Case Reports
doi: 10.3390/medicina58050605.
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Case Reports
Medicina (Kaunas).
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Abstract
Background and Objectives: Thoracolumbar kyphosis is one of the most frequent skeletal manifestations in patients with achondroplasia. Few papers have been published on the surgical treatment of this condition, especially in skeletally mature patients. With this study, we presented a retrospective case series of long-term surgical results for achondroplastic patients with severe thoracolumbar kyphosis. This study was conducted to evaluate the outcome of surgical treatment for thoracolumbar kyphosis in patients associated with achondroplasia presenting with paraparesis. Materials and Methods: Three patients with achondroplasia who developed neurologic deficits due to severe thoracolumbar kyphosis and underwent surgical treatment were evaluated (mean age 22.3 years; mean follow-up 9.3 years). All patients were treated with posterior vertebral column resection (p-VCR) of hypoplastic apical vertebrae with a cage and segmental instrumentation. Neurologic outcomes (JOA scores), correction of kyphosis, and operative complications were assessed. Results: All patients had back pain, neurological deficits, and urinary disturbance before surgery. The average preoperative JOA score was 8.3/11 points, which was improved to 10.7/11 points at the final follow-up (mean recovery rate 83%). All patients obtained neurologic improvement after surgery. The mean preoperative kyphotic angle was 117° (range 103°-126°). The postoperative angles averaged 37° (range 14°-57°), resulting in a mean correction rate of 67%. All patients had postoperative complications such as rod breakage and/or surgical site infection. Conclusions: The long-term results of p-VCR were acceptable for treating thoracolumbar kyphosis in patients with achondroplasia. To perform this p-VCR safely, spinal navigation and neuromonitoring are inevitable when resecting non anatomical fused vertebrae and ensuring correct pedicle screw insertion. However, surgical complications such as rod breakage and surgical site infection may occur at a high rate, making informed consent very important when surgery is indicated.
Keywords:
achondroplasia; long-term follow-up; navigation; vertebral column osteotomy.
Conflict of interest statement
The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results.
Figures
Figure 1
A 16-yearold girl with achondroplasia; preoperative figure and radiograms. (A,B) The patient’s posture shows dwarfism with short limbs and severe thoracolumbar kyphosis; (C) hands are three-pronged (trident) in appearance; (D,E) radiograms at initial visit; (F,G) lateral radiogram shows severe sagittal malalignment; T11-L2 local kyphosis 126°, SVA 55 mm, PT 32°, and PI 52°.
Figure 2
A 16-year-old girl with achondroplasia in preoperative state; preoperative CT and MRI. (A) Sagittal reconstruction CT image; (B) 3-D CT; (C) thoracolumbar sagittal T2-weighted MR imaging shows severe kyphosis and stenosis.
Figure 3
A 16-year-old girl with achondroplasia; intraoperative image and navigation.
Figure 4
A 16-year-old girl with achondroplasia; intra- and postoperative radiograms. (A) Flexible surgical table; (B–D) intraoperative images; (E,F) postoperative radiograms.
Figure 5
A 16-year-old girl with achondroplasia; final follow-up images. (A) Posteroanterior radiogram; (B) lateral radiogram; (C) coronal reconstruction CT. T11-L2 local kyphosis 14°; SVA 31 mm; PT 16°; PI 52°.
Figure 6
A 25-year-old man with achondroplasia; preoperative figure and radiograms. (A,B) The patient’s posture; (C,D) preoperative radiograms show T11-L2 local 123° kyphosis, SVA 62 mm, PT 25°, and PI 27°; (E) thoracolumbar sagittal T2-weighted MR imaging shows severe kyphosis and stenosis.
Figure 7
A 25-year-old man with achondroplasia; intra- and postoperative radiograms. (A,B) Intraoperative images; (C,D) postoperative radiograms.
Figure 8
A 25-year-old man with achondroplasia; final follow-up radiograms and CT. (A)Anteroposterior radiogram shows good coronal alignment with triple rod-fixation; (B) Lateral radiogram indicates acceptable local kyphosis (T11-L2 local 39° kyphosis, SVA 43 mm, PT 5°, PI 27°); (C,D) Final coronal and sagittal reconstruction CT shows solid bony fusion.
Figure 9
A 26-year-old man with achondroplasia; preoperative radiograms and CT. (A) Posteroanterior radiogram; (B,C) lateral radiogram (T11-L2 local kyphosis 103°, SVA 60 mm, PT 27°, PI 81°); (D) sagittal reconstruction CT; (E) 3-D CT.
Figure 10
A 26-year-old man with achondroplasia; preoperative MRI. (A,B) Thoracolumbar sagittal T2-weighted MR imaging; (C–F) axial T2-weighted MR images show severe stenosis at the T11/12 and L1/2 level.
Figure 11
A 26-yearold man with achondroplasia; intra- and postoperative images. (A) Intraoperative images; (B,C) postoperative radiograms; (D) sagittal reconstruction CT.
Figure 12
A 26-year-old man with achondroplasia; final follow-up images. (A,B) Follow-up radiograms show T11-L2 local 58° kyphosis, SVA 32 mm, PT 23°, and PI 82°; (C,D) follow-up reconstruction CT shows solid bony fusion.
References
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Fredwall S.O., Steen U., de Vries O., Rustad C.F., Eggesbø H.B., Weedon-Fekjær H., Lidal I., Savarirayan R., Manum G. High prevalence of symptomatic spinal stenosis in Norwegian adults with achondroplasia: A population-based study. Orphanet. J. Rare Dis. 2020;15:123. doi: 10.1186/s13023-020-01397-6.
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Grant support
This study received funding from Japan Organization of Occupational Health and Safety.