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  • Tethered Cord Syndrome and Occult Spinal Dysraphism

     
    Tethered Cord Syndrome and Occult Spinal Dysraphism

    Return to main page of Tethered cord

    Occult Dysraphic Elements

    Intraspinal anomalies common to OSD include the lipomyelomeningocele, the dermal sinus, diastematomyelia, the tight terminal filum, the neurenteric cyst, the terminal myelocystocele, and meningocele manqué.

    Specific Dysraphic Elements

    Lipomyelomeningocele/Spinal Lipoma/Fatty Filum

    Fatty accumulations within the spinal cord represent 70% of the lesions associated with tethering and take three different forms. The lipomyelomeningocele is a subcutaneous lipoma within the spinal cord that extends through a defect of the lumbosacral fascia, lamina, dura, and pia into a low-lying spinal cord. It is the most common form of spinal lipoma. Patients with these lesions usually come to clinical attention within the first few months to years of life. A subset of the lipomyelomeningocele is the lipoma of the conus medullaris.

    The intradural lipoma (spinal cord lipoma) is a rare intramedullary lesion that is usually found within the thoracic spinal cord. It is not associated with cutaneous or bone anomalies and often presents with symptoms of spinal cord compression.

    The fatty filum involves fatty infiltration the whole length or part of the terminal filum. The fat within the short, thick filum is discernible by unenhanced CT or MR imaging. The occurrence of incidental fat within the terminal filum in the normal adult population has been estimated to be 3.7% in cadaveric studies 22 and 1.5 to 5% in MR imaging studies.

    Lipomyelomeningoceles may be diagnosed by the associated subcutaneous lumbosacral mass that is found in approximately 90% of patients  or by sagittal MR imaging (Figure 4) or ultrasonography studies. For those who come to medical attention before the age of 1 year, 59% of patients with the lipomyelomeningocele or fatty filum are asymptomatic whereas 41% are symptomatic. Of those patients with symptoms in one study, 60% presented with urological symptoms and 58% with neurological symptoms, whereas 58% presented with orthopedic abnormalities (extremity abnormalities and scoliosis). Of patients presenting with motor deficits, 50 to 70% do not experience improvement after surgery. Surgical intervention in the symptomatic patient has been advocated by many authors to prevent further decline in the neurological status.[7,33] A few authors believe that some of the lower motor neuron symptoms and orthopedic symptoms cannot be prevented by surgery.

    Click to zoom
    Figure 4 (click image to zoom) . Sagittal MR images demonstrating a lipomyelomeningocele. A: The spinal cord extends to the level of the sacrum. An associated superficial subcutaneous lipoma is discernible. B: The spinal cord extends to the level of the sacrum. A syrinx is present.

     

    The operation for lipomyelomeningoceles has been significantly advanced by the use of the carbon dioxide laser and the ultrasonic aspirator. Intraoperative monitoring of nerve roots may be useful to distinguish those that are functional from those that are not. Once the spinal cord has been untethered, closure and enlargement of the dura with a graft material has been recommended. In one study  of those patients younger than 1 year of age who underwent an untethering procedure for lipomyelomeningocele or fatty filum and who presented with motor, urological, or orthopedic symptoms, 39% improved, 58% stabilized, and 3% worsened as a result of surgical intervention.
     

     

     


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