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Double Major Curve in an Adolescent

History

This is a case of an 11+1 year-old, premenarchal female who presented with worsening spinal deformity per the patient's Mother. She reported minimal back pain and no complaints of weakness. There was no family history of scoliosis.

Examination

  • Gait, motor and sensory exams were normal
  • No pathologic reflexes
  • No leg-length discrepancy
  • Obvious right thoracic and left lumbar prominence on bending test
  • No shoulder asymmetry was appreciated

Pre-Treatment Imaging

The standing scoliosis series (Figures 1-5) demonstrated a right main thoracic curve of 66-degrees that corrected to 57-degrees on bending films and left lumbar curve measuring 62-degrees correcting to 30-degrees. Thoracic kyphosis was 12-degrees.  The patient’s Risser score was zero.

standing scoliosis pre-operative AP demonstrates a right main thoracic curve of 66-degreesFigure 1 (Above)

standing anterior double curve scoliosis radiographFigure 2 (Above)

side bending standing radiograph, double curve scoliosisFigure 3 (Above)

side bending standing radiograph, double curve scoliosisFigure 4 (Above)

lateral standing radiograph, double curve scoliosisFigure 5 (Above)

Diagnosis

Adolescent Idiopathic Scoliosis; double major curve.

 

Suggest Treatment

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Selected Treatment

Surgical correction of both the thoracic and lumbar curves: T3-L4 instrumented fusion with apical Ponte osteotomies, en bloc and segmental derotation.

Surgeon's Rationale
Considering the magnitude of the patient’s double curvature, her potential for growth—and, therefore, worsening scoliosis—the decision was made proceed with surgery. The surgical goal was to correct and stabilize both curves. Standard all-posterior pedicle screw fixation and correction techniques were utilized with locally harvested autograft for arthrodesis.

Outcome

At three months, the patient was doing very well with return to normal activities. The thoracic curve was corrected to 13-degrees and the lumbar to 3-degrees (Figures 6, 7). The residual proximal thoracic curve will be followed radiographically.

post-operative x-ray demonstrates AIS thoracic curve correction to 13-degrees, lumbar to 3-degreesFigure 6 (Above)

post-operative lateral x-ray; AIS thoracic curve correction to 13-degrees, lumbar to 3-degreesFigure 7 (Above)

Peer Case Discussion

Baron S. Lonner, MD
Professor of Orthopaedic Surgery
Mount Sinai Hospital

Dr. Baaj presents a case of adolescent idiopathic scoliosis (AIS) in an 11 1/12-year-old premenarchal girl. She has a "double major" or Lenke 3 curvature based on the radiographs presented. A number of points can be made in discussing this case that are important considerations for the patient/family and for the surgeon.

First of all, in obtaining a history from the family, it is important to determine the age of onset of the scoliosis. If the diagnosis was made before age 10, this would be considered a juvenile-onset as opposed to adolescent-onset. The likelihood of neural element abnormalities such as Chiari malformation, syrinx, and/or tethered cord is increased with onset prior to age 10. Therefore, for patients with earlier onset, screening MRI of the spinal neural axis is indicated. Assuming this is indeed a case of adolescent-onset, advanced imaging is not required unless the patient has pain at a level that is persistent and limiting to activities, unusual in this age group, if she has neurological deficits or symptoms such as sciatica, or if she has an unusual curve pattern such as a left thoracic curve, proximal thoracic apex, or hyperkyphosis, for example.

In the decision-making process for surgery in AIS, clinical findings and radiographic parameters should be evaluated. Dr. Baaj did not mention an assessment of the clinical rotational prominences or angle of trunk rotation (ATR) that is typically measured with an inclinometer. This parameter can dictate the surgical maneuvers performed as well as the possibility of doing a selective thoracic fusion rather than treating both curves. If there is a large clinical rotation, vertebral derotation maneuvers using pedicle screw anchors bilaterally at the apex of the deformity facilitate improvement in the vertebral apical rotation and helps to diminish the clinical deformity. The anchor density at the apex in this patient is not high so that the amount of derotation may not be as complete had Dr. Baaj used more screws.

