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Updated: Jun 24 2021


Images mri.jpg
  • summary
    • Diastematomyelia is a congenital spinal condition caused by a fibrous, cartilaginous, or osseous bar creating a longitudinal cleft in the spinal cord.
    • Diagnosis is made with a prenatal ultrasound in the 3rd trimester. 
    • Treatment is observation if patient is asymptomatic. Surgical resection is indicated if patient is symptomatic or has neurological deficits.
  • Epidemiology
    • Demographics
      • typically presents in childhood
      • adult presentation is rare
    • Anatomic location
      • more common in lumbar spine (L1 to L3)
  • Etiology
    • 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
        • if the cord does not reunite distally to the spur, it is considered a diplomyelia (true duplication of the cord)
    • 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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