Diastematomyelia

Diastematomyelia

Diastematomyelia is a rare congenital condition characterized by the splitting of the spinal cord, typically by a bony, cartilaginous, or fibrous septum. Because it is a congenital anomaly, its symptoms and severity can vary widely among individuals. In some cases, symptoms may not be noticeable and can be diagnosed incidentally during evaluation for another health issue. However, if symptoms do manifest, problems such as back pain, leg weakness, or loss of bladder or bowel control may occur. Diastematomyelia often requires surgical intervention because the division of the spinal cord can lead to nerve damage or other complications.

What is Diastematomyelia?

Diastematomyelia is characterized by the division of the spinal cord, typically by a septum composed of bone, cartilage, or fibrous tissue. This rare congenital condition commonly occurs in the lumbar (lower back) or thoracic (mid-back) regions. It can impede normal spinal cord and nerve root function, leading to various neurological and physical issues.


Symptoms of Diastematomyelia

Symptoms of diastematomyelia typically emerge during childhood and may include:

  • Motor and Sensory Issues: Weakness below the waist, risk of paralysis, loss of sensation.
  • Bladder and Bowel Problems: Difficulty with urinary and bowel control.
  • Difficulty Walking: Foot deformities and abnormal gait patterns.
  • Spinal Deformities: Spinal curvature such as scoliosis or kyphosis.
  • Skin Abnormalities: Birthmarks, dimples, or abnormal hair growth in the lower back region.

Diagnosis and Tests

Methods used in the diagnosis of diastematomyelia include:

  • Magnetic Resonance Imaging (MRI): Provides detailed images of the split structure of the spinal cord and spinal canal.
  • Computed Tomography (CT): Details bone structures and identifies the presence of a septum.
  • Ultrasound: Particularly used in evaluating the spinal cord and surrounding tissues in infants.
  • X-ray: Shows spinal deformities.

Treatment Methods for Diastematomyelia

Surgical Treatment:

  • Spinal Cord Detethering Surgery: Performed to release tethered spinal cord and remove the septum. This procedure aims to reduce tension on nerve tissue, alleviate symptoms, and minimize progressive neurological damage.

Early Intervention: Should be undertaken as early as possible to prevent neurological damage.

Supportive Treatments:

  • Physical Therapy and Rehabilitation: Aids in improving motor function and sensory abilities.
  • Orthopedic Supports: Special shoes or supports may be needed to correct foot deformities and support walking ability.

Regular Monitoring:

  • Neurological and Physical Examinations: Regular follow-up post-surgery and in the long term is crucial.

Diastematomyelia is a complex condition that can lead to serious neurological and physical issues due to the abnormal division of the spinal cord. With early diagnosis and appropriate surgical intervention, most patients can experience improvement and lead active lives. Long-term success requires a multidisciplinary approach and regular medical supervision. Patients and healthcare providers should be aware of the nature of the condition and its potential impacts, carefully evaluating suitable treatment strategies.

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