This decision may be affected by the size of the pedicles and concern for an apical breach of the pedicle wall, particularly on the concavity in which the spinal cord is at greater risk. Furthermore, if there is minimal deformity in the lumbar region and the truncal shift is in the direction of the thoracic curvature as opposed to the lumbar curvature, as in this case, one might consider not including the lumbar curvature in the fusion.

Other radiographic parameters can assist in this decision as well, including the amount of radiographic rotation and translation of the apical vertebrae in the thoracic spine versus the lumbar spine. If the ratio of these parameters is >1.2 (thoracic versus lumbar), a selective thoracic fusion is likely to be successful.1 Furthermore, the adequacy of the bending x-ray is uncertain. The lumbar curvature would certainly correct more if a fulcrum bending x-ray in which the patient leans over a bolster was performed or if greater effort on a supine bending x-ray was exhibited. This might have changed the assessment of structurality of the lumbar curvature.

Dr. Baaj did a nice job of restoring overall alignment and correcting the curvatures. Global coronal and sagittal alignment are established, the shoulders appear level on the x-ray, and thoracic kyphosis, which is often diminished pre-operatively and can flatten further with operative correction, has been restored. This is important for its impact on the lumbar and cervical reciprocal lordosis.2,3 We do not know what the impact of surgical correction on the ATR was for this patient as this has not been reported.

Finally, fusion to the L4 level, although likely dictated by the nature of the pathology, is not particularly desirable. Patients with fusion caudal to L3 have increased loads on the remaining motion segment intervertebral discs, and have a higher likelihood of adjacent segment disc degeneration compared to more cephalad lowest instrumented vertebral levels.4,5 An alternative approach, which is not the mainstream standard for patients, is anterior scoliosis correction or vertebral body tethering in which a fusionless correction is performed. This can be performed as a hybrid procedure in which the thoracic curve is fused, and a fusionless correction of the lumbar curvature can be performed as shown in the case I performed, below.

Pre- and post-operative radiographs, hybrid correction of adolescent idiopathic scoliosis

References:
1. Chang MS, Bridwell KH, Lenke LG, Cho W, et al. Predicting the outcome of selective thoracic fusion in false double major lumbar "C" cases with five- to twenty-four-year follow-up. Spine. 2010;35(24):2128-33. doi: 10.1097/BRS.0b013e3181e5e36e.

2. Newton PO, Yaszay B, Upasani VV, Pawelek JB, et al. Preservation of thoracic kyphosis is critical to maintain lumbar lordosis in the surgical treatment of adolescent idiopathic scoliosis. Spine. 2010;35(14):1365-70. doi: 10.1097/BRS.0b013e3181dccd63.

3. Lonner BS, Lazar-Antman M, Sponseller PD, et al. Multivariate analysis of factors associated with kyphosis maintenance in adolescent idiopathic scoliosis. Spine. 2012;37(15):1297-302.

4. Auerbach JD, Lonner BS, Errico TJ, Freeman A, et al. Quantification of intradiscal pressures below thoracolumbar spinal fusion constructs: Is there evidence to support "Saving a Level?". Spine. 2012;37(5):359-66.

5. Lonner BS, Ren Y, Marks MM, Newton PO, et al. Disc degeneration in unfused caudal motion segments ten years following surgery for adolescent idiopathic scoliosis. Whitecloud Clinical Award Nominee. Paper WC#3. 24th International Meeting on Advanced Spine Techniques (IMAST), July 12-15, 2017. Cape Town, South Africa.

Author's Response to Peer Discussion

I thank Dr. Lonner for his review of this case and excellent commentary.

A few points in response:

  • Selective thoracic fusion for Type C curves is certainly an option if the radiographic and clinical parameters allow.
  • Given the magnitude of the lumbar curve, including it in the construct likely leads to more predictable and durable long-term results.
  • A higher implant density at the apex allows for better derotation, but as Dr. Lonner points out—sagittal and coronal correction was achieved with excellent post-operative clinical alignment of the trunk and shoulders.

Finally, though promising, fusionless correction is not the mainstream standard for patients as Dr. Lonner accurately suggests.

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