Diastematomyelia, also called split cord malformation (SCM), is a rare congenital spinal condition in which the spinal cord is divided into two distinct segments. This division is usually caused by a fibrous, cartilaginous, or bony septum and is most often located in the thoracic or lumbar spine. Many cases are identified in childhood, but mild forms may go undiagnosed until adulthood.
Causes and Risk Factors
Diastometamyelia develops during early embryonic growth due to abnormal neural tube formation.
Congenital origin: Present at birth as a result of faulty spinal cord development.
Associated anomalies: Frequently occurs with spina bifida, tethered cord syndrome, scoliosis, or other neural tube defects.
Genetic and environmental influences: Believed to contribute to abnormal spinal development.
Diastematomyelia Types
Two main subtypes are recognized:
Type I: Two hemicords are enclosed in separate dural sacs, typically divided by a rigid bony or cartilaginous septum.
Type II: Both hemicords are located within a single dural sac, separated by a fibrous band.
Diastematomyelia Symptoms
Clinical presentation varies depending on the type and severity of the malformation:
Persistent back or lower spine pain.
Weakness, numbness, or tingling in the legs.
Difficulty walking and balance problems.
Spinal deformities such as scoliosis.
Cutaneous markers over the spine (birthmarks, patches of hair, dimples).
Bladder or bowel dysfunction in advanced cases.
Diagnostic Methods
Accurate diagnosis relies on imaging studies:
Magnetic Resonance Imaging (MRI): Best method to visualize hemicord separation, soft tissues, and associated anomalies.
Computed Tomography (CT): Provides detailed images of bony septa and structural changes.
X-rays: Helpful in detecting scoliosis and bony abnormalities.
Diastematomyelia Treatment Options
Management depends on the severity of symptoms and associated conditions.
1. Non-surgical approach
In asymptomatic or mild cases, careful monitoring is recommended.
Physical therapy and rehabilitation can help maintain strength and flexibility.
2. Surgical treatment
Excision of the septum: Most common procedure to release the spinal cord and restore space.
Tethered cord release: Performed if the spinal cord is abnormally stretched or attached.
Corrective surgery for scoliosis: Indicated in patients with significant spinal curvature.
The primary goal of surgery is to prevent progressive neurological deterioration, relieve pain, and improve quality of life.
Conclusion
Although rare, diastematomyelia can cause significant neurological problems if left untreated. Early detection and appropriate treatment are crucial to reduce complications. Regular follow-up, especially in children, is essential to monitor scoliosis and neurological symptoms.