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pediatrics > Spinal Anomalies > Flashcards

Spinal Anomalies Flashcards

1
Q

Another name for spinal dysraphism

A

Spina Bifida

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2
Q

What three types of spinal dysraphism are there?

A
  • Spina bifida occulta
  • Meningocele
  • Myelomeningocele
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3
Q

What does spina bifida aperta mean?

A

Open or cystic

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4
Q

What is the reported prevalence of Spina bifida occulta in north americans?

A

5-10%

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5
Q

Spina bifida occulta may be associated to another deformity that might give problems. Which?

A

Tethered cord.

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6
Q

What are the most common symtoms of tethered cord?

A
  • gait disturbance
  • leg weakness
  • atrophy
  • urinary disturbance
  • foot deformities.
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7
Q

Why is closure prefered before 36h from birth?

A

Due to colonization and increased risk of infection.

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8
Q

How common is spina bifida with meningocele or myelomeningocele?

A

1-2/1000 live births.

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9
Q

Is there any inheritance involved in meningo/myelomeningocele?

A

Probably multifactorial. Increased risk if a sibling has had a MM/MMC child or if the couple has had another child w MM/MMC.

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10
Q

What food is associated with lessened risk of MM/MMC?

A

folic acid.

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11
Q

How many percent of patients w MM develop hcph?

A

65-85% (over 80% before age 6mo)

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12
Q

How many % of pt w MM are born with clinically overt hcph?

A

5-10%

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13
Q

How common is latex allergy in MM children?

A

up to 73% thpught to be iatrogenically caused.

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14
Q

Prenatal detection of tube defects

A

p 307

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15
Q

What might be good with intrauterine closure of MM defect?

A
  • reduced chiari II defect
  • possibly reduced incident of hcph.

Obs does not improve distal neurologic deficits.

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16
Q

If a MM lesion is ruptured, what needs to be done?

A
  • beta-lactamase-resistant pencillin
  • Gentamicin
  • cover lesion with wet sponges not to dry out.
    *Keep child in trendelenburg position on the stomach to keep pressure off lesion.
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17
Q

How soon after birth should a MM lesion be closed?

A

within 36h from birth.

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18
Q

Levels of MMs and associated problems

A
  • complete paralysis - Th12
  • Possible problems w clawing toes with growth - S2
    läs mer page 282
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19
Q

How many other additional abnormalities are seen in a MM child on average?

A

2-2.5.

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20
Q

What additional abnormalities needs looking out for before surgery?

A
  • pulmonary immaturity - may preclude surgery.
  • Bladder - start pt on regular urinary catheterizations and consult urologist
  • Assess scoliosis and kyphosis
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21
Q

What does postoperative leakage usually mean?

A

A need for shunting.

22
Q

How does most surgeons usually time shunting?

A

Wait at least 3 days after MM repair. If not an overt hcph, then simultaneous shunting may be performed.

23
Q

What are the most important late problems/issues for MM patients?

A
  • hcph or shunt malfunction
  • syringomyelia
  • Thethered cord syndrome (70% have on imaging but only minority is symtomatic)
  • scoliosis
  • Dermoid tumor at MM site incidence 16%
  • Chiari II formation
24
Q

How does scoliosis correlate to tethered cord?

A

Early untethering might improve scoliosis.

25
Q
A
26
Q

What are the most common symtoms of tethered cord?

A
  • gait disturbance
  • leg weakness
  • atrophy
  • urinary disturbance
  • foot deformities.
27
Q

What is the worst symtom of a tethered cord?

A

Dealyed neurological deterioration.

28
Q

What is the outcome with modern treatment for MM ?

A
  • 85% of infants survive. Most common early cause of death is Chiari, late is shunt malfunction.
  • 80% normal IQ. -mental retardation usually correlated to shunt infection.
  • 40-85% ambulatory with bracing! But most use wheelchairs for ease.
  • 3-10% normal urine continence, most use intermittent catheterization.
29
Q

Lipomyeloschisis

A

page 284

30
Q

What is a Dermal sinus

A

Tract lined with epithelium. cephalic or caudal. Most common lumbosacral.

