Ch 10- Peds: Limb Deficiencies

Question 1

What is a transverse limb deficiency?

Answer 1

No distal remaining portions

Named after the segment beyond which there is no skeletal portion

Question 2

What is a longitudinal limb deficiency?

Answer 2

Have distal portions

Name the bones that are affected

Question 3

What is Acheiria?

Answer 3

Missing hand or foot

Question 4

What is Adactyly?

Answer 4

Absent metacarpal or metatarsal

Question 5

What is Amelia?

Answer 5

Absence of a limb

Question 6

What is Aphalangia?.

Answer 6

Absent finger or toe

Question 7

What is Hemimelia?

Answer 7

Absence of half a limb

Question 8

What is Meromelia?

Answer 8

Partial absence of a limb

Question 9

What is Phocomelia?

Answer 9

Flipper-like appendage attached to the trunk

Question 10

What are associated with limb deficiencies?

Answer 10

Craniofacial anomalies

Question 11

What is TAR syndrome?

Answer 11

Thrombocytopenia with Absence of Radius

Question 12

What is Faconi’s syndrome?

Answer 12

Anemia and leukopenia developing at 5 to 6 years of age

Upper extremity deficiency

Question 13

What is Holt-Oram Syndrome?

Answer 13

Congenital heart disease, especially atrial septal defects and tetralogy of Fallot
Upper extremity deficiency

Question 14

What is Baller-Gerold Syndrome?

Answer 14

Craniosynostosis

Upper extremity deficiency

Question 15

What is VACTERL (or VATER) Syndrome?

Answer 15

Vertebral defects
Anal atresia 
Cardiac defects 
Tracheo Esophageal fistula 
Renal dysplasia 
Limb deficiency

Question 16

What is the most common congenital limb deficiency?

Answer 16

Left terminal transradial deficiency

Question 17

What should Prosthetic fitting should follow?

Answer 17

Attainment of normal developmental milestones, with the first fitting for a unilateral deficiency occurring when the child achieves sitting balance at around 6 to 7 months

Question 18

What should the first upper extremity Prosthetic a child has have?

Answer 18

Passive mitt in which the infant can practice placing objects

Question 19

When should a more sophisticated prosthesis and terminal device be provided?

Answer 19

~ 11-13 months when the child begins to walk, performs simple grasp and release activities, and has an attention span >5 minutes

Question 20

What is a Krukenberg procedure?

Answer 20

Reconstructs the forearm and creates a sensate prehensile surface for children with absent hands by separating the ulna and the radius in the forearm

Question 21

What is a Vilkke procedure?

Answer 21

Attaches a toe to the residual limb

Question 22

What is the most common congenital lower limb deficiency?

Answer 22

Fibular longitudinal deficiency (fibula hemimelia)

25% Bilateral

Question 23

What are Partial proximal femoral focal deficiency (PFFD) associated with?

Answer 23

70-80% associated with fibular deficiencies

Question 24

What is a Van Ness Rotation?

Answer 24

Simulation of below-knee function by rotating the foot by 180° so ankle motion can control the prosthesis

Question 25

When should a lower limb-deficient child be fit with a prosthesis?

Answer 25

When ready to pull up to standing position at 9 to 10 months

Question 26

What type of prosthesis should a lower limb-deficient child be fit with for the first prosthesis?

Answer 26

Jointless, above-the-knee prosthesis to the toddler

Question 27

When is a heel-to-gait established?

Answer 27

~2 years

Question 28

When is a Prosthetic heel-strike to toe-off gait established?

Answer 28

~5 years or when the child can demonstrate sustained one-legged standing

Question 29

When is a knee joint added to a prosthesis?

Answer 29

18 months of age

Question 30

When is a suction socket prescribed?

Answer 30

Child can assist in donning a prosthesis, at about 5 years of age

Question 31

What is the most common complication after amputation in the immature child?

Answer 31

Terminal overgrowth at the transected end of a long bone

Question 32

Where is Terminal overgrowth at the transected end of a long bone most common?

Answer 32

Humerus, fibula, tibia, and femur, in that order.

Question 33

When are motorized WC used in limb deficiency?

Answer 33

5 to 6 years old, although, in exceptional cases, children as young as 20 months

Question 34

Describe phantom limb sensation/pain in congenital limb deficiency.

Answer 34

Congenital limb-deficient children do not develop phantom sensation or pain even after conversion to surgical amputation of the limb