Severe curvature treated by anterior release and posterior fusion with thoracoplasty

History:The patient was a 9 year old girl that presented for evaluation with her mother due to a scoliosis. According to the mother the scoliosis was diagnosed several years earlier, brace treatment had been discussed but a recommendation to delay any surgery until near end of growth was made. Due to the markedly progressive deformity the mother sought an evaluation. Of note, the patient had a congenital thumb anomaly but no other known organ abnormalities.

Physical Examination:The patient was a cheerful and alert child she walked with a normal gait but had a marked deformity of her back with some imbalance of her trunk. In addition to a notable rib hump the patient had a significant kyphosis of the upper back. Lower extremity examination revealed full motion in all joints, good strength in all muscle groups, normal sensation and reflexes.

Radiographic Evaluation:On standing full-length radiographs of the spine, a severe left sided thoracic scoliosis measuring 115 degrees was evident. (see figure 1) The trunk was imbalanced with a shift to the left of 9cm. Kyphosis in the thoracic spine was over 65 degrees. Congenital malformation was suspected due to the rigid severe deformity (by bending x-rays, figure 2) and the associated thumb anomaly. Ct scan and MRI of the spine were obtained revealing evidence of a congenital bar (vertebrae which have fused together) but no anomaly of the spinal cord or brain. Pulmonary function tests revealed markedly reduced lung function.

Treatment Options:In general terms, scoliosis which is severe, markedly progressive and early in onset must be thoroughly evaluated for possible underlying congenital or neuromuscular problems. Early diagnosis and treatment is crucial in these cases. Unfortunately, in this patient treatment had been delayed and severe deformity had developed at a young age. The options at the point of office consultation were limited. A severe deformity had lead to imbalance, reduced lung function and risk of further lung and eventual cardiac dysfunction. Bracing is not very effective in the setting of congenital scoliosis particularly not in severe deformity. Surgical treatment can be considered to address first of all risk of further progression, secondly to offer some correction and restore balance. The options and risks/benefits of surgery were reviewed in detail with the family and a decision to proceed with surgery was made. Surgical options include: open anterior spinal release with posterior fusion or posterior fusion alone. A thoracoplasty can be considered to offer cosmetic correction of the rib hump deformity and increase ability to correct the spinal deformity. The risks of a posterior alone fusion are possible continued anterior spinal growth with further deformity (crankshaft phenomenon) and very limited correction potential of the scoliosis and kyphosis. A combined anterior and posterior surgery offers the advantages of increased correction of the kyphoscoliosis and markedly reduced risk of crankshaft.

Treatment and Results: The patient underwent an anterior release and fusion followed by a posterior osteotomy of a congenital bar combined with convex and concave thoracoplasties. This permitted a controlled and gradual correction of the deformity with re-establishment of proper spinal balance (figures 3 and 4). The patient achieved some immediate increased height due to the correction and most importantly obtained balance of her trunk. She had an anticipated prolonged pulmonary recovery due to her poor initial lung function but has returned to all activity and has excellent correction and balance of her spine. The major scoliotic curvature was corrected from 115 degrees to 45 degrees.