31
Q

Appearance of dermal sinus?

A
  • dimple or sinus 1-2mm
  • With or without hairs
  • with or without coloured skin.
  • may have an underlying mass.
32
Q

Why is it important to notice a dermal sinus?

A

Some connect with coccyx, some with the dural tube.
SOme form a epidermoid cyst containing keratine. Some contain sebum and hair

33
Q

What is NOT to be done when finding a dermal sinus?

A

Never inject contrast - it might induce infection or sterile meningitis.

34
Q
A
35
Q

Why has Klippel-feil patients short neck with limitation in movement?

A

Due to 2-more cervical vertebra fused.
Ranging from only fusion of the body-congenital block vertebra; to fusion of the entire vertebra.

36
Q

Sprengels deformity is associated to Klippel Feil syndrome. What is that?

A

Raised scapula due to embryonic failure to place correctly.

37
Q

What clinical associations are usually seen e Klippel-Feil syndrome?

A
  • scoliosis 60%
  • Sprengels deformity 25%
  • facial asymmetry, torticollis, with more!
    Non-neural are unilateral deafness 30!%, unilateral loss of a kidney, with more
38
Q

What is (strangely) the most common needed surgery for KLippel-Feil syndrome pt?

A

Fusion of unstable non-fused segments. At the risk of further loss of mobility!

39
Q

What is “Tethered cord syndrome”?

A

Abnormally low conus medullaris.

40
Q

What is Tethered cord usually associated with?

A
  • Short, thickened filum terminale
  • OR an intradural lipoma.
  • Myelomeningocele - most of these patients have tethered cord radiologically and need to be clinically assessed.
41
Q

What should you expect if a MM pt has:
* increasing scoliosis
* increasing spasticity
* worsening gait
* deteriorating urodynamics

A

1! always rule out shunt dysfunction
2. Pain? - tethered cord until proven otherwise
3. No pain - syringomyelia until proven otherwise
4. May be due to Chiari II malformation -brainstem compression.

42
Q

Obs! About scoliosis and tethered cord!!!
There is a great difference in outcome of de-tethering dependent on how the scoliosis looks. What is the difference?

A

If less than 10% scoliosis, 68% had neurologic improvement and the remaining were stabilized. When more than 50 % (severe) scoliosis, 16% deteriorated.

43
Q

How can filum terminale be distinguished from nerve roots?

A
  • characteristic squiggly vessels on the surface
  • distinctly whiter appearance
  • ligamentous like strands running through it.
  • Intra-op electrical stimulation and anal-sphincter EMG recording is more definitive.
44
Q

What is a “split cord malformation”?

A

embryologically developed double spinal cord

45
Q

Type 1 split cord malformation?

A
  • 2 hemicords
  • both have their own central canal and surrounding pia and separate dural tube.
  • SEPARATED BY A RIGID OSSEOCARTILAGINOUS MEDIAN SEPTUM.
  • abnormalities of the spine is seen by the split.
46
Q

2/3 of type 1 split cord malformation pt have associated overlying skin abnormalities:

A
  • nevi
  • hypertrichosis
  • lipomas
  • dimples
  • hemangiomas
47
Q

What associated deformity goes with split cord 1?

A

An orthopedic foot deformity.

48
Q

What is usually the symtoms of split cord deformity 1?

A

Symtoms of tethered cord.

49
Q

What is usually the treatment if a patient has symtoms of tethered cord and a split cord deformity type 1?

A
  • removal of the bony septum is needed.
  • Dura needs to be reconstructed to one neural tube.
  • Detethering
50
Q

! Obs! In surgery of tethered cord in split cord formation 1!!

A

The median septum should be removed before detethering!! Otherwise the cord may retract up against the septum. Auch.

51
Q
A

pediatrics (9 decks)

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