Paediatric Limb Deficiency

Children are not little adults.

Although there are some areas of general overlap, the management of limb loss in children differs substantially from that in adults. Key differences in rehabilitation programs include [1]:

1. High rates of congenital limb defects and acquired amputations
2. Classification of Congenital Limb Defects
3. An immature skeleton
4. Adjustment
5. Habilitation versus rehabilitation
6. Growth and development considerations
7. Pediatric Differences When Amputation

Congenital versus acquired loss:

Even with nomenclature, children and adults describe limb loss differently. In pediatrics, the terms “amputee” or “amputation” are replaced by limb differences or limb defects, since most children with limb loss are born this way (i.e. congenital) and Reduction or loss of part of a limb from normal size [2]. The ratio of congenital to acquired limb defects varies in different studies, but congenital defects are always the main cause [3]. A 2021 article describing lower extremity prevalence Congenital loss was cited as the cause in 84 percent of childhood losses in the United States [4].

There are two main reasons for the proportion of children suffering acquired losses [5]:

1. Trauma (eg lawn mower (29%) agricultural machinery (24%) motor vehicle accident (16%) etc.).
2. Among other causes of acquired limb loss, malignancy and infection are the leading causes of deficits.

Classification of Congenital Limb Defects:

Congenital limb defects are those born without some or all of a limb. Classification/description of these deficits is more challenging than simply stating the level of transfemoral or transtibial amputation of the lower extremity. Various limb classification systems have been used Over the years, these include Frantz and O’Rahilly (1961)[6]; McCready (1974)[7]; and Swanson (1976)[8]. The current and accepted form of classification used internationally since 1998 is the ISPO classification system [9]. This form of classification utilizes anatomical and Describe radiographic evidence of limb deficits.

It states whether the defect is transverse or longitudinal, and which anatomical structures are partially or completely missing. Although the ISPO classification is still used internationally, further research into classification options is ongoing. Classification The system published in the American Journal of Medical Genetics in 2011 included etiological and causative factors as well as anatomical classification and was developed specifically for the classification of congenital limb defects [10].

Management of Pediatric Prosthetics

Management of pediatric limb deficits requires longitudinal observation [1]. This is to take into account the many developmental stages that children go through during their growth spurt from 0-18 years, and the need for education and prospective guidance from families.

Unlike adult prosthetic prescriptions, children can experience a number of prosthetic changes as they grow, eg. Stumps extend prosthetics with feet to monoaxial knee units to polycentric knee units, etc.

In addition to managing prosthetic changes to match growth and cognitive development, there are skeletal immaturity factors that need to be monitored throughout childhood (ie, growth spurts, terminal overgrowth, differences in limb length, etc.).

Possible reasons for review by Pediatric Limb Impairment Services [1]:

1. Prosthetic provision
   • Temporary Prosthetic Program for Assessing Prosthetic Proficiency
   • Definitive prosthetic prescription
   • Prosthetic review (such as additions or changes to prosthetic components)
2. Prosthetic training
   • Initial prosthetic (interim program)
   • Specific tasks (e.g. upper extremity deficits)
   • Specific components eg. Myoelectric training
   • Developmental training (e.g. walking, running, etc.)
   • Recreational prosthetics (such as musical talent)
3. Developmental reviews
   • Key stages of development (e.g. 0-1 year preschool growth spurt, etc.)
4. Assistive and adaptive devices (especially for children with upper extremity deficits)
5. Psychosocial support
   • Antenatal counseling
   • Pre amputation counseling
   • Issues of bullying, body image, etc.
   • Peer support
6. Referral for genetic counseling
7. Pain management
8. Orthopaedic referral / collaboration
   • Pre amputation planning
   • Timing of epiphysiodesis
   • Evaluation and management of terminal overgrowth
   • Conversion amputation
   • Asymmetric deformity progression
   • Considerations for limb lengthening
9. Remnant care (e.g. skin breakdown/wound management)
10. Pain management
11. Prospective instruction (e.g. medical prosthetics psychosocial recreation, etc.).

Skeletal Growth and Amputations:

As is evident in pediatrics, we are dealing with an immature skeletal system, so growth-related issues are paramount. From a simple prosthetic point of view, children need more frequent prosthetic replacements. In the adult population, prosthetics may be replaced every third person years, and in children, it is more common each year, and is more common in early years and during the adolescent growth spurt.

