Updated: 10/6/2016

Diastematomyelia

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Introduction
  • A fibrous, cartilagenous, or osseous bar creating a longitudinal cleft in the spinal cord
    • if the cord does not reunite distally to the spur, it is considered a diplomyelia (true duplication of the cord)
  • Epidemiology
    • demographics
      • typically presents in childhood
      • adult presentation is rare
    • location
      • more common in lumbar spine (L1 to L3)
  • Pathoanatomy
    • a congenital anomaly believed to be caused by persistence of neuroenteric canal
      • present during 3rd and 4th week of gestation
    • leads to sagittal division of the spinal cord or cauda equina
  • Associated conditions
    • congenital scoliosis
      • as high as 79% in some series
    • tethering of cord
Presentation
  • Symptoms
    • muscle atrophy
    • weakness
    • bladder or bowel incontinence
  • Physical exam
    • sensory loss
    • reflex asymmetry
    • spinal cutaneous manifestations (>50% of patients)
      • hairy patch (hypertrichosis)
      • dimple
      • subcutaneous mass
      • teratoma
    • lower extremity deformity
      • cavus foot
      • club foot
      • claw toes
Imaging
  • Prenatal ultrasound
    • may be diagnosed in utero, during the third trimester
  • Radiographs
    • interpedicular widening is suggestive 
  • CT 
    • helpful to delineate bony anatomy  
  • Myelography 
    • spurs are often undetectable by plain radiographs or CT, especially if they are fibrous or there is rotation from concomitant scoliosis
    • myelography is a useful adjuvant
  • MRI
    • required to evaluate degree of neurologic compression 
Treatment
  • Nonoperative
    • observation alone
      • indications
        • may be observed if patient is asymptomatic and does not have neurologic sequelae
        • must watch closely for progressive neurological deterioration
  • Operative
    • surgical resection
      • indications
        • surgery is indicated if patient is symptomatic or has neurologic deficits
        • must resect diastematomyelia before correction of spine deformity
      • must resect and repair the duplicated dural sac
 

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