How is it treated and managed?
There are several things that can be done to prevent constipation and soiling. Children should be given a high fibre diet to help regular bowel function. Between 2 ½ and 4 years of age children should be encouraged to sit on the potty after every meal. If bowel control is not achieved after several months, oral laxatives or regular rectal suppositories or enemas can be tried. If this is not successful continence may be achieved by using a surgical technique such as creating an opening in the abdominal wall to the colon to allow irrigation of the colon on a regular basis with an antegrade – or forward flowing – colonic enema (ACE). Parents and children may require help with managing the stoma.
Biofeedback training may be used to achieve bowel control in a small number of older children with sacral meningomyeloceles. In this, a pressure sensor is placed in the rectum and connected to a visible pressure monitor.
If the child develops symptoms from the Chiari II malformation, treating any associated hydrocephalus may resolve the problem. If it does not then a surgical operation is performed. This is usually an operation to decompress the cerebellum within the skull and restore the flow of CSF, but may be an operation to untether the spinal cord at the level of the spina bifida allowing the tension in the cord to be released and the cerebellum to disimpact. Surgery usually stops progression of the symptoms but improvement is not guaranteed. Some symptoms are more likely to improve with surgery than others – pain and headaches are most likely to improve, weakness and sensory problems are least likely to improve.
Learning disability is managed by a combination of educational provision, psychological and drug treatments.
Seizures usually respond well to anti-epileptic medication.
Treatment involves surgical insertion of a shunt which drains CSF from the lateral ventricle of the brain to another place in the body, most commonly the abdomen (ventriculoperitoneal shunt). For further details see Hydrocephalus guidance.
Because children with meningomyelocele have complex physical and emotional needs, management should be carried out by a multidisciplinary team. This team includes paediatric doctors (general physicians, community physicians, neurologists, neurosurgeons, orthopaedic and general surgeons, urologists and endocrinologists etc), specialist nurses, occupational therapists, physiotherapists, orthotists and teachers.
Early exposure to latex should be avoided if possible. Surgery should take place in a latex free environment. Latex free catheters and gloves should be used. Toys that contain a significant amount of latex, such as rubber balls and balloons, and dressings that contain latex, should be avoided.
Physiotherapy and orthotics are used to treat both primary (from birth) and secondary musculoskeletal problems.
Club foot is usually treated by serial casting during the first 3 to 4 months of life followed by surgical correction between 4 months and 1 year of age. Other ankle and foot deformities may require surgical correction to allow the child to wear shoes. Hip deformities may be surgically corrected in order to improve mobility. Children should receive regular calcium and vitamin D and be encouraged to undertake weight bearing activities whenever possible in order to reduce the risk of osteoporotic fractures.
Scoliosis (curvature of the spine) may require orthotic support or surgical intervention depending upon the severity of the deformity. Kyphosis (hump back) may also require surgical intervention.
Some children have tethering of the spinal cord which may lead to rapidly progressive scoliosis. Surgery to untether the cord may halt or reverse progression of the scoliosis. It is important to identify tethering of the cord before carrying out spinal fusion, because it is more difficult to carry out surgery on the spinal cord following spinal fusion. If scoliosis is secondary to a Chiari malformation, treatment of the Chiari malformation may lead to an improvement in the scoliosis.
General preventative measures include avoiding tight fitting shoes or braces, avoid crawling on rough surfaces and regular checking of skin surfaces for abrasions and sores.
Pressure sores can be avoided by children in wheelchairs by adapting the seating system, for example by using special foam filled cushions. Children should be taught to perform regular pushups and to regularly change their position.
Established pressure sores are treated by alleviating the pressure on the sore and applying appropriate dressings. Infected pressure sores may require treatment with antibiotics. Children with large pressure sores that fail to heal may require admission to hospital for surgical debridement (removal of dead tissue) of the wound, skin grafting and intravenous antibiotics when necessary.
Children require support and encouragement from adults. They should be encouraged to manage their own condition whenever possible in order to achieve maximum independence. Depression should be treated as per normal.
Surgical closure of the defect is undertaken shortly after birth to prevent infection of the spinal cord and protect the spinal cord and nerves from subsequent physical injury. In addition, if the child has hydrocephalus, a shunting procedure is often required shortly afterwards to prevent cerebrospinal fluid (CSF) accumulating and causing progressive hydrocephalus.
If cystometry demonstrates raised pressure in the bladder, this needs to be reduced. Treatment is usually by way of clean intermittent catheterisation (CIC) in which the parents are taught to insert a clean catheter into the bladder at least four times a day to drain urine. This may be started soon after birth. Sometimes this is not successful and an operation called a vesicostomy is performed, in which an opening in the abdominal wall into the bladder is created so that urine can drain directly into the nappy. This is usually a temporary procedure and when the child is older the vesicostomy is closed and the child begins a CIC programme.
Confirmed urinary tract infections require treatment with appropriate antibiotics. If these occur frequently, long term preventative (prophylactic) antibiotics should be given either by mouth or instilled directly into the bladder through the catheter.
At age 3 to 4 the child and parents are taught CIC in order to try and achieve continence. Certain medications can be given by mouth or instilled into the bladder to improve the storage capacity of the bladder and help with continence. About 70% of children treated with CIC and medication achieve continence during primary school years.
If CIC and medication are not successful in achieving continence, there are a number of surgical procedures that can help.
Alternatively “volitional voiding” can be tried in which an artificial sphincter is placed around the urethra and is attached to a bulb which is placed under the skin in the genital area. When the bulb is squeezed the sphincter relaxes and urine is able to drain. This is however, only suitable for a small number of highly motivated people because if the person forgets to “void” regularly, the bladder pressure rises and this risks subsequent kidney damage.
Squint often requires surgical correction.
Weight and stature abnormalities
Children should be encouraged to be exercise regularly and eat a healthy diet.
