S3_L4: Pediatric Limb Deficiency Flashcards
Intramembranous ossification is the process of forming _____ by direct differentiation of mesenchymal cells into bone.
osteoblasts
Endochondral ossification is the process where mesenchymal cells undergo ____ to form cartilage that matures to become bone.
chondrogenesis
Most commonly occurring type of bone formation or ossification
Endochondral ossification
This period in pregnancy is crucial for the genesis of limb production
first trimester
Mesodermal formation of the limb occurs at ____ days of gestation and continues with differentiation until 8 weeks of gestation
26
A disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, shoulder, arm, and hand
Poland Syndrome
Note: This syndrome is an example of a vascular malformation.
A rare condition caused by strands of the amniotic sac that separate and entangle digits, limbs, or other parts of the fetus
Amniotic Band Syndrome
Note: This syndrome is an example of a vascular disruption.
Upper vs Lower Extremity Prosthetic Acceptance
- Acceptance of prosthesis is variable
- Prosthesis are used more as a mechanical tool
- Generally have high acceptance rates
A. Upper Extremity
B. Lower Extremity
- A
- A
- B
The highest incidence of malignancy (tumors) occurs between what age range?
12-21 years old
Absence of a limb (No limb)
Amelia
Partial absence of a limb (incomplete limb deficiency)
Meromelia
Meromelia characterized by flipper like appendages attached to the trunk
Phocomelia
Note: Phoco means seal (hand attached to shoulder, no arm)
Meromelia characterized by absence of half a limb (e.g. half radius/ulna)
Hemimelia
Meromelia characterized by missing a hand or foot
Acheira
Meromelia characterized by absence of a metacarpal or metatarsal
Adactyly
Meromelia characterized by absence of a finger or toe
Aphalangia
Frantz-O’Rahilly describes this deficiency as complete absence distal to level of loss
Transverse terminal deficiency
Frantz-O’Rahilly describes this deficiency as unaffected parts do not occur distal to and in line with deficient portion
Terminal deficiency
Frantz-O’Rahilly describes this deficiency as the absence of central elements with foreshortening of limb
Phocomelia intercalary deficiency
Frantz-O’Rahilly describes this deficiency as segmental absence of either pre or post-axial elements; intact proximal and distal
Paraxial intercalary deficiency
Frantz-O’Rahilly describes this deficiency as complete longitudinal absence either in pre or post-axial elements
Paraxial terminal deficiency
Frantz-O’Rahilly describes this deficiency as intermediate parts are deficient; elements proximal to and distal to deficient portion are present
Intercalary deficiency
- More complications (e.g., psychological anger, resentment, rejection, etc.) and harder acceptance
- Can achieve (later) functional milestones with or without prosthetic intervention
A. Congenital limb amputation
B. Acquired amputation
- B
- A
- Difficulty with training patients
- Children with upper extremity limb deficiency adapt and compensates easily with ADLs
- Cause for amputation also affects overall health of child
A. Congenital limb amputation
B. Acquired amputation
- B
- A
- B
- Scoliosis is common especially in unilateral limb deficiency
- Few complications as the baby was born without a limb; they can adapt faster.
A. Congenital limb amputation
B. Acquired amputation
- A
- A
Timing of fitting UE prosthetics for Congenital Limb Deficiency: Myoelectric device
A. 6 months when sitting independently
B. 12-15 months when ambulatory
C. As young as 1 year old, when able to operate
D. As young as 2 years old
D. As young as 2 years old
Timing of fitting UE prosthetics for Congenital Limb Deficiency: Body Powered Prosthesis
A. 6 months when sitting independently
B. 12-15 months when ambulatory
C. As young as 1 year old, when able to operate
D. As young as 2 years old
C. As young as 1 year old, when able to operate
Note: This prosthesis uses the muscles of the UE to control movement of a terminal device
Timing of fitting UE prosthetics for Congenital Limb Deficiency: Active Terminal Device
A. 6 months when sitting independently
B. 12-15 months when ambulatory
C. As young as 1 year old, when able to operate
D. As young as 2 years old
B. 12-15 months when ambulatory
Timing of fitting UE prosthetics for Congenital Limb Deficiency: Passive Upper Extremity Device
A. 6 months when sitting independently
B. 12-15 months when ambulatory
C. As young as 1 year old, when able to operate
D. As young as 2 years old
A. 6 months when sitting independently
Upper limb prosthetic with no moving part/function. It is used for cosmesis and for support for bimanual UE activity.
