Inherited
disorders
Friedreich’s
ataxia
This is hereditary autosomal recessive. The child presents in early
childhood with both a motor and a sensory loss presenting as clumsiness and
difficulty walking. From an orthopaedic point of view the main problem is a
cavovarus deformity of the foot, which in the young child can be corrected with
soft-tissue release but in the older adolescent may require a wedge osteotomy.
Duchenne
muscular dystrophy
This is cross-link recessive and again presents in early childhood with
difficulty walking and classically with difficulty rising from a sitting
position and climbing stairs. There are high levels of serum creatinine kinase
and the muscle biopsy is diagnostic. In their early teens patients are
wheelchair bound and they normally die in their early twenties. The main input
from orthopaedics is correction of scoliosis to improve breathing.
Multiple
sclerosis
This is a patchy demyelination of the central nervous system which
develops later in life. The patient develops spasticity in the lower limbs with
gross deformities. Splintage should avoid these causing too much trouble but
tenomities are frequently required round the hip and knee to help the patient to
sit.