Changes in thoracolumbar kyphosis in achondroplasts

Achondroplasia is characterized by a defect in the cartilage rebuilding. As a
result, not only shortening of the limbs, but also the anatomy of the spinal
column is abnormal. This predisposes to all sorts of problems. One of these
problems is the frequent occurrence of a thoracolumbar kyphosis (TLK) which can
lead to compression of spinal cord and / or cauda equina. This can lead to
motor and sensory disturbances in the legs and micturition and pain. This
research is focused on this kyfosis identification and optimal treatment
strategy for ensuring the prevention of progression of kyphosis.

The transition from the thoracolumbar spine normally does not exhibit a
kyphotic angle. In children with achondroplasia, most seem to be born with a
kyphotic angle of the thoracolumbar transition. A retrospective study that we
conducted using a database of 105 achondroplastic children of the orthopedic
department of the UMCU showed that 68% of the children had TLK.
The achondroplast children who were reported to the orthopedic department were
checked for the presence of these TLK and a treatment protocol was instituted.
This protocol consisted of avoiding the upright position until the child sits
by itself. Furthermore, the kyphosis was frequently checked and a redressing
corset was applied if the kyphosis angle exceeded 30 degrees. Retrospective
analysis of the database showed that in about half of the children TLK
disappeared (with or without therapy) and that in the other half of patients
TLK deteriorated and that in half of those patients bracing or surgery was
required.

Long term follow up is lacking in this database. A follow up study in which
patients in the database are invited to a follow up exam and imaging would be a
much more complete picture of the development of TLK in achondroplasten.

In the LUMC adult achondroplasts with symptoms of spinal canal narrowing in the
high lumbar and low thoracic transition are frequently seen. TLK often occurs
in these patients. This study will place TLK in a context in which anatomy of
the spine and in particular sagittal balance and pelvic incidence of
achondroplast, are further studied. It is suspected that sagittal balance and
pelvic incidence (terms to the anatomy and distribution of forces in the spine
in size and number to indicate) the achondroplast correlated with symptom
pattern. These parameters would even have prognostic value.

question:
How does TLK develop as the child gets older?
Is there a correlation between clinical symptoms and TLK?
Does progression of TLK exists in the adult population?
Is the presence of TLK correlated with an increased number of surgical
interventions on the spine?
Is sagittal balance affected by the thoracolumbar kyphosis?
Is pelvic incidence influenced by the thoracolumbar kyphosis?

There will be an x-ray. This gives a low radiation dose