A. Passive Upper Extremity Device
B. Active Terminal Device
C. Body Powered Prosthesis
D. Myoelectric powered prosthesis
A. Passive Upper Extremity Device
Upper limb prosthetic that is characterized by an active hook (prehension like pincer grasp) or hand that can be opened or closed voluntarily, but does not have a dexterity function. It is used for simple tasks or activities, and the other hand is used to control it.
A. Passive Upper Extremity Device
B. Active Terminal Device
C. Body Powered Prosthesis
D. Myoelectric powered prosthesis
B. Active Terminal Device
TRUE OR FALSE: For bilateral UE deficiency, UE prostheses are not prescribed as the child will utilize the feet in a natural manner.
True
UE prostheses are generally considered at ____ months of age
3 to 6
For the use of UE prosthesis, what 2 peripheral nerves are grafted to denervated muscles?
Median and distal radial nerves
Preferred age for initial fitting of UE prosthesis
6th month of age (time where sitting balance is achieved)
Early prosthetic fitting is designed to:
1. encourage bimanual tasks
2. establish (1)____
3. increase overall independence
4. provide (2)____
5. reduce (3)____
6. Faster acceptance if given early
- Wearing pattern
- Symmetrical crawling
- Stump dependence
Note: Use the prosthesis for transitional movements such as crawling, leaning on the prosthesis for weight bearing. A terminal device is to be used for bimanual activities (legos, stringing beads) then later concentrate on other activities.
TRUE OR FALSE: For upper extremity congenital limb deficiency, the goal of early intervention and training revolves around achieving age appropriate milestones.
True
Four phase UE amputee protocol of care:
Occurs approximately 8-16 weeks after starting rehabilitation
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
D. Phase 4: Advanced prosthetic training
Four phase UE amputee protocol of care:
Goal is to prepare the patient and the residual limb to accept a well-fitted prosthetic socket and functional prosthesis. If wounds prevent socket use, myosite testing/training for myoelectric prostheses occurs.
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
B. Phase 2: Preprosthetic training
Four phase UE amputee protocol of care:
Management consists of tolerance to be able to wear prosthesis at least 8 hours a day, integrate prosthesis use in activities, achieve independence in all ADLs, and independently don/doff the prosthesis.
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
C. Phase 3: Intermediate prosthetic training
Four phase UE amputee protocol of care:
Management consists of ROM, physical conditioning, desensitization, limb shaping (conical, not spiral), progression in ADLs, and psychologic support.
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
B. Phase 2: Preprosthetic training
Four phase UE amputee protocol of care:
Major turning point in the UE amputee’s rehabilitation. The goal is for the patient to master the mechanical actions required for prosthetic limb control.
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
C. Phase 3: Intermediate prosthetic training
Four phase UE amputee protocol of care:
Management consists of comprehensive evaluation, wound healing & edema control (with casting or elastic bandage wrapping), pain control, desensitization, scar management, and exercise for flexibility, gross motor activity, psychologic support, and basic ADLs.
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
A. Phase 1: Initial management & protective healing
TRUE OR FALSE: Community reintegration is incorporated in every stage of the four phase upper limb amputee protocol of care.
True
Four phase UE amputee protocol of care:
Highly individualized phase, the patient should use their prosthesis of choice and goal is to conserve energy, decrease biomechanical stress to the intact limb, decrease extraneous body movements, and decrease use of adaptive equipment.
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
D. Phase 4: Advanced prosthetic training
Four phase UE amputee protocol of care:
This phase begins immediately after injury and continues until all the wounds are closed and infection free, and usually lasts for 1-3 weeks.
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
A. Phase 1: Initial management & protective healing
Four phase UE amputee protocol of care:
Occurs 2-3 weeks after amputation, and this phase ends with the acquisition of a prosthesis.
