Syrinx, Syringomyelia and Disatematomyelia Flashcards Preview

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Flashcards in Syrinx, Syringomyelia and Disatematomyelia Deck (14):
1

Define syringomyelia?

  • A syrinx ( fluid filled cavity) within the spinal cord 
  • Pregressively expands
  • leads to neurological defects

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2

What is a syringobulbia?

  • A syrinx ( fluid filled cavity) within the brain stem 
  • leads to neurologic symptoms

3

What are the predisposing factors for devleoping syringomyelia?

  • Usually from lesions that partially obstuct CSF
    • Craniocervical junction abnormalities
      • >50% chiari malformation
    • Spinal cord trauma
      • 4-10 % pt, found yrs post injury
    • Spinal cord tumours
      • ​30% pt with spinal cord tumours develop a syrinx

4

What are the signs and symptoms of Syringomyelia?

Symptoms

  • Insidious between adolscent and 45 yrs
  • central cord syndrome

Signs

  • Flaccid weakness, atrophy, hyporeflexia of UL
  • deficit in pain/temp sensation in capelike distribution over neck
    • light touch, position & vibration sense not affected

 

5

What are the signs and symptoms of syringobulbia?

  • Symptoms related to cranial nerve involvement
    • tongue weakness and atrophy- CN XII
    • Sternocleidomastoid /trapzius weakness = CN XI
    • Dysphagia adn dysarthria CN IX, X
    • Facial palsy CN VII

6

What imaging is useful in dx of syringomyelia?

  • MRI 
    • dx
    • used with gadolinium to rule out chances of tumour

7

What is the TX of syringomyelia and syringobulbia?

  • non operative
    • Observe
    • if asymptomatic syrinx
    • MRI + gadolinium- rule out tumour
  • Surgery
    • Surgical decompression of foramen magnum and upper cervical root
      • neurological deficit
      • doesn't reverse neuroloigcal deterioration

8

what is Disatematomyelia?

  • A fibrous, cartilagneous or osseous bar creating a cleft in spinal cord
    • if cord doesn't unite distally  to the spur, it is considered a diplomyelia ( true duplication of the cord)

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9

What is the epidemiology of Disatematomyelia?

  • Typically presents in childhood
  • adult presentation rare
  • location= more common in lumbar spine L1-3

10

What is the pathology of Disatematomyelia?

  • A congential abnormality believed to be caused by persistence of NEUROENTERIC canal
    • present during 3-4 wks gestation
    • lead to sagittal division in spinal cord or cauda equina

11

What are the associated conditions of Disatematomyelia

  • Congential scoliosis
    • 79% some series
  • Tethering of cord

12

What are the signs and symptoms of Disatematomyelia?

Symptoms

  • Muscle atrophy
  • weakness
  • bladder/bowel incontinence

Signs

  • Sensory loss
  • reflex asymmetry
  • spinal cutaneous manifestations >50% pts
    • hairy patch
    • dimple
    • subcutaneous mass
    • teratoma
  • lower leg deformity
    • Cavus foot
    • club foot
    • claw toes

13

What imaging helps in dx of Disatematomyelia?

Prenatal ultrasound

  • inutero , during 3rd trimester

Xrays

  • interpedicular widening

CT

  • delinate bone anatomy

Myelography

  • spurs often fibrous not seen bt xray/ct so useful adjunct

MRI

  • elvaluate degree of neurological compromise

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14

What are the tx for Disatematomyelia?

  • non operative
    • Observation alone
    • pt asymptomatic, no neurological sequelae
    • watch closely for neuro deterioration
  • surgery
    • pt symptomatic/ neurological deficits
    • must resect Disatematomyelia before correction of spinal deformity
    • must resect and repair the duplicated thecal sac