Surgery for Spinal Deformity in Marfan Syndrome

Johns Hopkins University, Baltimore, MD
and Children's Hospital of Philadelphia, PA, USA

PURPOSE:
To describe the results, complications and unique features of spine deformity surgery for Marfan syndrome.

METHODS:
We reviewed the results of all patients (41 in all) having surgery for spine deformity at two hospitals over the past fifteen years. We separately analyzed the results for each type of deformity. There were 25 patients having surgery for scoliosis with a mean age of 15 + 6 years, 7 with kyphosis (7 of them males; mean age of 21 + 12 years) and 3 with spondylolisthesis (mean age, 23 + 16 yrs) and 6 with late revision for deformity with osteotomy (mean age, 22 + 14 yrs). Radiographs and records were reviewed, and a pain and function questionnaire was administered. Minimum follow-up was two years.

RESULTS:
The scoliosis curves were corrected from a mean of 64 + 25 degrees by 60% initially and 48% at follow-up. Blood loss was a mean of 2400 cc. Complications included dural tears and deep wound infections in 3 patients each (12%), pseudoarthrosis, instrumentation failure and lamina fractures in two patients each (8%). Two patients had additional vertebrae added onto the scoliosis curve postoperatively and three had distal junctional kyphosis. The spondylolisthesis was corrected from 70% preoperatively to 10% postoperatively. Two of them had a dural tear and two, a symptomatic delayed union. The patients who had a late revision with osteotomy had a mean blood loss of 5300 cc and a mean initial correction in the coronal plane of 36% and in the sagittal plane of 11%; these were 28% and 11% at follow-up. There was one death, one neurologic deficit and one deep infection.

CONCLUSIONS:
Additional postoperative deformity outside the fused region appears to be a unique feature of this disorder, despite fusion levels ending in the stable zone. Avoiding major correction and short fusion levels may help to minimize this risk. The blood loss and rates of dural tear and deep wound infection were greater than commonly reported for idiopathic deformity.