First Part of the Long Journey

First Part of the Long Journey
© 2010
Bryce Kroll

With the birth of any baby, one of the anecdotal phrases commonly heard is “Are all the fingers and toes there?” followed by giggles. To the shock and dismay of many parents not only are there missing toes, but whole limbs may be deformed sending the families into a state of confusion. When things are not right, the obstetrician and/or the pediatrician refer the parents to a pediatric orthopedist, who will proceed with caution. At Shriners Hospitals for Children Tampa we have a clinical team approach to ease the family through the transition of dealing with physical challenges, especially amputations. The team is led by the orthopedist, but usually the nurse practitioner initially sees the child with the family and calls in reinforcements including the care coordinator, the physical therapist and the prosthetist. The role that the prosthetist plays in the first leg of a long journey is an essential ingredient for this team so tangible solutions to the situation can be presented. Of course this approach is not always the case, but perhaps the tools and background presented here can assist in enabling success in the prosthetic fitting of a toddler’s first leg.

The family

The core of this team that ties it all together is the patient represented by the family. Who shows up at the initial visit often reflects the support system the child will have throughout his/her life and gives clues to the clinicians to the potential success of treatment. How much research has the family already done or not done also suggests a level of acceptance to the child’s deficiency and your level of communication to explain the treatment. Siblings to the child usually offer an advantage of encouraging activity on par with normal development; however the “perfect twin” can precipitate rivalry that may require attention.

Emotional baggage may come with families and often is exhibited in levels of guilt, sadness, despair, blame, denial, anger or confusion. Reading these emotions and tempering your explanations and even timeliness of the agenda to proceed need to be considered. Referrals for counseling or support groups may be appropriate. Meeting other families with similar situations can be extremely helpful. The practitioners can allay the fears and anxieties felt by the family members with assurances of hope for success.

The age of the child when first seen is also critical. If it is an infant, then time to digest the surgical and non-surgical options is a benefit. Sometimes clinical presentation is delayed until the milestone of first steps is imminent, and pulling to stand or cruising the furniture is already difficult. Parents then are anxious to know what exactly is wrong, and what the solution will be.


Congenital absences or the resultant amputation secondary to skeletal longitudinal deficiencies of the lower extremity are the leading causes for the infantile amputee population, according to Prosthetic Management of Children with Limb Deficiencies, an article by lead author David E. Krebs and other researchers. The pediatric orthopedist will determine the diagnosis with radiographs and review treatment options with the family. Amniotic band syndrome that has resulted in a complete absence at any level is straight forward and the prosthetist is called in right away to “solve the problem.” Longitudinal deficiencies of the fibula or tibia typically create deformities that cannot be salvaged through surgery, leaving the limb so unstable that amputation is the obvious choice. Fibular longitudinal deficiency customarily receives a symes amputation that can be fit with a symes transtibial prosthesis. Tibial deficiency can have the knee salvaged in some cases if there is enough ligamentous stability and residual bone to make it a transtibial amputation. More often a knee disarticulation is necessary.

Proximal Femoral Focal Deficiency (PFFD) is probably the most difficult diagnosis to explain and for the family to accept. PFFD has four levels of severity: Type A being the least affected to Type D, the most involved. It is characterized by a shortened femur with an underdeveloped proximal femur and acetabulum. The tibia can be of normal length, but often has a fibular deficiency as well presenting a deformed foot. If the tibia and fibula are normal, then clinically the foot can be perfectly normal, but the limb-length discrepancy due to the shortened femur will be significant. Families struggle with the decision of amputation especially in the latter case. All treatment options should be discussed so an informed decision can be made, and the family feels comfortable with their decision.

The decision

Choosing not to amputate, especially with a normal foot, may start out as a simple lift, but eventually necessitates the design of a foot-on-foot prosthesis. This incorporates orthotic techniques to accommodate the leg and make up the difference in length with a prosthesis, which is never cosmetic.

Another option is to have a symes amputation followed by the production of a modified transfemoral type socket that contains the anatomical knee, and is attached to a mechanical knee above the foot when space allows. Congruency of the knee height to the sound side is a constant challenge. If the femur is long enough and the knee is stable, then a transtibial socket design is an option, but the trade off is that knee heights will not be equal.

The final surgical solution that is not offered by every surgeon is to have the Van Ness procedure where an osteotomy is performed to allow the foot and ankle to be rotated 180· and act as the knee. The unusual cosmetics of this deters it from being a common choice, however the natural mechanics create an efficient gait pattern.

Visual examples of all of the options through photos or actual prostheses should be shown to the family during the initial clinic visit. Establishment of a realistic timeline of the process from amputation through the prosthetic delivery and gait training with physical therapy should be set forth, so the family can plan their life around it. All of this requires a true commitment from the team to encourage an open dialogue with the family so when their decision is final, they are confident that their child’s best interest is assured. It is difficult for professionals to realign the balance of what may seem to be a straightforward decision based on professional experience, and transfer ourselves into the shoes of the parents trying to make this difficult decision for their child. Compassionate understanding and respect for their decision is a necessary step in the whole picture.

The prosthesis

Casting a kicking, screaming, Houdini-like toddler is the first challenge. Assuming the cast, which loosely resembles the leg, can be converted into a well-fitting socket, then the height issue is tackled.

For a transtibial symes, the space for a foot may not be adequate and a temporary small lift on the sound side may be necessary.

A transfemoral socket may not have the necessary space for a knee component and foot requiring either to have labor intensive outside hinges that pinch, or a straight leg. This dilemma creates difficulty in the crawling-to-stand transitions, since there is no knee to bend.

If a knee component can fit in, the resistance of flexion/extension poses the problem of the knee getting stuck in those positions and the parent or child must learn to physically release it. If this isn’t frustrating enough, the suspension of the leg is a constant challenge so the child will crawl right out of it and be delighted with that accomplishment.

Using custom knee sleeves or converted elbow sleeves can work if there is enough thigh to grip. If not, a holster-like waist belt with an extended sleeve can be made to suspend a transtibial prosthesis. For the transfemoral-type socket the suspension is slightly easier using a Silesian belt or Tes belt, and then the challenge is to control the rotation during all of the transitioning. Even with all of the obstacles involved, using a knee when at all possible is the choice of preference.

Play to train

The parent’s involvement in the training of using the first leg is paramount. A toddler is not going to follow directions at all, but they love to play. By strategically placing toys where the child must reach for them and shift weight onto the prosthesis, will build confidence that the leg will not fail. Finding push toys that act as walkers develops gait and lends stability until independent walking is achieved. Going from sitting to standing in some interactive game strengthens the muscles that are necessary for proper ambulation and control of the prosthesis. Essentially, the parents are the ones in training and will act as the full-time therapist at home. The prosthetist and the physical therapist need to act as a team to prepare the parents for this lifetime role.

There are few things in life as satisfying as seeing your child take his or her first steps. The prosthetist becomes a key player in accomplishing this milestone for the parents of a child whose life has been compromised by a fluke of nature. It is a huge responsibility for the practitioner and the team surrounding this situation to make it not only possible, but a good experience for a difficult time in the family’s life. The prosthetist will continue to be a constant ally to the family to ensure relative normality in the growth of their child.

For more information:

  • Krebs DE, Edelstein JE, Thornby MA. Prosthetic management of children with limb deficiencies. Physical Therapy. 1991; 71: 23.
Janet G. Marshall

Janet G. Marshall, CPO/LPO is a pediatric specialist at
Shriner’s Hospital in Tampa, Fla. She is the vice president of the
Association of Children’s Prosthetic-Orthotic Clinics and is a member of
the O&P Business News Practitioner Advisory Council.

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