28. Congenital/Developmental Problems Flashcards

Question

Describe the non-Ponseti treatments for clubfoot (French, surgery) Problems with each?

Answer 1

French: combo of splinting, stretching, taping. (Problem: may need to do Ponseti afterwards....) Surgery: foot reconstruction. (Problem: results in very stiff and scarred foot)

Answer 2

- DDH - Tortocollis (fixed rotation of neck due to tight SCM) - Spinal anomalies (myelomeningocele aka Spina Bifida)

Answer 3

aka Myelomeningocele Dev defect of neural tube, resulting in an open spinal cord lesion. 1. **Anencephaly** (no brain/skull/scalp, kiddo looks like a frog) 2. **Spina Bifida Occulta** (mild) 3. **Meningocele** (absence of bone covering spinal cord) 4. **Myelomeningocele** (skin, nerve, bone all extruding out)

Answer 4

–Defect of posterior bony elements of the spine –Usually asymptomatic -Location: L5/S1

Answer 5

**•folic acid deficiency\*\*** **•Obesity\*\*** * Diabetes * Being exposed to hyperthermia * Certain medications * valproic acid * carbamazepine

Answer 6

Depends on level of myelomeningocele defect. 1. if at thoracic level: functional ambulation is rare. 2. If at L1-L3 level: will need bracing/assistive devices to ambulate. 3. L4-L5 level: milder, will ambulate later if given aids when learning to walk (braces) 4. Sacral level: children can often learn to walk without aids or braces. Distal weakness may lead to foot deformities --\> may benefit from orthotics.

Answer 7

bc muscle strength is associated with ambulatory outcome. Ex: iliopsoas strength between 0-3 is associated with reliance on wheelchair. (Strength: 0 = none 1 = twitch, 2 = moves but cannot oppose gravity, 3 moves against gravity, 4 can be overcome, 5 strong)

Answer 8

–A group of permanent **disorders of the development of movement and posture**, causing activity limitation, that are attributed to non-progressive disturbances that **occurred in the developing fetal or infant brain** –Defect is CNS-related: **motor neuron problem**. The resulting muscle and connective tissue problems you see are the result of this initial motor neuron abnormality

Answer 9

Subtype determined by underlying muscle tone _–Spastic_ **•Most common** * Velocity-dependent increase in muscle tone * Hyperreflexia _–Athetoid_ * Dyskinesia (writhing) * Dystonia _–Ataxic_ * Uncommon * Disturbance of coordinated movement _–Mixed_ Both pyramidal and extrapyramidal motor control abnormalities (mix of fine and gross motor problems)

Answer 10

- Spastic diparesis: both legs involved - hemoparesis: one leg and ipsilateral arm involved - Triparesis: both legs + one arm etc etc

Answer 11

incidence: 2 or 3 per 1000 live births: **more common than DDH, spina bifida, clubfoot (individually). **Spina bifida most rare of all these. The more **premature**, the higher chance of CP higher chance in **twins, triplets, etc**

Answer 12

(All these are associated with a single motor defect) –joint/muscle contractures–foot deformity **(equinus foot is the most common musculoskeletal deformity in CP)**–hip dysplasia (chronic hip subluxation and progressive dislocation); even if the hip joints start normal at birth, it will eventually become dysplastic so watch out for this!!!!–neuromuscular scoliosis–Osteopenia (bc of less walking, esp on affected side) –cognitive impairment (risk increases with greater degree of neuromuscular impairment)–seizure disorder–visual impairment–dysphagia–speech impairment–failure to thrive (high metabolism and it’s hard to keep up with the spastic muscles!) –impaired airway clearance–Chronic pain

Answer 13

- p**ersistence of primitive reflexes** is bad, since these make it hard to ambulate - **sitting by age 2** is predictive of ambulation - **if not walking by 6, probably will never walk** - adverse factors are mental retardation, epilepsy, certain types of CP - lack of control of facial muscles doesn't correlate to intelligence - keep working with these kids

Answer 14

- coordinated care with ortho, PT, etc etc - orthoses, adaptive equipment - spasticity management: GABA agonists, botox inj in calf muscles - dorsal rhizotomy (neurosurg that breaks problematic nerve roots)

Answer 15

- voluntary control, tone, balance - start with head control and work way caudal (if they can't control head, trunk they will never learn to walk)

Answer 16

- prevent deformity - substitute for, & protect weakened parts - stabilize - improve sitting balance - night splinting to maintain positioning

Answer 17

There is basically a page of info on this (EFF HER). The basics: ## Footnote Level 1 = **normal**. 2= must use handrail. 3= self-powered 4= short distances only with help, 5= no good head control **WORST**

Answer 18

Opposite from GMFCS in that 1 is worst, 5 is best. This scale represents mobility over 3 sets of distances (to represent home, school, community)

Answer 19

(I condensed about 8 slides into one here.....) _Point of surgery:_ gait improvement, positioning/posture improvement, hygeine access, pain control. _Types of treatment:_ - Tendon lengthening (ex Achilles) - Long bone realignment - hip reduction (femoral ostetomy) - spinal fusion (PSF = Posterior Spinal Fusion: fusing the **whole** **spine**, because if you fuse only one section the other parts tend to curve even more) - hip resection

Answer 20

**–Every 6 -12 months** –**Screening AP pelvis x-ray:** Repeat yearly for ambulatory patients, Repeat twice yearly if subluxation **–Screening scoliosis x-ray** –Repeat if needed, based on exam –Help coordinate all disciplines

Answer 21

From Wiki: **partial or complete dislocation of a joint.** Significant structural displacement, visible on imaging. (means slightly different things to ortho, opthomology, dentistry but this seems the most relevant)

Answer 22

**•Manipulation and casting** –Useful for ankle, knee, elbow, wrist, hand –Change every few days to weeks –Sometimes used in conjunction with Botox –Constraint therapy (immobilize good side to force dev of the bad side) *Pic: example of constraint therapy*