Congenital Anomalies/Amputations/Prosthetics Flashcards
Osteogenesis Imperfecta
genetic disorder characterized by bones that break easily, often with little or no apparent cause
Osteochonridtis dessicans
localized fragmentation of bone and overlying cartilage of the capitellum
Arthrogryposis multiplex congenita
genetic or hereditary condition which is characterized by congenital joint contractures. it is actually a number of conditions which describe varying degrees of joint contractures, absent or hypoplastic muscles, and possible internal organ involvement
Clinodactyly
finger is bent in a coronal/radioulnar plane and most often affects DIPJ due to middle phalanx hypoplasia. no intervention needed
camptodactyly
finger bent in sagittal or flexion/extension plane and most offen at PIP
Congenital Deformities that fall under “Failure of formation (arrest of development” classification
Transverse deficiencies
Phocomelia
Congenital Deformities that fall under “Failure of differentiation (separation)” classification
Thumb-clutched hand
Kirner’s deformity
Congenital Deformities that fall under “Duplication” classification
Polydactyly
Triphalangism
Congenital Deformities that fall under “Overgrowth” classification
Gigantism
Macrodactyly
Congenital Deformities that fall under “Undergrowth” classification
Brachydactyly
Hypoplasia
Congenital Deformities that fall under “Congenital Constriction band syndrome” classification
compression neuropathy
acrosyndactyly
Congenital Deformities that fall under “Generalized skeletal abnormalities” classification
Madelung’s deformity
Maffucci’s syndrome
Syndactyly
webbed fingers
What are Swanson’s classifications of congenital hand anomalies?
- Failure of formation
- Failure of differentiation (separation)
- dupication
- overgrowth
- undergrowth
- congenital constriction band syndrome
- generalized skeletal abnormalities
pediatric metabolic conditions associated with hypoplasia of the radius
thrombocytopenia absent radius syndrome (TAR)
Fanconi’s syndrome
VACTERL syndrome
Madelung’s deformity
genetic disorder
limited development of distal radius
more common in females
shortening of the radius at the wrist, resulting in ulna longer than radius
wrist may appear sublimed with prominent ulnar head
limited bilateral wrist extension and forearm supination
Kirner’s deformity
palmar and radial curving of the distal phalanx
not considered congenital anomaly alone b/c not observed until 12 yrs of age
more common in females
When is a child ready for a fitting of an UE prosthesis?
most appropriate time for fitting a first prostheseis on a congenital unilateral amputee is between 3-9 months, or at about 6 months, when the child is achieving sitting balance
what are appropriate goals in an advanced prosthetic training program?
- using the prosthesis for both basic and advanced daily tasks
- to demontrate a natural motor pattern using the prosthesis efficiently
in an above-elbow amputation, what % of the total functional task can the prosthesis be expected to perform?
in an above-elbow amputation, the sound limb is used to perform fine more prehension activities and the prosthetic terminal device (TD) is most useful for gross prehension activities and performing the stabilization aspect of a task. It is unreasonable to expect the prosthesis to assume more than 30% of the total function of a task in Bilateral UE activities
what is the most commonly used body-powered terminal device (TD)?
The Hosmer-Dorrance voluntary opening hook
“voluntary opening” means patient uses their own body power to pull on the cable and open the TD; the spring automatically closes the device
how does a “volunary closing” terminal device work?
uses the pulling action on the cable to close the TD. Allows for greater pinch stretch and graded prehension, but is more expensive and more prone to break down over time
in pediatric prosthetic program participation, how frequently should re-assessment be performed?
every 3-4 months secondary to residual limb growth and possible changes due to additional surgical interventions and/or decreased skin integrity
what are goals for pre-prosthetic training?
addressing residual limb shrinkage and shapng
maintaining or increasing ROM, increasing muscle strength and flexibility, participating in myotesting, desensitization of residual limb, proper hygiene, increasing self-reliance, maximizing independence, orienting to prosthetic options, changing dominance training
what’s a myotester?
useful in assessing signal strength of potential muscle groups for a myoelectric prosthesis. It assists in determining the ideal location for electrode placement. Visual feedback is provided with a meter or a light
what’s the advantage of a canted hook vs a mechanical hand?
canted hooks are designed to be rotated laterally for a side approachto the object, maximizing pt opportunity for visual feedback during prehension attempts. Prothetic hands usually block line of sight, making them less functional
what is the most common cause of limb deficiency?
vascular disruption (i.e. amniotic band related)
what is a hypoplastic thumb?
a digit that appears incomplete in typical development
ranges from minimal shortenng to complete absence of the thumb
what is VACTERL syndrome?
