H. Chataigner et al., SPINE SURGERY IN DUCHENNE MUSCULAR-DYSTRO PHY, Revue de chirurgie orthopedique et reparatrice de l'appareil moteur, 84(3), 1998, pp. 224-230
Purpose of the study The authors present a retrospective review of 27
patients presenting a Duchenne muscular dystrophy and who were operate
d for spinal deformity, with special reference to functional result an
d postoperative evolution of vital capacity. Material and methods Age
at surgery averaged 14. Mean scoliotic angulation was 42 degrees. A th
oraco-lumbar kyphosis was present in 15 cases (kyphotic index less tha
n 10 degrees). A pelvic obliquity averaging 17 degrees was associated
in 19 cases. Mean pre-operative vital capacity was 56 per cent. Preope
rative evolution of vital capacity was documented in 18 cases : annual
rate of decrease was 4,3 per cent. Heart ejection fraction averaged 6
3 per cent in 23 cases, and was normal in 4 cases. Instrumentation was
extented from D3, D4 or D5 to L5 (5 cases) or S1 (22 cases). Spinal f
ixation was done in all patients by subliminar wiring with Luque rods
(5 cases) or Hartshill rectangle (22 cases). Sacral fixation was done
with ilio-sacral screws linked to the rectangle by Cotrel Dubousset ro
ds and dominos (15 cases). Results Mean blood loss was 1750 cc. Postop
eratively, 25 patients were extubated on the operative day, 1 patient
at D+1, and one patient underwent a tracheostomy after one month. Scol
iosis was reduced to 10 degrees after surgery and 13 degrees after 30
months follow-up, Pelvic obliquity was reduced to 4 degrees after surg
ery and 7 degrees after 30 months. A good spinal balance was present a
fter surgery in 20 patients; at follow-up, a coronal or sagittal imbal
ance averaging 40 mm was observed in 22 patients. Postoperative evolut
ion of Vital capacity was documented in 21 cases. The annual decrease
rate was 6,4 per cent. Rate was higher in patients presenting a good p
reoperative vital capacity (over 70 per cent) and very low In patients
presenting a preoperative vital capacity under 40 per cent. 10 patien
ts were deceased at review after a mean 53 months survival, at a mean
age of 19. 17 patients were alive with a 50 months follow-up. Discussi
on Spinal surgery in Duchenne muscular dystrophy has a low morbidity.
It allows to keep sitting position to the child and to preserve qualit
y of life. Surgery should be considered as soon as frontal or sagittal
collapse of the spine is observed. However surgery does not result in
respiratory improvement nor in life duration lengthening.