitis, is an overuse injury commonly diagnosed in children, particularly those active in sports such as running¹ and soccer.^1,2 The primary complaint of Sever disease is heel pain associated with repetitive microtrauma of the calcaneal apophysis. The age range of the children affected is typically 7 to 15 years, corresponding to the time period that starts when the calcaneal growth center appears and ends when it fuses.^1,3,4

Sever disease is considered self-limiting, meaning the disease will resolve itself over time (through fusion of the growth center), but that can take years. Because most patients respond to treatment within three to six weeks and are able to return fully to their normal activities,⁵ experts said early intervention is warranted.

“If you have a child who’s in pain for two years of a twelve-year period, that’s a substantial amount of time, so you do want to jump on it and treat it,” said Alicia James, BPod, MHealth Sci, director of the Kingston Foot Clinic in Cheltenham, Australia, who is currently studying interventions in children with Sever disease as a PhD candidate at Monash University in Melbourne, Australia.³

Approximately 2% to 16% of the musculoskeletal complaints reported in children are attributed to Sever disease, but these data come primarily from sports medicine clinics and therefore are not necessarily representative of the incidence within the general population.^1,6

Possible mechanisms

It is widely accepted that Sever disease is the result of microtrauma sustained during repetitive loading. Beyond that, there is no clinical consensus on what causes this microtrauma.

There are two main schools of thought on the biomechanics behind Sever disease. One theory is that the calcaneal apophysis is placed under traction during this period of rapid growth as the Achilles tendon creates a shear force upward on the heel; the plantar fascia may contribute to this traction by pulling in the opposite direction.^3,7 A second potential explanation for the symptoms of Sever disease is damage caused by the repetitive impact forces during heel strike, particularly during high-impact activities.²

Not all Sever patients present with symptoms beyond heel pain. However, some children with Sever disease also present with tight heel cords,⁸ limited ankle dorsiflexion,^1,8 or an underlying biomechanical malalignment.^1,8

A prospective study in which investigators compared a group of Sever patients to a control group demonstrated little evidence of these factors, however. The one exception was a significant difference in forefoot position between groups, providing evidence in support of biomechanical malalignment within the Sever disease population.⁹

“Excessive pronation unlocks the foot, making it a mobile adaptor rather than a rigid lever,” said study author Rolf Scharfbillig, PhD, a podiatrist and lecturer at the University of South Australia in Adelaide. “The calf muscles have to work harder to achieve heel lift as [a mobile adaptor] is less mechanically efficient.”

Diagnosis

The most common clinical diagnostic technique is known as the squeeze test, in which medial-lateral compression is applied to the injured heel in an effort to reproduce the pain. Other techniques include tests for the presence of pain when the patient balances on the affected leg¹⁰ or when they do a calf raise.¹¹ However, the squeeze test remains the gold standard for diagnosis.

Radiography has also been suggested as another possible way to diagnose Sever disease, but clinical consensus is that radio­graphy is not necessary for diagnosis. Nevertheless, radiographs may play an important role in ruling out other possible causes of heel pain.^7,8,12 In a 2011 study by Rachel et al, approximately 5% of children diagnosed with Sever disease had abnormal radiographic findings.¹²

Identification of such radiographic abnormalities could result in an amended treatment plan, said study coauthor Derek Kelly, MD, a pediatric orthopedic surgeon at the Campbell Clinic in Germantown, TN.

“Based on the sample used in that article, we recommend a single lateral x-ray of the calcaneus as part of the initial work up for children presenting with a chief complaint of heel pain,” Kelly said.

Treatment options

The first objective of any Sever disease treatment regimen is to minimize inflammation and control the child’s pain. This is typically accomplished by modified rest, ice, and anti-inflammatory medications.⁷ Once pain and inflammation have been addressed, however, clinicians can begin to implement interventions that address the biomechanical factors associated with Sever disease.

Gabriel Gijón-Nogueron, PhD, a podiatrist and associate professor at the University of Málaga in Spain, summarized the biomechanical goals of treatment in three points: (1) increased contact area beneath the foot to minimize high-pressure areas, (2) raising the heel to reduce the tension on the Achilles tendon, and (3) correcting any biomechanical malalignments such as overpronation. The emphasis on foot posture stems from published reports of pronation in up to 18% of Sever disease patients,¹³ and clinical estimates that are even higher.

“If the foot is very pronated, it’s possible to see [it]—this movement [causes] tension on the fascia or the Achilles tendon,” Gijón-Nogueron said. He estimated that almost 50% of the patients he sees with Sever disease demonstrate overpronation.

The two main strategies clinicians use to achieve their bio­mechanical goals involve stretching and strengthening programs or in-shoe orthotic devices.

Stretching and strengthening

Stretching and strengthening regimens may include either static or active stretching exercises. They are typically designed to increase ankle dorsiflexion range of motion, stretch out the gastrocnemius and soleus muscles, and reduce strain on the Achilles tendon. Like many other aspects of Sever disease, the effectiveness of stretching programs has not been studied adequately in a controlled environment.

However, Adam Potteiger, ATC, a certified athletic trainer in the Institute for Sports Medicine at Lurie Children’s Hospital of Chicago, and colleagues are conducting a new study in this area.

“We are currently enrolling patients in a study to compare rest [symptomatic care], static stretching, or active elongation exercises as the best treatment,” Potteiger said. “Active elongation exercises involve lengthening the muscle groups by trying to minimize the stress on active growth plates.”

Scharfbillig also emphasized the importance of stretching in ­patients with Sever disease.

“If the patient displays tight hamstrings and/or calves, then these must be stretched as a matter of urgency, as otherwise I have found other treatment options will have little effect,” Scharfbillig said.

