Syrinx, Syringomyelia and Disatematomyelia

Question 1

Define syringomyelia?

Answer 1

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

Question 2

What is a syringobulbia?

Answer 2

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

Question 3

What are the predisposing factors for devleoping syringomyelia?

Answer 3

• 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

Question 4

What are the signs and symptoms of Syringomyelia?

Answer 4

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

Question 5

What are the signs and symptoms of syringobulbia?

Answer 5

• 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

Question 6

What imaging is useful in dx of syringomyelia?

Answer 6

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

Question 7

What is the TX of syringomyelia and syringobulbia?

Answer 7

• 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

Question 8

what is Disatematomyelia?

Answer 8

• 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)

Question 9

What is the epidemiology of Disatematomyelia?

Answer 9

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

Question 10

What is the pathology of Disatematomyelia?

Answer 10

• 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

Question 11

What are the associated conditions of Disatematomyelia

Answer 11

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

Question 12

What are the signs and symptoms of Disatematomyelia?

Answer 12

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

Question 13

What imaging helps in dx of Disatematomyelia?

Answer 13

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

Question 14

What are the tx for Disatematomyelia?

Answer 14

• 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