Purpose: To investigate surgical outcomes and complications of scoliosis associated with Marfan syndrome.
Methods: Inclusion criteria were patients who were 10-20 years of age, had a diagnosis of Marfan syndrome by the Ghent nosology, had scoliosis and had undergone spinal fusion, and had at least 2 years of postoperative follow-up. The medical records of all patients were reviewed for age at the time of surgery, surgical procedures performed, instrumentation type, estimated blood loss (EBL) during surgery, operation time and complications related to surgery. Health-related quality-of-life measures (obtained with the SRS-22 Questionnaire before operation and at the last clinical follow-up) were also recorded. Patients were analyzed as two different groups, Group 1 and Group 2, according to the different approaches employed. Patients receiving combined anterior and posterior surgery were assigned to Group 1 and those who received posterior-only surgery to Group 2.
Results: Group 1 consisted of 30 patients (14 males, 16 females) with a mean age at surgery of 16.8 years (range: 10-20 years). Complications in Group 1 included two cases of instrumentation loosening with one removed, one case of instrumentation breakage and one case of chylothorax and hemothorax during video assisted thoracoscopic release. 66 patients (28 males, 38 females) with a mean age at surgery of years 16.4 years (range: 10-20 years) were included in Group 2. Complications in Group 2 included six cases of cerebro-spinal fluid leak, one case of deep wound infection secondary to cerebro-spinal fluid leak, one case of leg weakness and one case of pleural rupture cause by misplacement of pedicle screw. There is no difference of age at surgery, preoperative Cobb angles, and SRS-22 total scores (3.0 vs. 3.1) between the two groups (P > 0.05). Group 1 yielded larger correction rate than Group 2 for both thoracic (62.5 % vs. 56.2 %) and lumbar scoliosis (68.3 % vs. 62.7 %). Loss of correction was similar between the two groups for both thoracic (4.1° vs. 3.6°) and lumbar (4.2° vs. 4.6°) curves (P > 0.05). EBL (1972 ml vs. 1530) and operation time (669 min vs. 419 min) were significantly increased in Group 1 than in Group 2 (P < 0.05). No difference was noted for SRS-22 total scores in the last follow-up between the two groups (3.7 vs. 3.8, P > 0.05).
Conclusion: In conclusion, as compared to anterior release combined with posterior spinal fusion, posterior-only spinal fusion could yield comparable clinical outcomes for scoliosis associated with Marfan syndrome with lower EBL, and operation time. However, longer follow-up is needed to verify these findings.
Keywords: Approach; Marfan syndrome; Scoliosis; Surgical treatment.