Clinical and radiological data were reviewed for all patients
with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed
non-operatively or operatively in our institution. In all 16 patients were included (eight female: eight male; 50%
male), of whom nine had Hurler, five Morquio and two Hunter syndrome.
Six patients were treated non-operatively (mean age at presentation
of 6.3 years; 0.4 to 12.9); mean kyphotic progression +1.5o/year;
mean follow-up of 3.1 years (1 to 5.1) and ten patients operatively (mean
age at presentation of 4.7 years; 0.9 to 14.4); mean kyphotic progression
10.8o/year; mean follow-up of 8.2 years; 4.8 to 11.8)
by circumferential arthrodesis with posterior instrumentation in
patients with flexible deformities (n = 6).Aims
Methods
Acute angulation at the thoracolumbar junction
with segmental subluxation of the spine occurring at the level above
an anteriorly hypoplastic vertebra in otherwise normal children
is a rare condition described as infantile developmental thoracolumbar
kyphosis. Three patient series with total of 18 children have been
reported in the literature. We report five children who presented
with thoracolumbar kyphosis and discuss the treatment algorithm. We
reviewed the medical records and spinal imaging at initial clinical
presentation and at minimum two-year follow-up. The mean age at
presentation was eight months (two to 12). All five children had
L2 anterior vertebral body hypoplasia. The kyphosis improved spontaneously
in three children kept under monitoring. In contrast, the deformity
was progressive in two patients who were treated with bracing. The
kyphosis and segmental subluxation corrected at latest follow-up
(mean age 52 months; 48 to 60) in all patients with near complete
reconstitution of the anomalous vertebra. The deformity and radiological
imaging on a young child can cause anxiety to both parents and treating
physicians. Diagnostic workup and treatment algorithm in the management
of infantile developmental thoracolumbar kyphosis is proposed. Observation
is indicated for non-progressive kyphosis and bracing if there is evidence
of kyphosis and segmental subluxation deterioration beyond walking
age. Surgical stabilisation of the spine can be reserved for severe
progressive deformities unresponsive to conservative treatment. Cite this article:
