Pediatrics & Child Neurology, University Hospital Gasthuisberg, K.U.Leuven, Belgium
Our first aim is to provide useful information about the natural history of children born with spinal dysraphism and how to prevent more disability if the child survives (with or without surgery). As people working in rural rehabilitation know a lot about other movement disabilities (polio, cerebral palsy) and also about secondary or additional disabilities (contractures, spinal curve, developmental delay), we thought it would be good to build on what they already know and practice. So if they can learn about the specific needs of spina bifida children, they can help them a lot by sharing their experience disorders (polio, cerebral palsy) or sensory function disorders (lepra).
We realize that, for many families, the operations for closure of the back and for shunting will not be possible. Except where free hospital services are available they are very costly. Before deciding on surgery, there are several things to consider. What will the child’s future be like, if he lives? is he likely to suffer greatly, or might he have a chance to live a full and happy live, despite his limitations ? If the family spends much money on operations, and then on daily care of the child, how will this affect the health and wellbeing of the other children in the family?
In short, before deciding whether to operate, it is important to consider carefully how this may affect the quality of life for both the child and the family.
The initial management of a newborn with spina bifida is intended to minimize complications. The sac should be covered immediately with non-adhering sterile dressing and kept moist with sterile saline. This will minimize infection and injury to exposed neural elements. A thorough clinical examination is necessary to assess the anatomic and functional levels of defect, head configuration and the presence of other anomalies. From recent observations it is concluded that there is no urgency in surgical intervention for the initial management of newborns with myelomeningocele. No significant associations exist between time of surgery and development of ventriculitis, developmental delay or worsening of paralysis. Some studies even claim that there is a medical advantage in delaying the closure of the defect because of a lower incidence of hydrocephalus, thus deferring shunt operations until the child is immunologically more competent. It is an erroneous assumption that almost all babies with open spina bifida would die within a short period if not surgically corrected. Some studies reported a 10% to 30% survival rate due to the natural development of granulation tissue that seals the defect, if there is no infection. The presence of active hydrocephalus is the single most critical factor in survival. The main causes of death in affected neonates are increased intracranial pressure and infection of the central nervous system.
Progressive hydrocephalus can be monitored by neurological examination, developmental milestones and frequent head circumference measurements. It can be controlled with a shunt (ventriculocardial or ventriculoperitioneal). Some children with enlarged ventricles do not develop progressive hydrocephalus and can be managed without shunting. Others with overt hydrocephalus at birth will show very soon progressive hydrocephalus and they will die within a few weeks after closure of the back. But despite frequent complications (hydrocephalus or infection) 10-15% of infants with myelodysplasia will survive without shunting or antibiotics. Unfortunately the survivors often develop monstrous deformities and loss of intellectual skills, thereby presenting a formidable problem to their parents. Blindness is one of the first clinical consequences of uncontrolled hydrocephalus followed by progressive retardation and spastic deplegia. To prevent further and more disability, those children with spina bifida who survive after spontaneous closure of the back and with progressive – but not lethal – hydrocephalus need our help and support to find a possibility to get a shunt.
But closure of the back and shunting are only the first steps in a lifelong rehabilitation program.
What is the future for a child with spina bifida? This will depend first on how serious the defect is, next on medical treatment and general care and finally on special training and on family and community support. The higher up the back the defect is or the more severely the spinal cord is affected, the worse the paralysis and other problems are likely to be.
In spite of the best medical attention, at least 1 out of every 4 of 5 children born with severe spina bifida dies in the first months or years of life. However, the child with a defect that is low down on the back usually has less paralysis and has a good chance of living a full and happy life. With good family and community support, many children with spina bifida go to school, learn to do many kinds of work, get married and have children.
In all children with spina bifida functional goals should be established. These goals vary with the severity of motor and sensory defect, and with the child’s developmental progress. The goals of orthopedic management are to establish a stable posture with minimal flexion deformity (avoiding contractures) and to maximize mobility. Mobility implies more than walking and is essential to attain social maturation and educational and vocational goals. Most children with lesions below S1 can walk unaided, those with lesions above L2 are wheelchair dependent. The child with an intermediate lesion is capable of brace and crutch-assisted ambulation but often deteriorates in the absence of careful management.
For independent living, other skills are more important than walking and the family and child should place greater importance on these: skills like dressing, bathing, and toileting. Self-care in toileting is especially important and is more difficult because of the child’s lack of bladder and bowel control.
Often these children are late in learning basic skills for self-care. This is partly because of the disability. But it is also because their parents often overprotect them and do everything for them. It is important for parents to help these children to do more for them selves. A child with spina bifida usually does not develop the same bladder and bowel control as other children do. But most children can be helped to take care of both their bladder and bowel so that they stay relatively dry, clean and healthy. Therefore it is extremely important that rehabilitation workers and family members help the child work out a good bladder and bowel program. The goals of urologic management are to prevent renal damage by preventing infection and by treating outflow obstruction and to achieve social continence. The ideal method of urine control empties the bladder completely and in a clean, regular, easy and self-reliant way (clean intermittent catheterization).
The goal of bowel management is to prevent constipation and to achieve continence: the child can learn to help the stool come out, with assistance, certain times of the day. This kind of “bowel program” can greatly increase the person’s self-confidence and freedom for school, work and social activities.
Many children with spina bifida are paralyzed from the waist down. In spite of their disability it is important for them to develop their bodies, their minds and their social abilities as much as possible. Certain “adaptive aids” can be used to help paralyzed children go through the same stages of development as able-bodied children, at close to the same age.
In summary, we can say that the chances of a child with spinal dysraphism , leading a fairly normal life are good, provided that we can manage the shunt problems, and avoid important medical risks : skin problems (pressure sores), renal damage, and contractures
We can help the child to become more self-reliant : home training and encouragement to master basic self-help skills such as moving about, dressing, toileting.; by education: learning of skills that make keeping a household, helping other people and earning a living more possible.
The ultimate goal in managing a child with spina bifida or spinal cord injury is to achieve the greatest possible degree of self-reliance and dignity, both for the disabled child and for his/her family.