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Diastematomyelia
What Is It?Diastematomyelia is a congenital malformation of the spinal cord. It is one of many forms of spinal dysraphism or neural tube defects, which arise due to abnormal development of the lower end of the nervous system. This particular malformation is a complete or partial separation of the spinal cord into 2 separate "hemicords". The separation generally occurs in the middle of the spinal cord and the hemicords reunite below it.Diastematomyelia can vary in its severity. Most are split cords that lie within the same spinal coverings (dura and arachnoid). However, in some, there is a complete split and each hemicord has its own covering dura and arachnoid. Oftentimes these also have a dividing "spur" of vertebral bone, cartilage or fibrous tissue that separates them as well. What Types of Symptoms Are Typical?Presenting symptoms of diastematomyelia can vary depending on the severity of the malformation. Superficial stigmata of the underlying malformation can often be seen on the overlying skin of the back. Neurological symptoms depend on the location and severity of the malformation but in severe cases can significantly compromise the functioning of the lower extremity (legs) and some bowel and bladder function.In particular, if a patient has a complete split with a spur separating the hemicords, symptoms often occur as the patient grows. The spur prevents the normal movement of the cord with growth and a tethering of the cord phenomenon may occur, leading to progressive injury to the spinal cord and resulting dysfunction of the legs, bowel and bladder. The traction on the cord may also lead to the formation of associated syringomyelia. How Is The Diagnosis Typically Made?As mentioned above, stigmata on the overlying skin of the back is common and may provide a clue to the presence of the malformation below. For this reason, physicians routinely examine the skin of the back of newborn infants to identify any suspicious findings which may indicate the need for further work-up. As a patient gets older, neurological symptoms in the lower extremities and bowel and bladder may bring the malformation to medical attention if it has not been diagnosed previously.Generally, an imaging study, such as an MRI of the spine, is required to fully elucidate the malformation and characterize its location, type and severity. X-rays or CT scan may also be performed to detect the bony abnormalities involved in the malformation, such as the presence of a bony spur between the hemicords. What Are Some Common Treatments?Treatment does not aim to "reattach" the halves of the split spinal cord. However, treatment varies depending on the symptoms and severity of the malformation. Asymptomatic patients or patients who have stable symptoms which do not progress may only require conservative treatment and follow-up. However, for those patients with progressive neurological symptoms, surgical treatment may be indicated to attempt to preserve function and prevent further injury of the spinal cord. If a patient has tethering due to a bony or fibrous spur, removal of this spur may help to alleviate tension on the spinal cord.Because each patient and each malformation is different, general recommendations or guidelines for treatment cannot be made. Each patient and their family should seek the advice of their own treating physicians to determine the best course of action for their unique case. Return to the Pediatric Neurological Disease section. Return to the Nervous System Diseases home page. Important Note: This site is not intended to offer medical advice. Every patient is different, and only your personal physician can help to counsel you about what is best for your situation. What we offer is general reference information about various disorders and treatments for your education. |
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