A. Phase 1: Initial management & protective healing
B. Phase 2: Preprosthetic training
C. Phase 3: Intermediate prosthetic training
D. Phase 4: Advanced prosthetic training
B. Phase 2: Preprosthetic training
Timing of LE prosthesis:
lightweight extension of limb
A. 6 months
B. 9-10 months
C. 2 ½ to 3 years
A. 6 months
TRUE OR FALSE: Lower limb deficiency especially above the knee may delay independent achievement of sitting & standing and/or encourage asymmetric sitting posture.
True
Note: The more proximal the limb deficiency, the more likely the delay or asymmetry
Timing of LE prosthesis:
prosthesis with weight bearing capacity to allow pull to stand; usually uses monolithic type of prosthesis
A. 6 months
B. 9-10 months
C. 2 ½ to 3 years
B. 9-10 months
Timing of LE prosthesis:
lockable knee joint; therapy includes control of knee joint
A. 6 months
B. 9-10 months
C. 2 ½ to 3 years
C. 2 ½ to 3 years
3 important characteristics of a LE prosthesis
- Simple
- Allow growth adjustment
- Lightweight to propel
Timing of fitting LE prosthetics for Congenital Limb Deficiency: Energy storing-releasing feet
A. Infancy
B. Toddler
C. 2- 3 years old
D. 3-5 years old
B. Toddler
Note: foot can be propelled/rocked using this prosthesis
Timing of fitting LE prosthetics for Congenital Limb Deficiency: Knee joint below the knee
A. Infancy
B. Toddler
C. 2- 3 years old
D. 3-5 years old
C. 2- 3 years old
TRUE OR FALSE: Goal of early fitting of LE prosthesis is to allow normal two legged standing and provide a means for reciprocating gait development; provide normal appearance.
True
Timing of fitting LE prosthetics for Congenital Limb Deficiency: Solid-ankle cushion-heel (SACH) [passive terminal device]
A. Infancy
B. Toddler
C. 2- 3 years old
D. 3-5 years old
A. Infancy
Timing of fitting LE prosthetics for Congenital Limb Deficiency: Knee joint above the knee for transfemoral amputation
A. Infancy
B. Toddler
C. 2- 3 years old
D. 3-5 years old
D. 3-5 years old
Training and Timing for LE
- Stand and ambulate with wide base
- Stand for several seconds on 1 foot
A. 20 months
B. Toddler
C. 2 years old
D. 3 years old
E. 4 years old
F. 5 years old
- B
- E
Training and Timing for LE
- Standing on one foot with help
- Stand on one foot longer
A. 20 months
B. Toddler
C. 2 years old
D. 3 years old
E. 4 years old
F. 5 years old
- A
- F
Due to the problem of growth, normally LE prostheses are changed annually until (1)___ years, every 2 years between (2)___ and ___ years, then every 3 to 4 years until adulthood.
- 5
- 5 and 12
Fitting timetable of congenital LE limb deficiency is around ___ months, when the child is ready to pull up to a standing position.
9-10
Training and Timing for LE
- Can stand on one foot momentarily
- Heel to stride length develops
A. 20 months
B. Toddler
C. 2 years old
D. 3 years old
E. 4 years old
F. 5 years old
- D
- C
Training and Timing for LE prosthesis
- Proper positioning
- Play is the primary motivation for desired movements and activities
- Goal is to develop normal pattern of gait including stride and step length and velocity
- Dwell on phantom pain
A. Preprosthetic period
B. Post-amputation period
- B
- B
- A
- B
TRUE OR FALSE: Normally, arm span is almost equal to standing height
True
By (1)___ years old, birth height usually doubles and child is approximately 60% of adult height. The child is approximately 80% of final height at (2)___ years old.
- 5
- 9
Therapy and Training for LE prosthesis
- Instruct on how to care for prosthesis
- Focus on addressing information needs of parents
- Edema control
- Evaluate good leg for strength (specific exercises done to achieve strength)
A. Preprosthetic period
B. Post-amputation period
- B
- A
- B
- B
During puberty, the standing height increases by approximately ___ cm/month
1
Note: Rate of height and growth increase is not constant and varies with growth spurts.
Limb development occurs between ___ to ___ weeks after fertilization.
4 to 8
Note: Most limb defects occur during 4 – 6 weeks.
The centers of ossification (endochondral ossification) were derived from ___.
Mesoderm