MULTISEGMENTAL FUSION OF SCOLIOSIS IN DUC HENNE MUSCULAR-DYSTROPHY

Operations in scoliosis in patients suffering from an advanced stage o f Duchenne muscular dystrophy are associated with a higher risk due to the extent of the curves, the respiratory insuffiency and frequent ca rdiomyopathia. Progressive scolioses in 20 wheelchairpatients with an age between 10.5-18.3 years (mean 14.6 years) were treated by CDI. The mean preoperative angle in this group was 70.6-degrees, the postopera tive angle 31.2-degrees (mean correction 39.4-degrees or 55.8%). The p reoperative lordosis of the lumbar spine (mean angle 4.1-degrees) was corrected to 17.8-degrees postoperativly. The average intraoperative b lood loss (2300 ccm) was evident more compared with idiopathic scolios is. One neurologic complication (postoperative disturbance of bladder function) was observed. Nowadays early surgical correction and stabili zation (Cobb angle > 20-degrees) has to be recommended as the treatmen t of choice for scolioses in Duchenne muscular dystrophy using multise gmental instrumentation methods to enable rapid mobilisation and a pos toperative care without brace or cast. This conception allows a prophy lactic operation including the following targets: prolongation of life expectancy, improvement of sitting position and prevention of rapid d eterioration of lung function including assisted mechanical ventilatio n in late stages.