Abstract
BackgroundSpinal deformities in Ehlers-Danlos syndrome are usually progressive and may require operative treatment. There is limited number of studies describing late results of surgery in this disease.MethodsThis is a retrospective study of the records of 11 patients with Ehlers-Danlos syndrome type IV, treated surgically between 1990 and 2007. All patients underwent surgical treatment for spinal deformity. Duration of operation, type of instrumentation, intraoperative blood loss, complications and number of additional surgeries were noted. Radiographic measurement was performed on standing AP and lateral radiographs acquired before surgery, just after and at final follow up.ResultsThe mean follow up period was 5.5 ± 2.9 years (range 1-10 years). The mean preoperative thoracic and lumbar curve were 109.5 ± 19.9° (range 83° - 142°) and 75.6 ± 26.7° (range 40° - 108°) respectively. Posterior spine fusion alone was performed on 6 patients and combined anterior and posterior fusion (one- or two stage) on 5 cases. Posterior segmental spinal instrumentation was applied with use of hooks, screws and wires. The mean postoperative thoracic and lumbar curve improved to 79.3 ± 16.1° (range 56° - 105°) and 58.5 ± 27.7° (range 10° - 95°) respectively, with a slight loss of correction during follow up. The average thoracic and lumbar correction was 26.4 ± 14.9% (range 5.3 - 50.4%) and 26.3 ± 21.2% (range 7.9 - 75%). Postoperatively, the mean kyphosis was 79.5 ± 40.3° (range 21° -170°), and lordosis was 50.8 ± 18.6° (range 20° -79°). Hyperkyphosis increased during follow up while lordosis remained stable. Mean Th12-L2 angle was -3.5 ±9.9° (range -19° - 15°) postoperatively and did not change significantly during follow up.ConclusionsHuge spinal deformities in patients with Ehlers-Danlos syndrome require complex and extensive surgery. There is a big risk of sagittal imbalance in this group.
Highlights
Spinal deformities in Ehlers-Danlos syndrome are usually progressive and may require operative treatment
In addition we evaluated late results of operative treatment
Spine correction without fusion according to Moe was applied in 5 cases. These patients underwent 3-6 operations with additional distraction before the main surgery. This is a retrospective study of the records of patients with Ehlers Danlos syndrome type IV, treated surgically between 1990 and 2007
Summary
Spinal deformities in Ehlers-Danlos syndrome are usually progressive and may require operative treatment. There is limited number of studies describing late results of surgery in this disease. Ehlers-Danlos syndrome is a group of different inherited diseases caused by various defects of collagen metabolism [1]. It is characterized by joint hypermobility and joint laxity. Surgery is much more difficult due to stiff deformity and is connected with significant risk of serious complications [5,6]. That is why individual approach to the treatment of these patients may be advisable. There are limited number of studies describing late results of operative treatment in patients with Ehlers Danlos syndrome
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