Spinal Anomalies Flashcards by Unknown Unknown

Another name for spinal dysraphism

Spina Bifida

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What three types of spinal dysraphism are there?

Spina bifida occulta
Meningocele
Myelomeningocele

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What does spina bifida aperta mean?

Open or cystic

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

5-10%

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

Tethered cord.

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What are the most common symtoms of tethered cord?

gait disturbance
leg weakness
atrophy
urinary disturbance
foot deformities.

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Why is closure prefered before 36h from birth?

Due to colonization and increased risk of infection.

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How common is spina bifida with meningocele or myelomeningocele?

1-2/1000 live births.

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Is there any inheritance involved in meningo/myelomeningocele?

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

folic acid.

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How many percent of patients w MM develop hcph?

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

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

5-10%

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How common is latex allergy in MM children?

up to 73% thpught to be iatrogenically caused.

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Prenatal detection of tube defects

p 307

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What might be good with intrauterine closure of MM defect?

reduced chiari II defect
possibly reduced incident of hcph.

Obs does not improve distal neurologic deficits.

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

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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How soon after birth should a MM lesion be closed?

within 36h from birth.

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Levels of MMs and associated problems

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

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

2-2.5.

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What additional abnormalities needs looking out for before surgery?

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

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What does postoperative leakage usually mean?

A need for shunting.

How does most surgeons usually time shunting?

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

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

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

How does scoliosis correlate to tethered cord?

Early untethering might improve scoliosis.

What are the most common symtoms of tethered cord?

* gait disturbance * leg weakness * atrophy * urinary disturbance * foot deformities.

What is the worst symtom of a tethered cord?

Dealyed neurological deterioration.

What is the outcome with modern treatment for MM ?

* 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.

Lipomyeloschisis

page 284

What is a Dermal sinus

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

Appearance of dermal sinus?

* dimple or sinus 1-2mm * With or without hairs * with or without coloured skin. * may have an underlying mass.

Why is it important to notice a dermal sinus?

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

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

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

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

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

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

Raised scapula due to embryonic failure to place correctly.

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

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

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

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

What is "Tethered cord syndrome"?

Abnormally low conus medullaris.

What is Tethered cord usually associated with?

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

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

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.

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?

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

How can filum terminale be distinguished from nerve roots?

* 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.

What is a "split cord malformation"?

embryologically developed double spinal cord

Type 1 split cord malformation?

* 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.

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

* nevi * hypertrichosis * lipomas * dimples * hemangiomas

What associated deformity goes with split cord 1?

An orthopedic foot deformity.

What is usually the symtoms of split cord deformity 1?

Symtoms of tethered cord.

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

* removal of the bony septum is needed. * Dura needs to be reconstructed to one neural tube. * Detethering

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

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