Myelomeningocele Repair

This type of surgery is done when a baby is born with an open spinal cord that sticks out through a hole in the back. This causes a saclike bulge called a myelomeningocele that is filled with fluid, or open neural tube defect. It is diagnosed before birth using an ultrasound. It can be found anywhere along the spine but is more commonly found in the lower or lumbosacral spine

When a child is born with a myelomeningocele, they may have problems such as leg weakness and sensation and bowel and bladder problems. They might also have other problems such as hydrocephalus, Chiari II malformation or orthopedic deformities of their lower extremities, such as club feet.

Shortly after birth, the baby is kept on their belly to prevent breaking the sac and avoid trauma to the spinal cord. The sac is covered with sterile saline soaked gauze to prevent the exposed spinal cord from drying out. Antibiotics are given to decrease the risk of infection. A myelomeningocele is typically closed within 24 to 72 hours after birth or, in some cases, before birth. The goals of the surgical repair are to restore the nerve tissue to the spinal canal for protection, close the protective membrane (the dura) in order to help prevent a spinal fluid leak, and provide skin coverage for the defect.

Closure involves separating the open spinal cord and dura from the skin on the back and repairing them. The dura must be closed around the spinal cord and, if needed, there is a correction of any bone abnormality that may be present. This operation is done using general anesthesia and is generally well tolerated by most newborns. The skin may be closed by nylon sutures at the time of surgery and will need to be removed 2 – 3 weeks after surgery.

Common postoperative complications include cerebrospinal fluid leak, problems with wound healing and tethering of the spinal cord. Careful closure of the dura and skin are very important. Many patients with myelomeningocele also have hydrocephalus. This is a condition where the fluid spaces in the brain become enlarged because the fluid contained within the spaces, cerebrospinal fluid, does not drain properly. In these children, the brain fluid may have to be removed from the brain by placing a ventriculoperitoneal shunt, either at the time of closure of the myelomeningocele or as a separate operation.

If your baby is diagnosed with myelomeningocele before birth, your obstetrician will refer you to a pediatric neurosurgeon for consultation. The pediatric neurosurgeon will discuss the findings on the prenatal ultrasound or fetal MRI, talk about possible limitations in function, discuss possible treatment options and discuss postnatal/postoperative care. Questions and concerns will also be addressed at that time. 

A typical stay in the newborn intensive care unit for a child with a myelomeningocele defect is about 1 – 2 weeks. During that time, the myelomeningocele will be closed and hydrocephalus, if present, will be addressed. A urologist will be consulted to assess the function of the bladder. Orthopedic surgery may be consulted to assess the status of the hips, legs and feet.

The nerves that supply the bladder and bowel can be impaired in an infant born with myelomeningocele, causing them to have a neurogenic bladder and bowel. Management of neurogenic bladder usually begins very early in life. Testing of bladder function in the newborn intensive care unit often leads to recommendation for intermittent catheterization to begin in infancy and continue lifelong. Parents are taught how to do this prior to the infant being discharged from the newborn intensive care unit if this recommendation is made by the urology team. Management of neurogenic bladder can also include medication and even surgery later in life with the goal being social continence, which can be achieved for the majority of patients. Likewise, social continence of bowel can be achieved for the majority of individuals with a comprehensive bowel management program, which often includes laxatives, suppositories in young children, enemas and surgical procedures in some patients.

Our team is specially trained on this diagnosis and is expert in the care of babies with myelomeningocele and its associated diagnoses. We know it can be a scary time, and we have a team of medical professionals available to answer any questions or discuss any concerns you might have.

You will receive specific discharge instructions tailored to you and your child’s needs. All children with myelomeningocele are ideally followed in a multidisciplinary clinic where they can be monitored for any deterioration in function as they get older. If this happens, the reason can quickly be diagnosed and treated. From a neurosurgical perspective, this would include problems such as shunt malfunction or infection or re-tethering of the spinal cord. Being close to a medical center with a neurosurgeon capable of treating these types of emergencies is often an important factor for families when deciding where to establish care for a child with this diagnosis.

The baby will remain on their side or belly for roughly 5 – 7 days after surgery. The baby can be held and breastfed or bottle fed when ready. Pain medicine will be given as needed to keep the baby comfortable. 

The baby may be referred for physical and occupational therapy consultations after discharge from the hospital depending on the limitations of their function.