Spinal Dysraphism

The term “spinal dysraphism” is broadly used to identify any number of spinal conditions that result in a particular birth defect. This can make it confusing, but knowing which conditions fall under this heading will allow you to better understand the symptoms and treatments available.

About Spinal Dysraphism

Spinal dysraphism covers a wide range of conditions that can be present from the time your baby is born. It has been established that the different forms of spinal dysraphism stem from a malformation of the neural tube that starts around the third week of development. Regardless of which condition this malformation results in, it is due to the neural tube not closing completely to form the spinal cord and spine.

There are several different forms of dysraphism that occur. They include:

  • Myelomeningocele (frequently known as open spina bifida) and spina bifida occulta
  • Split cord malformation (diastematomyelia)
  • Dermal sinus tract
  • Tight filum terminale
  • Spinal cord lipoma (lipomyelomeningocele)
  • Tethered spinal cord

Symptoms of Spinal Dysraphism

The symptoms of spinal dysraphism can vary according to the type and location of the defect:

  • Myelomeningocele and other forms of spina bifida: This condition, the cause of open spina bifida may cause hydrocephalus (fluid on the brain), leg weakness or paralysis, sensory loss in extremities, a tethered spinal cord, incontinence and orthopedic deformities such as neuromuscular scoliosis. It can also cause trouble walking or moving around properly.
  • Split cord malformation (diastematomyelia): With this condition, your child’s spine may have a physical separation of the two halves of the spinal cord with each portion of the spinal cord having its own dura mater (the tough outer membrane that surrounds the spinal cord). This causes the most severe symptoms, such as pain and scoliosis, and can also result in a tethered cord.
  • Dermal sinus tract: While this issue can be dormant or asymptomatic, it can result in a tethered cord and leave your child open to repeated infections.
  • Dermoid and epidermoid cysts of the spine: These cysts usually do not cause neurological symptoms, but if they are pressing on a nerve root or the spinal cord, they can bring about clumsiness, weakness or incontinence.
  • Tethered spinal cord: This condition, where the spinal cord is abnormally attached to structures inside or outside of the spinal canal can cause numbness, tingling, difficulty walking and back pain.

Diagnosis of Spinal Dysraphism

Depending on the type of spinal dysraphism you are dealing with in your child, the diagnostic techniques will vary.

Myelomeningocele is frequently diagnosed before birth during a prenatal ultrasound. In newborns, the presence of a lump or opening on the spine at birth is an indication that your child might be suffering from myelomeningocele.

In the instance of most dermal sinus tracts, a dimple or pit directly over the spine is a good indication of its presence and may be picked up by parents or pediatricians. Despite being readily visible externally, it is hard to know if the hole leads all the way into the spinal cord. If it does, it can cause myriad problems. An MRI can allow doctors to see how far this tract reaches and whether it is involving the spine.

Lipomyelomeningocele is occasionally visible on ultrasound prior to your baby’s birth. If not, a pad of fat over the spine may be an indication of such a problem and can be diagnosed with imaging.

In the cases of spina bifida occulta, dermoid and epidermoid cysts and diastematomyelia, the naked eye cannot pick these abnormalities up. Luckily, there are certain signs that might indicate there’s a problem, including extra skin, a thick patch of hair, pigment changes or differences over the defect or neurological signs such as leg weakness, scoliosis, and incontinence. Imaging such as MRIs or CT scans can help isolate these issues.

Treatment Options for Spinal Dysraphism

When you’re considering treatment options for your child’s spinal dysraphism, it’s important to understand his or her particular condition. Based on the type and severity, your doctor or surgeon may recommend one or more treatments. Below are some of the most effective treatments for each of the different conditions that fall under the definition of spinal dysraphism.

Myelomeningocele

The first course of action for babies with myelomeningocele is to close the opening in the back. This is done either in utero or within the first 48 hours after birth. A team of neurosurgeons and plastic surgeons will work together to ensure that everything is repaired as thoroughly and carefully as possible.

As your child grows, he or she may need additional treatment. Some babies develop hydrocephalus (an excess of cerebrospinal fluid around the brain), requiring that a shunt is placed. The shunt will work to drain off the excess fluid and prevent pressure from building around the brain.

Physical and occupational therapy may also be needed to help your child develop and maintain stronger muscles and increase mobility.

Split Cord Malformation

Spinal cord malformation is almost always treated surgically. Your child will undergo a microsurgery called decompression. This surgery is performed by removing the spiny bone or fibrous formation that is causing tethering or binding of the spinal cord within the spinal canal. This surgery allows for more freedom of movement of the spinal cord and resolves the tethering issue that can also occur with this defect.

In some cases, physical therapy may be recommended to help your child regain mobility and strength following this surgery.

Dermal Sinus Tract

Dermal sinus tracts provide the perfect pathway for infection to occur. Because they can be problematic, surgical exploration with removal is the most effective option. Only around 25 percent of these defects will close on their own, making removal a logical decision. Most children recover with no lasting effects from a dermal sinus tract.

Dermoid and Epidermoid Cysts of the Spine

Dermoid and epidermoid cysts tend to be slow-growing and usually won’t cause issues for your child. However, if they’re positioned in such a way that they interfere with the spinal cord, surgical removal is the best option. Diagnostic imaging can reveal the positioning of these cysts and determine if they are or will eventually be pressing on the spinal cord.

Depending on how severe the compression of the spinal cord is and whether any permanent damage is present, your surgeon may recommend physical therapy following recovery from surgery.

Tethered Spinal Cord

A laminectomy may be in order if your child is experiencing the symptoms of a tethered spinal cord. Because the cord is being restricted by structures within or surrounding the spinal canal, separating these adhesions allows the spinal cord and nerves to move more freely.

In some cases, physical therapy may be necessary for your child to regain mobility and strength following the surgery. Your doctor can give you a good idea of what else may be necessary following a laminectomy.