We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity.

We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis.

Survivors of infantile meningococcal septicaemia often develop progressive skeletal deformity as a result of physeal damage at many sites, particularly in the lower limb. Distal tibial physeal arrest typically occurs with sparing of the distal fibular physis leading to a rapidly progressive varus deformity. There have been reports of isolated cases of this deformity, but to our knowledge there have been no papers which specifically describe the development of the deformity and the options for treatment.

Surgery to correct this deformity is complex because of the patient’s age, previous scarring and the multiplanar nature of the deformity. The surgical goal is to restore leg-length equality and the mechanical axis at the end of growth. Surgery should be planned and staged throughout growth in order to achieve the best functional results.

We report our experience in six patients (seven ankles) with this deformity, who were managed by corrective osteotomy using a programmable circular fixator.