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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.

The purpose of our study was to analyze limb lengthening in fibular hemimelia type II. Ten patients underwent 16 tibia lengthenings. The mean tibia shortening was 5.8 cm. We used the Ilizarov technique in all cases. The mean follow-up time was 7.2 years. The mean lengthening was 23% of the former length. The healing index was 50.8 days/cm. In the final examination six patients were skeletally mature, equal limb length and functional foot positioning were achieved in four of them. Complications were observed during 14 lengthenings (87.5%). Although lengthening in fibular hemimelia is difficult, elongation with axis and foot correction may offer an alternative to amputation.

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surgery in caudal regression syndrome is challenging and bears high risk of complications.

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