a non-randome association of birth defects affecting multiple systems of the body
V-Vertebral
A - Anal atresia
C - cardiac defects
T -Tracheal anomalies including tracheoesophageal (TE fistula
E - Esophageal atresia
R - Renal and Radial sided hand and forearm deficiencies and
L - other limb abnormalities
Cleft hand deformity
AKA “split hand” or Ectrodactyly
absence of digits or metacarpals within the central portion of the hand
Acrosyndactyly
a form of constriction band syndrome in which the fingers were once completely separated, but then a band formed around the fingers during embryonic development resulting in re-fusion of adjacent digits
Symbrachydactyly
largest group of anomalies associated with shortened fingers
the UE may be foreshortened and somewhat smaller than the opposite side, but the fingers are the most severely affected. Can be all of the digits or only the thumb
Transverse amelia
forequarter amputation
all or majority of UE is missing from the shoulder and distally
Transverse hemimelia
below-elbow amputation
all or majority of UE is missing from the elbow and distally
Longitudinal amelia
partial amputation
one forearm bone is missing; digits may or may not have formed
Phocomelia
bones missing in upper or lower arm; total or part of hand remains intact
preaxial deficiencies
thumb-sided abnormalities
postaxial deficiencies
ulnar sided abnormalities
what parameter yields the best predictive value for shoulder dysplasia in the child with obstetric brachial plexus birth palsy (OBPBP)?
deficits of passive shoulder external rotation at 3 months
what is the mechanical finger ring (MFR)?
a fully articulating bio-mechanical prosthetic finger appropriate for 24/7 use
high-impact plastic that is non-irritating and does not conduct heat, cold, or electricity
mimics bio-mechanical movement of the finger, allowing gripping, holding, throwing, etc.
in pediatric obstetric brachial plexus injury (OBPI), which muscle is used and an indicator of prognosis and as a guide for intervention?
biceps
what are the 6 different prehension patterns used by the physiologic hand?
- palmar (aka three-jaw chuck)
- hook
- cylindrical
- lateral
- tip
- spherical
what’s the most common prehension pattern used by prosthetic hands?
palmar (aka three-jaw chuck)
when is the “golden period” for prosthetic fitting
within the first 30 days of amputation
Immediate Post-Operative Prosthesis (IPOP)
an orthosis fabricated in the operating room or shortly thereafter
what motion is used to power a voluntary opening hook TD for a below-elbow (transracial) amputation?
humeral flexion
What is an active hybrid prosthesis?
combines body-powered control with myoelectric/external control
can be externally powered elbow and body-powered TD, or a body-powered elbow and externally powered TD
when using a transradial prosthesis, what body motion is used to perform activities at the midline and glenohumeral flexion?
biscapular abduction
brings the prosthesis close to the body. Biscapular abduction paired with and/or followed by GH flexion is beneficial for activities that require the prosthesis to work away from the body such as functional reaching tasks
for an above elbow prosthesis, what motions are used to lock and/or unlock the mechanical elbow device?
shoulder depression, shoulder extension, and shoulder abduction of the involved UE
in a patient with bilateral transhumeral amputation, which TD is best?
canted hook on the dominant side and lyre-shaped symmetric hook on non-dominant side
canted hook is better for fine prehension and offers better line of sight
lyre shape offers cylindrical prehension
wrist flexion devices are a necessity for participation in activities at midline, including eating, toileting, dressing
What is the best design for a transradial below-elbow myoelectric/external power prosthesis
self-suspension socket design, which eliminates any body harnessing
how far should a patient with a below-elbow amputation be able to open the TD with the elbow in full flexion and full extension?
70-100% of available TD opening
how far should a patient with an above-elbow transhumeral amputation be able to open the TD with the elbow in full flexion or full extension?
at least 50% of max available TD opening
max TD opening is at 90deg of elbow flexion
what are the different types of control systems for myoelectric devices?
two-state/two muscle: use contraction of one muscle to activate a motor in one direction and second (preferably antagonistic) muscle to operate a motor in another direction
two-state/two-muscle proportional: the speed or the prehensile force is varied with the intensity of the electromyographic signal
one-state/two-function: when controlling a prosthesis w/ only one muscle site is necessary. Rate-sensitive and level/amplitude-sensity systems exist where either a fast/slow contraction or a hard/gentle contraction determines the direction of movement
what are the deformations associated with apert hand?
- Spade hand - MOST COMMON
- Mitten hand
- Hoof hand
Spade hand
most common and least severe of apert hand deformation
thumb is visible and separate
IF, MF, and RF fused with osseous unions and form a FLAT palm
Synonychia (fusion of 2 more more nails) may be present
fingers are normal length
Mitten Hand
sometimes called spoon hand
more serious deformation
thumb separate, but not always easily seen b/c can be fused to the IF, revealing only a distal phalanx
IF, MF, RF, and SF syndactylized by osseous unions into a CONCAVE palm
Hoof Hand
aka Rosebud hand
rare and the most serious apert hand deformation
all digits have a solid osseous or cartilaginous fusion
fingers have one conjoined nail
no thumb
Maffucci’s syndrome
symptoms include enchondromas, bone deformities, as well sa dark irregular shaped hemangiomas and lesions begin to appear early in childhood
Sign/symptoms of spastic cerebral palsy
hypertonic muscles that lead to joint contractures
hypertonic wrist flexors and thumb adductor muscles typically seen
Radial club hand
aka radial dysplasia
failure of formation on radial side of UE
most common type is complete absence of the radius, forcing wrist into and angled position, which further shortens the limb
brachydatyly
shortening of the fingers
Exostosis
formation of new bone on the surface of a bone
what are the 3 forms of polydactyly?
- Preaxial polydactyly (radial) = thumb duplication
- Postaxial polydactyly (ulnar) = small finger duplication, MOST COMMON
- central polydactyly = a central digit is duplicated
