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Research ArticleOther & Special Categories

Adult Congenital Spine Deformity: Clinical Features and Motivations for Surgical Treatment

Javier Pizones, Lucía Moreno-Manzanaro, Alba Vila-Casademunt, Nicomedes Fernández-Baíllo, José Sánchez-Márquez, Gloria Talavera, Ibrahim Obeid, Ahmet Alanay, Frank Kleinstück, Ferran Pellisé and Francisco Javier Sánchez Perez-Grueso; On behalf of the ESSG (European Spine Study Group)
International Journal of Spine Surgery December 2021, 15 (6) 1238-1245; DOI: https://doi.org/10.14444/8157
Javier Pizones
1 Spine Unit, Department of Orthopedic Surgery, Hospital Universitario La Paz, Madrid, Spain
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Lucía Moreno-Manzanaro
1 Spine Unit, Department of Orthopedic Surgery, Hospital Universitario La Paz, Madrid, Spain
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Alba Vila-Casademunt
2 Spine Research Unit, Vall d’Hebron Institute of Research, Barcelona, Spain
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Nicomedes Fernández-Baíllo
1 Spine Unit, Department of Orthopedic Surgery, Hospital Universitario La Paz, Madrid, Spain
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José Sánchez-Márquez
1 Spine Unit, Department of Orthopedic Surgery, Hospital Universitario La Paz, Madrid, Spain
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Gloria Talavera
1 Spine Unit, Department of Orthopedic Surgery, Hospital Universitario La Paz, Madrid, Spain
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Ibrahim Obeid
3 Spine Surgery Unit, Pellegrin University Hospital, Bordeaux, France
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Ahmet Alanay
4 Department of Orthopedics and Traumatology, Acibadem Mehmet Ali Aydinlar University, Istanbul, Turkey
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Frank Kleinstück
5 Department of Orthopedics, Schulthess Klinik, Zurich, Switzerland
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Ferran Pellisé
6 Spine Surgery Unit, Hospital Universitario Vall d’Hebron, Barcelona, Spain
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Francisco Javier Sánchez Perez-Grueso
1 Spine Unit, Department of Orthopedic Surgery, Hospital Universitario La Paz, Madrid, Spain
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    Figure 1

    A 24-year-old woman with chronic pulmonary disease. She underwent previous surgery during childhood with a T4-T7 early short fusion due to hemivertebrae. Along the years, she developed a crankshaft phenomenon showing a residual Cobb angle of 100°. Patient-reported outcome measure scores were as follows: Numerical Rating Scale back, 5; Oswestry Disability Index, 27%; Scoliosis Research Society 22-item self-image, 2; and 36-item Short Form Health Survey physical component summary, 45. Surgical indication was established for a T6-T8 vertebral column resection with T2-L3 post instrumentation and added thoracoplasty.

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    Figure 2

    A 29-year-old woman with cardiopathy (previous cardiac surgery). She had a T12 hemivertebra producing a T10-L1 coronal curve with 67° Cobb angle, and a thoracolumbar rotational kyphosis with a sagittal T10-L2 of 47°, well aligned. Patient-reported outcome measures scores were as follows: Numerical Rating Scale back, 6; Core Outcome Measurement Index back, 6; Oswestry Disability Index, 29%; Scoliosis Research Society 22-item survey (SRS-22) pain, 2.8; SRS-22 self-image, 2.2; and 36-item Short Form Health Survey physical component summary, 41.3. Surgery was indicated and a T9-L3 posterior fusion with T12 asymmetric osteotomy was planned.

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    Figure 3

    A 26-year-old man with chronic pulmonary disease. He suffered a T7 hemivertebra and ipsilateral T2-T5 rib fusion. Coronal Cobb angle from T1-T5 was 35° and from T5-T9 was 61°. Patient-reported outcome measure scores were as follows: Numerical Rating Scale back, 0; Core Outcome Measurement Index back, 2; Oswestry Disability Index, 2%; Scoliosis Research Society 22-item survey (SRS-22) pain, 5; SRS-22 self-image, 3.6; and 36-item Short Form Health Survey physical component summary, 58.8. He followed conservative treatment.

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    Figure 4

    A 34-year-old woman with no comorbidities. Radiographs showed multiple anomalies: T10 right hemivertebra, L1 left hemivertebra, T4-T10 ribs fusions, and L4-L5 left bar. Coronal Cobb angles were T1-T9 73° and L3-L5 54°. Patient-reported outcome measure scores were as follows: Numerical Rating Scale back, 4; Core Outcome Measurement Index back, 3; Oswestry Disability Index, 18%; Scoliosis Research Society 22-item survey (SRS-22) pain, 3.2; SRS-22 self-image, 3.2; and 36-item Short Form Health Survey physical component summary, 42.7. Conservative treatment was indicated.

