Introduction The development of scoliosis in neuromuscular diseases
is frequently seen in early childhood. Unfortunately, management of
scoliosis with braces or physical therapy is usually not successful and
leaves the child with a severe spinal deformity at maturity. The
treatment with telescopic VEPTR devices theoretically offers the
possibility to avoid rapid progression and the need for braces. It also
avoids the need for early spine fusion with the deleterious effects on
pulmonary growth.
Material and methods 13 Children, seven female and six male patients,
with an average age of 8 years and 8 months had an Eiffel tower VEPTR
construct performed due to progression of neuromuscular scoliosis.
Underlying diagnosis were myelomeningocele in six and muscular diseases
in five patients. One patient had cerebral palsy and another patient
had arthrogryposis. The average follow-up was 11 months.
Results Average time for surgery was 110 min. No blood transfusions
were required. Average hospital stay was 9 days. The Cobb-angel of
the main curve decreased from 62
before to 31
after surgery which
is a correction of 50%. Pelvic obliquity was corrected in all cases. No
complications during surgery were encountered. Immediate complications
after surgery included one wound slough in a patient with
spastic quadriparesis and one pneumonia in a patient with spinal
muscular atrophy and severe restrictive lung disease. Two patients
with myelomeningocele had urinary tract infections which resolved
after antibiotic treatment. Late complications included two broken
ilium hooks in one patient with myelomeningocele and flexible thoracolumbar
kyphoscoliosis and two proximal cradle migrations in
patients with myelomeningocele. These problems were revised during
the lengthening procedures which were scheduled every 6 months.
One patient with poor skin coverage suffered from a deep infection
which was revised successfully without removing the device. Patient
and parent satisfaction was very high in all cases.
Discussion VEPTR was devised as a non fusion technique especially
for congenital scoliosis. Recently it has also been recommended for
neuromuscular deformities. It is our strategy to apply a bilateral rib to
pelvis construct (Eiffel tower-construct) to patients with neuromuscular
spinal deformities who are not ambulatory. Our preliminary data
suggest that this approach offers a very good correction of spinal
deformities during growth with low morbidity and reasonable complication
rate. It may avoid performing early spine fusions and the
development of thoracic insufficiency syndrome.