Syrinx, Syringomyelia and Disatematomyelia Flashcards Preview

Spine FRCS > Syrinx, Syringomyelia and Disatematomyelia > Flashcards

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

Define syringomyelia?

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


What is a syringobulbia?

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


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


What are the signs and symptoms of Syringomyelia?


  • Insidious between adolscent and 45 yrs
  • central cord syndrome


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



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


What imaging is useful in dx of syringomyelia?

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


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


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)


What is the epidemiology of Disatematomyelia?

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


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


What are the associated conditions of Disatematomyelia

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


What are the signs and symptoms of Disatematomyelia?


  • Muscle atrophy
  • weakness
  • bladder/bowel incontinence


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


What imaging helps in dx of Disatematomyelia?

Prenatal ultrasound

  • inutero , during 3rd trimester


  • interpedicular widening


  • delinate bone anatomy


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


  • elvaluate degree of neurological compromise


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