Spina Bifida Long

Question 1

Functional Assessment in Spina Bifida?

Answer 1

1) Mobility: Type, Aids, Therapy
2) Incontinence Care: Urinary incontinence aids – pads, catheterisation, pants etc, Bowel incontinence aids – toileting, laxatives, enemas etc
3) Education
4) Developmental issues: Growth/obesity, Executive functioning, Adolescent problems, ADLs – bathing, dressing, feeding

Question 2

Spina Bifida Complications?

Answer 2

“PAST HUBS SSSSSS”

1) Paralysis/coordination
2) Arnold Chiari
3) Spine - scoliosis/kyphosis/hip deformity
4) Tethered Cord - present in virtually all –> Usually manifest during growth spurts - Gait changes, Back or lower limb pain, Worsening motor function, Change in sensory level, bowel/bladder habit
5) Hydrocephalus: From caudal hindbrain anomaly with tonsillar herniation
6) Urinary issues – renal/bladder
7) Bowel
8) Syringomyelia - Syrinx along spinal cord –> can swell + change with time. Associated with scoliosis and LL weakness/deformities, Can be 2o to hydrocephalus or shunt malfunction
9) Sleep disordered breathing (OSA in 20%)
10) Skin - pressure areas, skin care
11) Senses – vision (squint/ambylopia)/hearing –> treat squint early to prevent ambylopia
12) Size - short stature, obese for height
13) Seizures - 15-20% of children with spina bidifa
14) Social issues

Question 3

Important extra Qs in PHx for Spina Bifida?

Answer 3

• Pregnancy and birth history
• Antenatal screening
• FHx of neural tube defects

Question 4

Management of Paralysis in Spina Bifida?

Answer 4

1) Physiotherapy for ambulation training/wheelchair mobility
2) OT – functional training, help with ADLs
3) Gait analysis
4) AFOs/hip positioning braces
5) Mobility aids: Crutches, Gait orthoses, HKAFOs
6) Pressure sore management

Question 5

Problems assoc w Arnold Chiari II malformation in Spina Bifida?

Answer 5

• Swallowing difficulties
• GORD/aspiration
• OSA
• Stridor
• UL weakness
• Cerebellar symptoms

Question 6

Mx of Scoliosis in Spina Bifida?

Answer 6

• Usually due to intraspinal abnormalities
• Often needs surgical correction as bracing does not work

Question 7

Management of Tethered cord in Spina Bifida?

Answer 7

• Only 1/3rd need surgical intervention, rest = allied health

Question 8

Mx of Hydrocephalus in Spina Bifida?

Answer 8

• VP shunt – ensure not obstructed or infected

Question 9

Mx of Urinary Sx in Spina Bifida?

Answer 9

• Anticholinergics (oxybutynin) – help with ↑ bladder storage capacity
• Alpha-adrenergic drugs - ↑ bladder outlet resistance
• ?issues with intermittent catheterisation – usually from birth –> Hydronephrosis or VUR indicate need for this
• Pads and pants
• Penile appliance for boys
• Also think of CKD mineral bone disorder
• UTI frequency – washing catheters

Question 10

Mx of Constipation in Spina Bifida?

Answer 10

• Laxatives to manage constipation
• May need suppositories/enemas to empty rectum
• MACE procedure