Short and Longterm Evolution of Self-care and Functional Mobility After Single Event Multilevel Surgery in Children and Adolescents With Spastic Diplegic Cerebral Palsy

1. Introduction Cerebral palsy (CP) is the most common physical disability with a prevalence between two and three per 1000 living births. Cerebral palsy had been defined as a group of permanent disorders of the development of movement and posture, causing activity limitations that are contributed to non-progressive disturbances that occurred in the developing fetal and infant brain. The motor disorders are often accompanied by disturbances of sensation, perception, cognition, communication and behavior, by epilepsy and by secondary musculoskeletal problems. Cerebral palsy can have an impact on the body function and on the performance of daily life activities and as such may affect the child's independence, participation and quality of life.

The impact of cerebral palsy on individuals varies widely. The SCPE describes a hierarchical classification tree of cerebral palsy subtypes: spastic CP, dyskinetic CP and ataxic CP. Spastic CP is the most common type and is seen in 85% of the children with CP. The Gross Motor Function Classification System (GMFCS) classifies children and adolescents with cerebral palsy in five levels on the basis of their functional abilities in sitting and walking. Further subdivision is made according to the topographical distribution i.e. hemiplegia, diplegia and quadriplegia. Howard et al. reported that in a group of 323 children with spastic cerebral palsy 35 % of children had hemiplegia, 28 % diplegia and 37% quadriplegia. In their study the GMFCS levels of spastic diplegic cerebral palsy were 33% with GMFCS I, 33 % GMFCS II, 28% GMFCS III and 5% GMFCS IV. This means that nearly all spastic diplegic patients have the ability to walk with or without assistive devices.

A multidisciplinary follow-up is necessary during growth of the child with CP. A good knowledge of normal developmental milestones and natural history of children with CP is important to detect and prevent early problematic situations. In normal development the period of early childhood is characterized by rapid development of locomotor skills as walking, running and stair climbing. Typically developing children learn the skill of walking between the ages of eight and eighteen months. Children further learn to talk, start potty training and start to develop their independence in self-care skills. Achieving independence in dressing may take up to four years. Tying shoelaces and completing all clothing fasteners is achieved at age of six years. During this time, parents gradually perform fewer of the tasks, and encourage their children to do more, with the ultimate goal of independence.

In comparison with typically developing children, a child with spastic diplegic CP will show delay in all milestones but will generally stand at eighteen months and most will walk independently by four years. In the self-care domain, over half of the children with cerebral palsy needed help or supervision in the areas of grooming, bathing, dressing, and toileting. The gross motor function and function of self-care skills is steadily improving. At the age of nine years most children with spastic diplegic CP reached their maximum functional potential. Öhrvall et al. described that many children with CP achieve independence but at a later age than typically developing children. They reported that children with high functioning levels in GMFCS and in the Manual Ability Classification System (MACS) could achieve good functional performance for self-care and mobility at the age of 12 years. For children with GMFCS and MACS level III and more only minor development of functional skills is seen with age, although task-orientated and goal-directed therapy can improve their functioning.

Despite the improvement in self-care with age, several studies reported that the gait pattern in children with CP is deteriorating. Due to increased muscle tone and weakness children with spastic diplegic CP walk with hip and knee flexion, inward rotation and toe walking, hereby secondary problems like muscle contractures and bony deformities slowly arise. Further during the adolescents year's rapid growth and increase of body weight can lead to worsening deformities and changes in ratio of body mass to strength. Decrease of this ratio has a negative effect on the gait pattern.