Genetics 2

Question 1

Arnold-Chiari Malformation

Answer 1

Brainstem, 4th ventricle, and part of cerebellum are pulled downward through the foramen magnum

Question 2

Arnold-Chiari Malformation
Leads to blockage of

Answer 2

CSF flow

Question 3

Arnold-Chiari Malformation
Pressure may develop on brainstem where the

Answer 3

cranial nerves are located

Question 4

Arnold-Chiari Malformation
A ventriculoperintoneal (VP) shunt provides drainage of

Answer 4

CSF from the ventricles to an extra cranial compartment

Question 5

Arnold-Chiari Malformation
Extra tubing is left in the extracranial site to

Answer 5

uncoil as the child grows

Question 6

• Risk For Pressure Ulcers with Myelomeningocele
  
  • Factors which contribute to the development of pressure ulcers:

Answer 6

• Friction
• Pressure from casts or splints
• Bony prominence
• Vascular problem
• Poor transfer skill
• Obesity
• Asymmetrical weight bearing or posture (scoliosis, orthopedic deformities)

Question 7

Bowel and Bladder controlled by

Answer 7

S2-4

Question 8

NTDs are detected prenatally by

Answer 8

Ultrasonic scanning and MRI
Serum alpha‐fetoprotein (AFP) testing

Question 9

Prenatal Diagnosis of Myelomeningocele
Amniocentesis can detect only

Answer 9

Open NTDs
Is recommended women who have previously had children with NTDs

Question 10

Prenatal diagnosis makes it possible for

Answer 10

• Planned cesarean section
• Therapeutic abortion

Question 11

Translucent:

Answer 11

meningocele

Question 12

Non-translucent:

Answer 12

myelomeningocele

Question 13

Children with spina bifida have a greater risk of becoming

Answer 13

allergic to latex

Question 14

Latex allergy symptoms

Answer 14

Watery eyes, wheezing, hives, rash, swelling, and in severe cases, anaphylaxis, a life‐threatening reaction

Question 15

Unstable Hip Dysplasia

Answer 15

Hip is positioned normally but can be dislocated by manipulation (Barlow maneuver)

Question 16

Subluxation/Incomplete Dislocation

Answer 16

Femoral head remains in contact with the acetabulum but the femoral head is partially displaced or uncovered

Question 17

Dislocation

Answer 17

femoral head totally outside the acetabulum

Question 18

Carrying the infant with hip flex, abd, ER —>

Answer 18

increase hip stability

Question 19

Most dislocations occur in the

Answer 19

perinatal period

Question 20

Breech position in utero Places the hips in a position of acute

Answer 20

flexion and adduction

Question 21

• The longer the dislocation is present, the greater the _______
  Stretching of the _____
  Flattening of the ____

Answer 21

secondary changes
hip capsule
femoral head

Question 22

Congenital Hip Dysplasia
In the newborn and non-ambulatory up to 12 months, there may be one or more positive signs

Answer 22

• Physical asymmetries in ROM (even as little as 10 degrees is considered significant, especially limitation of hip abduction)
• Asymmetry in the buttock or gluteal fold (higher on the affected side)
• Extra thigh skin folds
• Leg length discrepancy

Question 23

Uncorrected bilateral dysplasia may cause

Answer 23

duck waddle gait

Question 24

Unilateral dysplasia may cause a limp with a

Answer 24

+ve Trendelenburg sign during the stance phase of gait on the involved side

Question 25

A flexion contracture on the involved side(s), which then contributes to

Answer 25

marked lumbar lordosis

Question 26

Hip dysplasia diagnosis Barlow and Ortolani maneuvers have a

Answer 26

high false positive rate

Question 27

Hip dysplasia diagnosis The Galeazzi sign (LLD) is

Answer 27

more accurate

Question 28

Most common treatment for hip dysplasia

Answer 28

infants hip in flexion and abduction

Question 29

Hip Dysplasia, <6 months treatment

Answer 29

The use of a hip harness such as the Pavlik harness

Question 30

Hip dysplasia >6 months treatment

Answer 30

- Reduction under anesthesia - Surgery may be necessarily followed by the use of a hip cast