MM Myelomeningocele

Question 1

Risk

Answer 1

Risk of 1st childe w MM same general population (0.1-0.2%)
One 👦 hade MM ➡️ risk of 2nd 👦 MM 2-3%
👦 👦 MM risk ➡️ 6-8 %
Risk f sibling families , 👦 in the family from mother side MM

Question 2

Allergy in MM

Answer 2

Latex allergy

Question 3

Intrauterine MM closure

Answer 3

⬇️ Chiari II defect
Not significant ⬇️ HC
Or improve neurological function

Question 4

Rupture MM TX

Answer 4

nafcillin and gentamicin; D/C 6 hrs after MM closure, or

continue if shunt anticipated in next 5 or 6 days)

Question 5

unruptured MM

Answer 5

Cover lesion with soaked Graz NS or RL ➡️ prevent desiccation

Trendelenburg and patient on stomachto ⬇️ pressure

Question 6

Time of MM closure

Answer 6

Should be within 24 hr regardless membrane intact or rupture

After 36 he ⬆️ risk of infection
Aim not improve neurological but ⬇️ infection
Should be latex free

Question 7

Assesment MM

Answer 7

Movement of LL indicate 😊 good prognosis

Question 8

Assessment of chiari2 in MM

Answer 8

OFC
Abnormal OFC > 1 cm / day
US head signing 24 hr
Check strider or apneic episode

Question 9

Position during MM and shunt

Answer 9

Prone head turn to right right hip and knee flex
Use left flank to avoid appendectomy scar later in life).

Question 10

Goal from surgery

Answer 10

1-Free the placode from dire to avoid tethering
2- 💧 tight dura closure
3 - skin closure trime undermine skin to avoid fermion tumors

Question 11

During MM closure avoid

Answer 11

Latex

Do not allow scrub solutions or chemical antimicrobials on neural palcode

No monoplolar
Avoid tension on placode

Question 12

Surgical techniques

Answer 12

Start by normal dura
Closure 5 layers ▶️ easy to releas if tether cord happen

Question 13

Post OP MM repaire

Answer 13

keep patient off all incisions

2. bladder catheterization regimen
3. daily OFC measurements
4. avoid narcotics (midbrain malformation renders these patients more sensitive to respiratory depression from narcotics)
5. if not shunted

a) regular head U/S (twice weekly to weekly)

b) keep patient flat to ↓ CSF pressure on incision

6. if a kyphectomy was done, use of a brace is optional (surgeon preference)

Question 14

Late ⏰ complication

Answer 14

HC always R/ shunt malfunction if MM patient deteriorated

syringomyelia

Tethered cord syndrom 70 radiological ; 20 % symptomatic ( myelopathy )

scoliosis early untethering ⬇️ scoliosis

dermoid tumo( trmimm retained skin )
Chiari 2
Short stature

Question 15

Outcome MM

Answer 15

80% normal IQ ( ⬇️ IQ due to shunt infection )
Early mortality due to Chiari ▶️ repoiratory arrest
Late mortality shunt infection
Mental retardation due to shunt infection
80 can ambulate with bracing
10 have normal ✅ urinary continence but remaine dry IC