Longterm Care and Outcome in Open Myelomeningocele

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The long-term care and follow-up of children with myelomeningocele requires the input of many disciplines, preferably in a combined multidisciplinary clinic devoted to the management of children with spina bifida and its complications. This will include assessment of all aspects of physical, as well as cognitive, development.

Overall survival with myelomeningocele after the first 4 years appears to stabilize at approximately 85% [6], although there continues to be a significant mortality. This can be related to many of the associated problems faced by these children, including hind-brain disturbance, causing respiratory distress, apneic spells and gastro-esophageal reflux with tracheal aspiration, shunt malfunction and infection, and bladder and renal problems.

Myelomeningocele itself does not appear to have a significant impact on intelligence. Although the average IQ of affected children is below the mean, the majority fall within the normal range, with only 9% having an IQ score below 70 in McLone's series, followed up for over 15 years. Factors which do seem to affect intelligence include initially severe hydro-cephalus, neonatal ventriculitis and shunt infections, as well as the level of the lesion. Hydrocephalus and Chiari malformations presumably also account for the consistently found deficits in fine motor function in the upper limbs and in visuo-spatial processing, as well as changes in age-related language ability. Two-thirds of children, with appropriate support, can be kept in mainstream education. Ultimate employment opportunity seems to be related more to intellectual ability than to physical disability [9].

Numerous factors contribute to mobility. The ability to walk will depend not only upon the level of motor and proprioceptive deficit, but also on factors such as spasticity, deformity, ataxia and obesity, as well as intelligence, support and motivation. In McLone's series, 75% of surviving children by school age were mobile without a wheelchair; nevertheless this figure declines with age [10], largely due to increasing difficulty and effort required to maintain posture, especially with weight increasing proportionately faster than strength.

Again, bladder and bowel continence depends chiefly upon the level of the neurological lesion. Most children manage the bladder with clean intermittent catheterization and many are able to perform this for themselves. Management of the bowel depends upon a combination of bulk laxatives and enemas, avoidance of constipation and the use of a Shandling catheter [11].

The long-term care and assessment of children with myelomeningocele requires attention to many details of their development, with particular scrutiny for the possible complications which may arise in time. From a neurological perspective, aside from the ever present risk of shunt malfunction and Chiari malformation and syringomyelia-induced problems, this will include the possibility of cord re-tethering.

Re-tethering of the spinal cord (see below) following closure of a myelomeningocele or after any other "untethering" operation, such as after surgery for lipomeningocele, should be considered in any child with a clinical deterioration. Re-tethering may manifest as a deterioration in motor power or gait, pain, altered sensation or deformity in the legs, or with changes in bladder function. Increasing scolio-sis has also been considered to result from re-tethering.

Most neural placodes or lipomas probably adhere to the dura soon after the initial operation; however, it is only with continued traction on the cord that symptoms are likely. Re-tethering remains a clinical diagnosis, since static MRI will only show the location of the conus, rather than its mobility.

Re-operation to release the cord should be considered in any child with a significant clinical deterioration. There are often dense adhesions and thick scar, making surgery difficult, and consideration to expanding the dura with a patch should be given. The results of surgery are reported to be gratifying in that most children can be stabilized and pain is often improved [12].

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