With rapid changes in economic growth, there are also biomechanical and orthopedic challenges that need to be predicted and managed throughout the development process.

The issue of congenital limb defects with respect to limb reconstruction and/or limb lengthening is a required discussion of various limb defects, eg. Focal defect of proximal femur; congenitally short femur Longitudinal insufficiency of the fibula.

Many children may choose the path of limb reconstruction rather than amputation (commonly known as conversion amputation). However, if the limb reconstruction pathway does not achieve the desired goals of the child’s family and treatment team, a switch to conversion amputation may be made later in childhood.

The appropriateness and timing of conversion amputations is another area of collaboration between the orthopedic and rehabilitation teams.

Joint preservation remains a common objection when amputation is considered. However, due to the immaturity of the skeletal system, problems such as terminal overgrowth and growth center maintenance arise. In pediatrics, amputation is preferably performed through the joint (disarticulations) rather than through the backbone. This principle may lead to less cosmetic results (i.e. bulbous shape of the distal stump), but preservation of growth centers and avoidance of terminal overgrowth problems means better biomechanical results and less in the future Surgical intervention throughout childhood. [11]

If amputation occurs through the diaphysis (e.g. trauma), terminal overgrowth becomes a problem that needs to be carefully monitored until skeletal maturity [12].

The theory behind the eventual overgrowth seems to be that the severed bone is trying to form a crust (like after a fracture). This overgrowth tapers and causes pain in the distal stump. Clinically identifiable by tapered shape of distal stump; bursa usually palpable most distal spots, and if severe, the area will feel warm and red. X-rays are usually ordered to confirm terminal overgrowth. [12][13]

Adjustment

Congenital Limb Deficiency

• In the pediatric population, adjustments to children’s body image are virtually impossible since most children are born with limb deficits. However, for those with amputations (whether through conversion amputations or traumatic amputations), adjustment issues do have to be Solved[14]
• While children may not go through a period of adjustment, parents and extended family members certainly will, so supporting these family members in the early stages is crucial. [15]
• Ideally, if a limb defect is found on a prenatal scan, an early referral helps the clinic team meet with the family before the birth to help address concerns and questions that parents and extended family may have. [16]
• It is also recommended to have follow-up examinations as soon as possible after birth to give the family more opportunities to discuss concerns and questions. [15]

Congenital limb defect undergoing conversion amputation

• In the event of a planned amputation (i.e., conversion amputation), pre-amputation counseling is undertaken so that the child and family can adjust to the upcoming change. This is often done through the use of therapy dolls and play therapy to illustrate the extent and use of amputations Prosthetics in the future of life. [14]
• The timing and content of pre-amputation counseling will vary for each child and family. It may be beneficial to meet another child and family who have gone through the same or a similar process. It helps children see what life might look like after enduring their impending surgery. [13]
• Pre-amputation counseling also includes clear guidance on the various stages the child and family will go through (i.e. pre-amputation planning surgery admission post-healing stump dressing pre-casting prosthetic fitting prosthetic rehabilitation and where appropriate) participation in recreational and leisure activities). [12]
• Postoperative medical (pain relief), physical (desensitization to prevent early mobilization of contractures, etc.) and psychosocial support are used to continue addressing issues surrounding adjustment. [16]

Traumatic Amputation

• In the event of a traumatic amputation, there is usually no time for a preoperative consultation.
• Key factors that facilitate adjustment often include timely support and re-instruction for all phases of the postoperative period.
• In the postoperative phase, a team approach was used to address pain caused by surgical hallucinations and phantom pain. In children, phantom pain is usually not a long-standing problem, but is more likely in trauma cases (and long-standing tumors) Pain.
• In trauma cases, a greater emphasis on postoperative medical (pain relief) physical (desensitization contractures prevention of early mobilization, etc.) and psychosocial support is needed to help with adjustment due to the lack of minimal input that can be provided in the pre-amputation phase. [1]

For children and families, many childhood milestones can lead to reoccurrences of grief and adjustment. Anticipation guidance is important as your child moves into daycare, first year sports and recreational activities, and more. [12]

Focusing on family adjustments is key, as children often reflect their adjustments to limb deficits by significant people in their lives (parents, grandparents, older siblings). [12]

