Hydromyelia can damage nerve cells.
Hydromyelia is an “abnormal widening of the central canal of the spinal cord that creates a cavity in which cerebrospinal fluid (CSF)…can accumulate”. As a result, pressure is put on the spinal cord, and nerve cells may become damaged. Some use the term hydromyelia synonymously with syringomyelia, another spinal cord cavitation condition. Even though syringomyelia and hydromyelia are sometimes used as interchangeable terms, there are differences. With hydromyelia, the cavity is “connected to the fourth ventricle in the brain” and it is usually associated with children or infants. Hydromyelia also has associations “with hydrocephalus or birth defects such as Chiari Malformation II and Dandy-Walker syndrome”. Syringomyelia, by contrast, is usually found in adults and “features a closed cavity”. The patients typically “have Chiari Malformation type 1 or have experienced spinal cord trauma”.1
Causes and Symptoms
With syringomyelia, the CSF accumulates outside of the central canal. Hydromyelia symptoms include disordered speech, difficulty walking, and weak extremities. A neurologist can assess the damage and recommend treatments. In fact, despite the differences with syringomyelia, the disorders have similar effects, and treatments tend to be comparable. Other symptoms are limb paralysis or spasticity, numbness, tingling, balance problems, or loss of bladder control. The changes can be rapid or they could occur over time.2 Patients may also complain of neck numbness, sensory loss, headaches, bowel incontinence, and visual changes. Hydromyelia causes are “idiopathic”; however, it may be associated with the earlier medication conditions mentioned, as well as spinal cord injury or infection, a tumor or mass in the spinal cord, or some genetic factors. Doctors diagnose the condition with a neurological exam and imaging. The exam looks at symptoms such as changes in gait, reflexes, loss of sensation, and weakness and balance issues. Generally, an electromyography will also be performed to assess muscle speed and strength. The main diagnostic tool, however, is the MRI scan, “specifically CINE MRI” which adds a “heart rate analysis” to the exam so that the CSF flow can be visualized.3 Doctors may also order blood tests to rule out infections. Prompt treatment is necessary so that neurologic complications do not continue to worsen. Additional potential hydromyelia causes include scoliosis (curved spine) and Klippel-Feil Syndrome, which is a “congenital fusion of the vertebrae of the neck limiting its movement”. If hydromyelia is associated with the Chiari Malformation, then the symptoms would appear when the patient is a teenager. If it is due to a trauma or tumor, the symptoms will arise “with any type of straining
The general treatment option for hydromyelia is surgery to ensure that the spinal fluid flows normally. Surgery might be recommended for patients who have “moderate or severe neurological deficits”. Symptoms may be relieved temporarily or permanently with surgery, but there is the potential for severe complications. Sometimes the condition can resolve itself without any intervention.5 A neurologist could also recommend waiting and seeing, depending on the size and location of the CSF buildup. For patients who are not good surgical candidates, doctors may prescribe supportive care and analgesia to manage symptoms.6 During surgery, the CSF flow is corrected. In addition, decompression is performed to help “reduce spinal cord injury from the increased CSF flow”. To reduce hypertension or intracranial pressure, a shunt may be placed. Post-surgically, it is important for patients to have various therapies, such as occupational and physical therapy. To manage the chronic pain, prescription medications may be suggested. Interventions may only provide temporary relief, and permanent damage may have occurred due to hydromyelia. Family support is also crucial for patients.7 There are various surgical options. One is surgery for the Arnold-Chiari Malformation, which involves “posterior fossa decompression”. The surgeon takes out some bone behind the skull to make room for brain expansion, reducing pressure. Another surgery is cyst draining, with a shunt. Yet another surgical option is “removal of obstruction”, in the case of a growth or tumor. Finally, for patients whose hydromyelia is caused by “a tethered cord”, then the abnormality would have to be corrected so that the CSF could flow normally.8 Syringomyelia treatment depends on the symptom progression and severity. The wait-and-see approach might also be used, or else surgery would be recommended. The goal or surgical intervention “is to remove the pressure the syrinx places on your spinal cord and to restore the normal flow of cerebrospinal fluid”. Surgery is similar to the types performed for hydromyelia, including treating the Chiari malformation, draining the syrinx, removing the growth or tumor obstructions, or correcting a spinal abnormality to restore CSF flow. Follow-up is necessary after surgery, as the syringomyelia may recur. MRIs and periodic exams would be ordered. The syrinx could grow back, leading to more treatments, and some patients may have sustained permanent nerve and spinal cord damage, even after surgical interventions.9