Case by Case

Scenario

Bryonie is now eight years old. She has a complex congenital deformity of her neck and thoracic spine which has rapidly deteriorated as she has grown.

Problem

Traditional methods of stabilising spine deformities involve instrumentation, correction of the deformity and a fusion. In young children, however, a fusion will prevent further growth of their spine. Instrumentation and correction at the base of the neck poses significant risks in terms of spinal cord function.

Assessment

Deformities are best understood with three dimensional reconstructions of CT images as well as bio-modelling, a technique used and studied by our unit over the last five years or so. This allows a real time understanding of the deformity in three dimensions. Operative repair can then be based on the exact replica of her spine.

Operation

Bryonie had previously undergone posterior spinal surgery to try to prevent the deformity progressing. However, the deformity then progressed above the level of the previous fusion.

The plan for correcting her deformity has involved the principle of releasing the deformity followed by prolonged traction to steadily improve the curve. This is a far safer procedure than any attempt at a surgical, one stage correction, but involves a series of operations.

In Bryonie’s case the first operation was to break down the previous fusion. A Halo ring was then applied with screws into the outer cortex of the skull. Through the Halo ring it is possible to put traction with weights up to about 25 to 30% of her body weight. Specially designed wheelie walkers have been made by GB Orthopaedics at Mater Hospital to allow Bryonie to be ambulatory and still in traction. At night time the traction is suspended over the back of the bed.

A further anterior release procedure has been performed and it is proposed that she continue with increasing traction weight for approximately three months until there has been satisfactory correction obtained.

Thereafter, it is planned that instrumentation and fusion over the congenitally abnormal area of her spine be performed to maintain the correction. However, she will require a further three months with the Halo vest to ensure satisfactory healing of the bone graft.

Congenital Scoliosis

Congenital Scoliosis accounts for only 10% of all spinal deformities presenting to the spine deformity clinic. By and large, they tend to be relatively innocuous and benign with little scope for progression.

In some children, congenital scoliosis rapidly progresses with growth, particularly those in the upper thoracic spine. Here they may pose a threat both to the spinal cord—as it stretches across the deformity—and to the development and maturation of lung tissue. Apart from the significant cosmetic deformity associated with a cervico-thoracic kyphosis, there may be respiratory compromise followed by right heart compromise not to mention the risk of paraplegia.

Surgical procedures are aimed at preventing progression of the deformity in most cases. When the deformity is already severe, attempts at correcting the curve within the limits of safety—particularly as they relate to risk of paralysis—are performed. These procedures are complex and often involve multiple operations with prolonged periods of immobilisation in orthoses.

The ultimate goal is to have a stable spine with a residual deformity which is non-progressive and allows for satisfactory development of heart and lung tissue. Preservation of the spinal cord's function is imperative.