Congenital malformations and scoliosis of the spine

Background

Neuromuscular scoliosis mainly affects children and young adults, but can also appear at a later stage in adults. The disease is a special challenge for those affected and their families. Causes of this disease are congenital or acquired neurological disorders (cerebral palsy, myelomeningocele, poliomyelitis, etc.), muscular disorders (muscular dystrophies), or connetive tissue diseases (Marfan's syndrome etc.). Malpositioning of the upper body (poor upper body posture) and pelvis are distinct, when there is a lack of muscle control.

Problem

The following case concerns a 10-year-old girl. The patient has severe brain damage since birth. It is therefore not possible for her to communicate orally. Due to spastic paralysis of her arms and legs, she is dependent on the care of her family and therapists. It is difficult for the child to sit in a wheelchair. An x-ray image of her spine shows the extent of malformation (Fig. 1.1) - the patient literally collapses into herself. As a result, her daily care is made more difficult, and it is assumed that the situation will cause additional problems to other vital organs (heart, lungs, abdominal organs) as well as contribute to a shortened life expectancy.

Solution

The course of the disease, all preliminary findings, and the results of the physical examination are discussed together with the parents. The decision to undergo spine surgery is not easy for them. The treatment goal is discussed and involves correction of the deformity and upper body, so that sitting upright in a wheelchair is again possible. The patient is admitted to the hospital several days before surgery to complete all preliminary examinations. The operation lasts several hours. For better monitoring, the patient will spend the first night in the intensive care unit and return to the paediatric ward in the morning in a stable condition. Until hospital discharge, another six days elapse without any unforeseen problems. The x-ray shows the result of long-range correction and stabilisation of the thoracic and lumbar spine with modern implants, so that the patient’s upper body is now straight and she can independently sit in a normal upright position (Fig 1.2).