Spina bifida, Pathology, Clinical features, Management.

 This is the condition that results from a developmental defect in the vertebral column resulting in a lack of fusion of the vertebral arches and so the vertebral canal is not closed. There may or may not be defects of the spinal cord and meninges, and in some cases there are little or no symptoms. 

Pathology:-There are two main types of this abnormalities, spina bifida occulta and spina bifida cystica.

Spinal bifida Occulta:-

This is the mildest form of defect and is observed in only a small percentage of children with spina bifida. The actual number may be larger because there may not be any obvious defect or abnormality externally. There is a defect in the fusion of the laminal arch but this may only be evident in X-ray. There is a dimple in the skin over the vertebrae, and sometimes there may be small tuft of hair over the area. A common feature of this type of lesion is abnormality of bladder control with either enuresis or urine retention.

Spina bifida Cystica:-

Meningocele:- The vertebral arch is not fused and a sac containing meninges and cerebrospinal fluid protrudes out.

Myelomeningocele:- this is the most severe form of spina bifida and inevitably there is neurological damage. Depending on the level of lesion, the protruding sac contains part of the spinal cord or the cauda equina. Because the cerebrospinal fluid does not flow properly due to obstruction, over 80% of children with this disorder have an associated hydrocephalus.

Clinical features:-

• There is an obvious lesion over the vertebral defect on the back.
• Muscle paralysis or weakness - This will depend on the levels and the extent of the lesions. If the upper motor neurons are affected there may be a spastic paraplegia; otherwise there is a flaccid paralysis.
• Sensory impairment can be variable depending on the extent and level of lesion.
• Rectal and/or bladder incontinence (neurological bladder) is very often troublesome complaint and the parents find it very difficult to send the child to school because of the continuous need for sanitary pads.
• Hydrocephalus may be an associated problem.
• Other congenital abnormalities of the lower limbs or spine (spina bifida or CDH) may be present.
• Mental retardation is not very common but could be present.

Management:-

• Surgery  may be necessary to repair the spinal defect and prevent further damage.
• Counseling of parents: Counseling and support for the family is an important aspect of management.
•  Education: Integration into a normal school is the goal whenever possible, but it must be after a careful assessment of the child to see whether he will be able to cope with the various aspects of school life.
• Physiotherapy: the problems that the physiotherapist may have to deal with are lack of movements or abnormal movement patterns. in addition. there are deformities, anesthesia over buttocks and heels, and psychological problems. The physiotherapist will try to prevent deformities from occurring by correct positioning and teaching parents. Passive movements will be carried out to prevent contractures.
• Phycological problems: There may be frustration, depression, irritability, or anger. If the reactions are severe the child may need help from a clinical psychologist or from a educational psychologist if there are learning problems.
• Functional activities: All treatment is designed to gain as much functional activity as possible. The physiotherapist must work with other members of the team, the child and the parents to achieve this.
• Orthosis: may be designed to train the patient's gait.