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Título

Posterior spondylectomy for thoracolumbar kyphosis in an adult with achondroplasia. A case report

Introdução

Achondroplasia is the most common skeletal dysplasia, with a prevalence reported from 1 in 30.000 live births, Thoracolumbar kyphosis has been reported with high prevalence in these patients , however the majority of the cases are on pediatric and immature spine .
Treatment of Thoracolumbar kyphosis (TLK) in adult achondroplasic patients has only been reported in 4 cases , here we report a rare case of an adult patient with achondroplasia that was treated with posterior spondilectomy in our spine department

Relato de caso

A 42 years old patient with achondroplasia with history of surgical Intervention for cervical spondylotic myelopathy was admited in our spine department with a complaint of low back pain associated with neurogenic claudication and radiculopathy , with a spinal deformity that wass diagnosed in his thoracolumbar region.
Panoramic plain radiographs revealed a high-grade thoracolumbar kyphotic deformity, with deformity apex located at L1, the kyphotic angle from T10 to L2 was 101,7°, and the anterior region was fused from T12 to L2 vertebrae (Figure 1 ). On spine MRI a narrow lumbar spine canal was diagnosed on the apex of the TLK (Figure 2 ). A single-stage posterior vertebral osteotomy with instrumented fusión was done by two spine surgeons, At the level of the apical vertebra, heads and the proximal portions of the attached ribs were exposed at the thoracic levels and pedicle screws were inserted on T10-T11 and L3-L4 spinal levels, with a freehand technique, complete remove of T12,L1 and L2 vertebral bodies was done with special caution to preserve the exiting nerve roots, an expandable lumbar spine cylinder was inserted and permanent rods were placed into the screw heads on both sides in a cantilever fashion, with intraoperative spine neuromonitorization , the TLK was corrected with compression forces applied to the pedicle screw, without neurological impairment during the complete kyphosis reduction. A control plain radiograph was done on the second postoperative day, with a reduction to 31,7° of his TLK . After 3 months the patient continued with complete neurological improve and without any discomfort during his walks .

Conclusão

In adult achondrolasic patients with TLK , a reconstruction of the spine for stabilization and fusion should be considered with neurological circumferential decompression of the spinal cord at the apical level, combined anterior and posterior approaches have been usually advocated, with only 4 cases being treated to date with a complete posterior vetrebrectomy for a total spondilectomy and spine reconstruction, been this the fifth case of a rigid kyphotic case of a skeletally mature achondroplastic patient whose rigid TLK was corrected with a single posterior approach and without any neurological impairment with complete resolution of his lumbar stenosis symptoms demonstrating that this surgical technique is safe and can guarantee a high grade of neurological improve.

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