Congenital Malformations

Question 1

Is spontaneous recovery in developmental hip dysplasia common

Answer 1

Yes in first 1-2 weeks

Question 2

Risk factors for developmental hip dysplasia

Answer 2

• F>M, family history
• breech position, LGA
• Tight swaddling

Question 3

Developmental hip dysplasia S&S

Answer 3

Hip subluxed or dislocated
Hip is at risk for dislocation
- Might not show signs but contact pediatrician if any of the following because they can lead to degenerative hip arthritis: LLD, Muscle weakness, waddling gait

Question 4

Developmental hip dysplasia Ax

Answer 4

• Barlow maneuver: F then Abd the Add w/ post pressure

- Ortlani maneuver: F then Add + slight traction

Question 5

Developmental hip dysplasia Rx:

Answer 5

Keep hips flexed and abducted

• Pavlik harness
• No double diapering

Question 6

What is another name for club foot

Answer 6

Talipes Equinovarus

Question 7

What causes club foot

Answer 7

• Congenital bone deformity
• CP
• Calf mm contracture

Question 8

What are the 4 types of club feet

Answer 8

(1) idiopathic - most common, otherwise healthy child, picked up in utero, intensive rehab;
(2) neurogenic - spina bifida, cerebral palsy;
(3) syndromic - möbius syndrome or arthrogryposis (contracture of at least two or more joints);
(4) postural - feet squished in utero, resolves quickly with minimal intervention

Question 9

What is the presentation for club feet

Answer 9

• PF (talocrural aka equinus)
• Forefoot adduction, inverted/varus hindfoot
• Small calcaneus
• Smaller calf + foot on affected side
• Usually part of larger problem: myelomeningocele, arthrogryposis

Question 10

What is a risk associated with club feet

Answer 10

intrauterine restriction (baby doesn’t grow @ normal expected rate in uterus)

Question 11

Club feet Rx

Answer 11

• Manipulation
• Serial casting + splinting
• Surgery

Question 12

What is osteogenesis imperfecta

Answer 12

Autosomal dominant connective tissue disorder

• Issue in converting pro-collagen to collagen type 1 (synthesis defect)
• leads to brittle bones

Question 13

What are the types of osteogenesis imperfecta

Answer 13

Type I: most common, least severe
II: lethal in perinatal period (lack ossification)
III: severe, progressive deformity, very short
IV: rare & mild, moderate deformity, ✓ambulate

Question 14

Osteogenesis Imperfecta S&S

Answer 14

• Joint laxity
• Muscle weakness (disuse)
• Long bone bowing (LE>UE)
• Kyphosis/scoliosis
• Diffuse osteoporosis (multiple recurrent #’s  immobilization, disuse osteoporosis, further #)
• Short stature (faciocranial disproportions)

Question 15

Osteogenesis Imperfecta Rx:

Answer 15

o No consistent meds to strengthen bone structure
o Orthopedic surgery (intramedullary rods)
o Social integration (isolation common d/t parental fear)
o Education (for those in contact with child)
o Prognosis – dependent on severity

Question 16

What is legg calve-perthes disease? Who is it most common in

Answer 16

Avascular necrosis of femoral head

Age 3-12, M>F

Question 17

Legg calve perthes disease S/S

Answer 17

• Small for age (delayed bone age)
• Usually unilateral (bilate 20%)
• hip, knee, groin pain (knee first)
• +ve trendelenburg
• limp
• Dec. ROM (Abd, IR)

Question 18

legg calve perthes disease Rx

Answer 18

Controversial (self-limiting disease)

• Containment to preserve femoral head
• ROM, gait, balance, strength
• Bracing, casting, surgery

Question 19

There is a relationship between legg calve perthes disease and what?

Answer 19

Smoking during pregnancy & secondhand smoke