7 Facts about Myelomeningocele Repair & Treatment
Myelomeningocele is the most complex anomaly of the central nervous system that is present at birth and still compatible with life. Also, this condition is the most common type of spina bifida. It has been observed that failure of the normal folding of the neural tube during the first trimester of pregnancy gives rise to this congenital malformation. Children born with this condition have abnormal bone development in their spine that results in incomplete formation of the spinal canal. In fact, the spinal cord and menengies stick out of the child’s back.
The exact cause of this disorder is still under medical discussion. It occurs almost once in every 1000 live births. There is geographical variation in the incidence of this malformation1. Other congenital malformations like syringomyelia and hip dislocation may also be present with this disability. Hydrocephalus is present in almost 90% of children with this anomaly. It can occur along any portion of the spine; however sacral myelomeningocele is most common variety1,2,5.
The exact cause of this malformation is still not known. However, the following factors may be associated with this malformation5:
- Low level of folic acid in the female in late pregnancy
- Possible family history
- Radiation exposure
- Some viral illness
It can present as a sac sticking out of the lower back in the newborn baby. The sac is not transluminant (i.e., one cannot see through the sac when shining a light behind it). Patients with condition may present with the following signs and symptoms:
- Loss of bladder control
- Loss of bowel control
- Partial or complete loss of sensations
- Partial or complete weakness of lower limbs
- Weakness of the hips in the newborn
- Abnormality of feet such as clubfoot
- Presence of extra CSF inside the skull, i.e., hydrocephalus
- Hair seen at the lower back
- Dimpling of lower back1,2,3
Several investigations can be performed to reach the diagnosis of this congenital ailment.
Prenatal screening is helpful in diagnosing this malformation.
A screening blood test called “quadruple screen” is performed during the 2nd trimester of pregnancy for diagnosis of this malformation as well as for the diagnosis of Down syndrome and other congenital defects in the baby. When the quadruple screen is positive then the following tests are performed to confirm the diagnosis4, 5:
- Ultrasonography of the abdomen
Alfa fetoprotein level may be normal or elevated.
Tests which are performed on the baby with this congenital anomaly after birth include:
- X-Rays of the spine
- CT scan of the involved area
- MRI of the affected portion4,5
Genetic counseling may be needed. When the severe defect is diagnosed early in the pregnancy, a therapeutic abortion may be recommended. After birth, repair surgery may be required at an early age. Before surgery the infant must be handled in such a way as to reduce the damage to the exposed spinal tissue. The strategy may consist of good care and special positioning, protective devices, and modification in methods of handling, feeding, and bathing. Babies with hydrocephalus may require a ventriculo-peritoneal shunt placed. Antibiotics may be required for prevention of meningitis. Most of children will need lifelong modifications for complications that can result from damage to spinal nerves3, 4. This includes:
- Downward pressure over the bladder may drain the bladder. In severe cases drainage catheters are required. High fiber diet may improve bowel function.
- Orthopedic or physical exercises may be required to treat musculoskeletal related symptoms. Braces are required for muscle and joint problems.
- Neurological deficits can be treated according to the type and severity of function loss3, 4.
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Mary Jo Dunleavy, RN, Nurse Coordinator in the Spina Bifida Clinic at Boston Children’s Hospital, talks about a controlled study that focuses on prenatal surgery to treat myelomeningocele in fetuses
Repair surgery can be performed for Myelomeningocele. Steps of this procedure include:
- Defect is covered with sterile dressing after birth
- Shifting the baby to a neonatal intensive care unit
- Get MRI spine and MRI of brain
- If the defect is not covered by skin after birth, surgery can be performed within 24-48 hours after birth
- Steps of surgery include reposition of the involved area, removal of protrusions, correction of deformities and relieving the CSF pressure by placing a shunt
- Give the baby good antibiotic coverage
- Child must be hospitalized for 2 weeks after surgery
- Child must lie flat
- Repeat MRI of the brain after surgery to look for hydrocephalus
- Later on introduce physical, occupational and speech therapy3,4
Complications of surgery: these include:
- Increased chance of urinary tract infection
- Bowel problems
- Infection or inflammation of the spinal cord
- Paralysis, weakness, or sensation changes due to nerve injury4.
Dr. Wetjen discusses surgery for in utero myelomeningocele repair verse postnatal repair.
Prognosis depends upon the initial condition of spinal cord and spinal nerves. After an early repair, prognosis may be excellent. Late repair has a poor prognosis with irreversible motor and sensory deficits4, 5.
- Gaskill SJ. Primary Closure of Open Myelomeningocele. Neurosurg Focus. 2004;16(2):E3.
- Ferschl M, Lee H, Rollins MD. Anesthesia for in utero repair of myelomeningocele. Anesthesiology 2013;118(5):1211-23.
- Meningocele repair. [internet]. [last updated 2011 Sep 2; cited2013 Aug 20]. Available from: http://www.nlm.nih.gov/medlineplus/ency/article/003020.htm.
- Nejat F, Baradaran N, Khashab ME. Large myelomeningocele repair. Indian J Plast Surg 2011;44(1):87–90.
- Adzick NS, Walsh DS. Myelomeningocele: prenatal diagnosis, pathophysiology and management. Semin Pediatr Surg 2003;12(3):168-74.