“Rehabilitation” and Rehabilitation

Pediatric physical rehabilitation is often more properly described as “adaptation training” because many of the skills taught are newly acquired rather than reacquired as in adult rehabilitation. [17]

Decisions surrounding prosthetic prescription and rehabilitation are often influenced by a variety of factors. Initial prescription may revolve around physical development (eg, “fit to sit” for upper limb deficits; “stand up” for lower limb deficiencies), but Composition and healing methods are often influenced by cognitive development. [18]

For children receiving a first limb, prosthetics are seen as tools that can aid in play (eg, being able to stand and cruise to reach higher toys, etc.). Families are often advised to leave the limb in the toy box when not in use so that the child develops positive Association with limbs. [12]

For children who acquire lower extremity deficits in the developmental sequence preceding upright mobility, the problem with physical rehabilitation is often that the child “gets up,” so input at this stage requires a good understanding of development A mature gait pattern is not immediately sought. Gait improvements can be made as the child matures, but again, treatment is tailored to their level of development.

The primary role of pediatric physical rehabilitation is not so much rehabilitation as providing anticipatory guidance to help children and families address upcoming developmental milestones, eg. Growth spurt Started going to school Participating in sports and recreation etc[11]

Pediatric Differences When Amputation

Preoperative Phase

• Where possible (planned amputation), children and families require preoperative counseling with a developmentally appropriate approach, as described in the Adjustments section. It involves not only children, but also parents, siblings, grandparents and other significant family members unit.
• All members of the team are usually involved in this phase
  • Medical (appropriate information and guidance on pain management, etc.)
  • Social Worker (preamputation counseling)
  • Occupational therapist (consult play therapy home modification and/or equipment planning prior to amputation).
  • Physiotherapist (before amputation consult body preparation equipment planning guidance throughout all stages)

Pain management includes preoperative initiation of medications that may limit postoperative phantom limb pain, eg. Gabapentin[17]

Surgical Principles

As noted in the “Skeletal growth and amputation” section, the principles guiding the level of amputation differ from those in adults: [18]

• Preservation of the growth center is a key principle both to limit the differences between the extremities and to maximize the mechanical advantage of the child as it grows.
• As in adults, preservation of the joint is a key principle, but in pediatrics it goes a step further and develops procedures such as rotationplasty, in which the ankle joint can replace the knee joint.
• In cases where the joint cannot be preserved, amputation is best performed through the joint rather than the backbone. The main reason for this surgical principle is that it preserves the growth center and prevents the risk of terminal bone overgrowth. In the adult population, through-diaphysis amputation is It is favored for its aesthetic appearance and prosthetic socket design.

Post Operatively

• The pediatric population rarely has the list of comorbidities that adults have, so postoperative issues surrounding wound healing are more related to the child’s activity than inactivity.
• Children recover relatively quickly and are discharged from the hospital within a few days to a week.
• Faster healing also allows them to get through the post-operative phase sooner, and it is possible to remove sutures and start shaping the stump as soon as 10 days post-op.
• Unlike the adult crowd, where shrink socks seem to be easier to use, these socks rarely fit or come in child sizes. Often due to the uniqueness of the anatomy of the residual limb (ie, congenital limb defects), residual limb dressings are used during the prenatal stage. [19]

Prosthetic Provision

• Prosthetic fitting can be performed as early as 3 weeks after surgery if no complications are encountered and there is little edema of the stump.
• Pediatric prosthetic designs require height adjustability and need to be lightweight and durable.
• Prosthetic designs also reflect developmental stages, eg. Children with dislocated joints may develop short amputations (sockets with peg bases) early in life, then develop foot attachments, and then knee joints. Component size and weight often determine when a component can be used Add rather than simply measure when a child is cognitively ready to use a new component, eg. Progression from extended prosthetics to included knee joints.
• Prostheses may include different design features than adults due to rapid growth, eg. Add socket or socket thickness more modular components to accommodate height growth; growth-oriented suspension system etc. [20]

Complications

• As shown in the “Imature Skeletal System” section, the major difference in pediatrics with regard to postoperative complications is the development of terminal overgrowth. This is more common in children than in adults.
• It can recur as frequently as every two years until skeletal maturity. [1]