Nonetheless, some skepticism exists regarding the effectiveness of stretching routines in Sever patients. Elengard et al in their 2010 article criticized the quality of evidence on stretching programs to date, and suggested that stretching may not be necessary in Sever disease patients who do not demonstrate decreased dorsi­flexion range of motion.¹¹

Orthotic interventions

In-shoe orthotic devices are a common method of Sever disease treatment, and may be used in combination with stretching or pain management therapies. The goal of orthotic management is to reduce shear stress on the apophysis, and, if necessary, realign the foot posture to correct for overpronation.⁴

The orthotic devices may take various forms, the most common being heel cups, heel wedges, and insoles (prefabricated or custom). Each provides a slightly different method of influencing the biomechanics of the calcaneal apophysis and the surrounding musculature.

A heel cup is used to compress the heel pad beneath the foot and maintain it in a central position with respect to the rest of the foot, maximizing the thickness of the anatomical heel pad beneath the painful calcaneal apophysis.^14,15 Also, many heel cups are manufactured out of gels and other viscoelastic materials that provide the patient with additional protection from impact forces.

Jeremy Uronis, CO, an orthotist at Lurie Children’s Hospital of Chicago, said of his own clinical experience treating children with Sever disease, “I can’t think of a good reason not to dispense gel heel cups right off the bat to help reduce pain, discomfort, and possibly even inflammation.”

A heel wedge raises the position of the heel to relieve tension from an overly tight Achilles tendon.¹⁴ Alicia James said she believes these wedges (also known as raises) are effective for treatment of Sever disease in patients who do not demonstrate overpronation.

“If you’ve got a neutral foot, then what’s the point of giving them [a full-length] orthotic? A heel raise should and probably would work quite nicely,” James said.

Orthotic insoles can provide much of the same function as heel wedges, but may also be used to correct pronation.¹⁴ Gijón-Nogueron provides more than 400 pairs of insoles per year in his clinical practice, and contends that, in his experience, insoles are an extraordinarily effective treatment method for Sever disease, working in just three to four weeks.

Given that each type of orthotic treatment has its supporters, it can be a challenge for practitioners to determine which one is best for each patient. There have been few well-controlled research studies comparing the relative effectiveness of treatments; consequently, there is no current gold standard for orthotic management of Sever disease.¹

Insoles fabricated for a boy with Sever disease. (Photos courtesy of Gabriel Gijón-Nogueron, PhD.)

Investigators have conducted only one comparative study to date: Perhamre et al reported significantly greater pain reduction with a heel cup than with a heel wedge during sports activity, but the study design was a crossover analysis in which children were able to choose which orthotic device they preferred.¹⁴

“Most children prefer the heel cup, but some children choose the heel wedge, and it would be very interesting to know why,” said study coauthor Maria Klässbo, PhD, a physical therapist and researcher at Landstinget i Värmland in Sweden.

Klässbo said she suspects biomechanical malalignment or shoe type might influence children’s preferences.

Practitioners’ opinions about which approach is most successful are incredibly varied, something that has an interesting potential interpretation: Perhaps all of the orthotic interventions are effective at relieving heel pain.

Heel cups, wedges, and insoles are typically fabricated out of viscoelastic materials such as foams, gels, and plastics. All of these materials are able to absorb shock beneath the foot. If impact forces play a larger role in the development of Sever disease than traction of the calcaneus, the addition of any shock absorber beneath the calcaneus might prove to be an effective treatment option.

“It really comes back to that old debate: Is it an impact force, or is it tractional? Yes, there is a tractional component of it,” James said, “but I think the impact is what we’re perhaps not addressing enough.”

Prevention

Given that the mechanism behind Sever disease is unclear,¹⁶ identifying children at risk for the condition in the hopes of an early intervention is next to impossible. Another challenge in predicting which children are at risk is that clinicians typically don’t have the opportunity to assess a child’s biomechanics prior to injury.

“It’s most likely not until the patient’s heel starts hurting that they seek treatment,” Uronis said.

Scharfbillig said his prospective study⁹ suggests that probable risk factors for Sever disease include forefoot or rearfoot varus, flexible forefoot, footwear with inappropriate support, and activity level.

Obesity has also been suggested as a risk factor for developing Sever disease.³ However, Scharfbillig’s study found no difference in the incidence of Sever disease between overweight and normal-weight children.⁹

Generally speaking, variables that contribute to overuse injuries—including poor sports technique, old or worn-out footwear, inadequate recovery time between training sessions, and a change in the characteristics of the playing surface—may also play a role in Sever disease.⁸

Going forward

Those who have studied the Sever disease literature agree on one thing: More research is required before a best-practice treatment option can be decided. The results of the first randomized
controlled trial comparing Sever disease treatments (the protocol for which was published in 2010)³ should be published this year, and James has hinted that the results might have a big influence on the typical treatment process.

“You almost have this script of how you’re going to treat something,” James said. “It [the study finding] really does teach me to go back and assess the child and to provide the treatment based on what you see in the child rather than the script.”

Determining which patients will respond to an insole versus a heel wedge is another important area of study, and the deciding factor may be foot alignment (eg, pronation).¹⁴ Technological advancements related to methods of quantifying foot posture and alignment should help researchers and clinicians address these issues objectively.⁹

Like James, Scharfbillig emphasized the importance of effective treatment for children with Sever disease, despite the self-limiting nature of the condition.

“This is a condition that … has a real effect on the child and should be dealt with aggressively, rather than just telling the child they will grow out of it or just stop sport,” Scharfbillig said. “Proper assessment and treatment mean this can be a minor condition, rather than a lingering one.”

Erin Boutwell is a freelance writer based in Chicago.