What is Split Spinal Cord Malformation (SSCM)?
Skull and brain deformities and abnormalities are typically congenital and in many cases genetic, except in cases of trauma. Diseases, disorders and abnormal development of skulls, brains and the spine range in severity from easily treatable to fatal. Genetic testing and screenings during pregnancy can help with early detection in cases of congenital abnormalities.
Split spinal cord malformation (SSCM) is a rare form of spinal dysraphism (a malformation) in which a person is born with splitting, or duplication, of the spinal cord. It may be characterized by complete or incomplete division of the spinal cord, resulting in two “hemicords.” There are two types of SSCM. In type I, each hemicord has a full protective covering (thecal sac) and they are separated by a bony structure, forming a double spinal canal. In type II, the two hemicords are surrounded by one thecal sac and there is a single spinal canal. While SSCM is present from birth, it may become apparent at any age and is more common in women than in men.
Types of Split Spinal Cord Malformation (SSCM)
This rare form of spinal cord malformation is classified into one of two types:
- Type I: Duplicated dural sac (the tube of matter that surrounds the spinal cord), each hemicord (the separated spinal cord) separated by a bony structure
- Type II: Split cord, forming two hemicords, each surrounded by a protective covering
Causes of Split Spinal Cord Malformation (SSCM)
Split spinal cord malformation (SSCM) is thought to be caused by abnormal formation of the notochord and neural tube (which ultimately form the brain and spinal cord) very early in embryonic development.
Risk Factors for Split Spinal Cord Malformation (SSCM)
Abnormal formations and developments during early embryonic stages of growth are connected to split spinal cord malformation. This congenital abnormality occurs in about 5 percent of all spinal congenital defects.
Screening for & Preventing Split Spinal Cord Malformation (SSCM)
Split spinal cord malformation can be detected by ultrasound, through fetal MRI and by monitoring amniotic fluid acetylcholinesterase levels.
Signs & Symptoms of Split Spinal Cord Malformation (SSCM)
The number and severity of symptoms experienced may differ among people with this disease. Your experience may be different from others, and you should consult your primary care provider for more information.
Frequent symptoms include the coexistence of two hemicords causing splaying of the posterior vertebral elements which results in neurological deficits in lower limb or perineum. Less often, there is abnormally increased hair growth over much of the entire body, and sometimes there is the presence of an abnormal lateral curvature of the spine.
Diagnosing Split Spinal Cord Malformation (SSCM)
These rare malformations can be diagnosed by ultrasound, through fetal MRI and by monitoring amniotic fluid acetylcholinesterase levels. They are repaired surgically, ideally early, to improve neurologic symptoms.
Treating Split Spinal Cord Malformation (SSCM)
These rare malformations should be diagnosed and repaired surgically early to improve neurologic symptoms.
Living with Split Spinal Cord Malformation (SSCM)
Treatment for split spinal cord malformation is surgery, and without it those with a diagnosis may end up paraplegic or quadriplegic. Quality of life for patients with a split spinal cord malformation diagnosis is similar to individuals with malignant neoplasms or heart failure.
To further your understanding of your diagnosis and to contribute to cutting-edge research, consider participating in a clinical trial so clinicians and scientists can learn more about causes, symptoms, treatment and prevention. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat or prevent disease.
All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.
For information about participating in clinical research, visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for participants at Clinicaltrials.gov.