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    Figure 5

    A 39-year-old woman without comorbidities. Radiographs showed multiple segmentation defects at T10-T11, L2-L3, and L4-L5 drawing a lumbar flat back with correct global sagittal alignment. Patient reported outcome measure scores were as follows: Numerical Rating Scale back, 9; Core Outcome Measurement Index back, 7; Oswestry Disability Index, 58%; Scoliosis Research Society 22-item survey (SRS-22) pain, 1.2; SRS-22 self-image, 3; and 36-item Short Form Health Survey physical component summary, 21. Cobb angle T12-L4 42°. Surgical treatment was proposed for an L4 PSO and T11-iliac instrumentation.

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    Figure 6

    A 40-year-old woman with no comorbidities. She presented a T7-T10 right incarcerated hemivertebrae and contralateral bar, and an L4 hemivertebrae. Coronal T6-T12 Cobb angle was 90° and L1-L5 Cobb angle was 66°. Patient-reported outcome measure scores were as follows: Numerical Rating Scale back, 6; Oswestry Disability Index, 33%; Scoliosis Research Society 22-item survey (SRS-22) pain, 2; SRS-22 self-image, 3; and 36-item Short Form Health Survey physical component summary, 40. Pelvic parameters of pelvic incidence, 45°; sacral slope, 20°; and pelvic tilt, 25° were compensating but well balanced. She was planned for a T8 vertebral column resection, surrounding Smith-Peterson osteotomies and T2-Iliac posterior instrumentation.

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    Figure 7

    A 60-year-old woman with hypertension. She had a T8 hemivertebra with coronal T6-T10 Cobb angle of 87° and T11-L3 of 90°. Patient-reported outcome measure scores were as follows: Numerical Rating Scale back, 3; Oswestry Disability Index, 18%; Scoliosis Research Society 22-item survey (SRS-22) pain, 3.7; SRS-22 self-image, 1.2; and 36-item Short Form Health Survey physical component summary, 46.4. The patient followed conservative treatment.

Tables

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    Table 1

    Demographic and radiographic comparisons between patients treated conservatively and those undergoing surgery.

    Preoperative DataSurgicalConservativeStudent t/χ2
    Demographic parameters
     Age, y36.5 ± 10.638.6 ± 14.20.55
     Gender, F/M86.4%/13.6%60%/40%0.038a
     Height, cm151.7 ± 10.1157.9 ± 11.60.07
     Weight, kg59.2 ± 14.565.9 ± 160.15
     BMI25.9 ± 4.826.4 ± 50.76
     Comorbidities40.9%33.3%0.121
     Previous surgery45.5%23.3%0.093
    Deformity type 10.746
     Scoliosis77.3%73.3%
     Kyphosis22.7%26.7%
    Deformity type 20.811
     Simple HV64%56%
     Multiple HV30%39%
     Segment defects6%5%
    Deformity Location0.166
     Proximal thoracic4.5%16.7%
     Main thoracic36.4%46.7%
     Thoracolumbar31.8%10%
     Lumbar27.3%26.7%
    Radiographic parameters
     Main Cobb, °59.3 ± 33.464.8 ± 27.10.52
     Coronal balance, mm−9.3 ± 36.9−0.8 ± 26.30.42
     SVA, mm14.2 ± 42.819.6 ± 56.10.73
     Global tilt, °22.9 ± 15.318.6 ± 14.60.36
     T10-L2 kyphosis, °13.7 ± 31.225.4 ± 350.24
     PI-LL mismatch, °−0.7 ± 26−2.9 ± 24.30.765
     LL, °49.2 ± 27.457.3 ± 23.60.265
     PI, °47.8 ± 13.951.2 ± 16.50.458
     T-DAR8.9 ± 4.89.9 ± 4.60.464
    • Data presented as mean ± SD and percentages.

    • aStatistically significant.

    • BMI, body mass index; HV, hemivertebrae; LL, lumbar lordosis; PI, pelvic incidence; SVA, sagittal vertical axis; T-DAR, total deformity angular ratio.

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    Table 2

    PROMs comparisons between patients treated conservatively and those undergoing surgery.

    SurgicalConservative
    Preoperative DataMean ± SDMedian (IQR)Mean ± SDMedian (IQR)Student
    t test
    Mann-Whitney U test
    PROMs
    NRS back pain5.2 ± 3.25.6 ± 30.61 
    NRS leg pain3.6 ± 3.24 (0–6)2.6 ± 3.30.5 (0–4.25) 0.233
    COMI back6.7 ± 2.43.8 ± 2.40.004a 
    COMI neck3.3 ± 2.12.6 ± 2.40.67 
    ODI (%)34.6 ± 19.226.9 ± 15.90.15 
    SRS-22
    SRS-22 function3.1 ± 0.83 (2.5–4)3.6 ± 14 (3–4) 0.089
    SRS-22 pain2.9 ± 1.23.1 ± 10.56 
    SRS-22 self-image2.5 ± 0.92 (2–3)3 ± 0.93 (2–4) 0.047a
    SRS-22 mental health2.9 ± 13 (2–4)3.5 ± 0.83.5 (3–4) 0.083
    SRS-22 satisfaction3.2 ± 1.3 3.5(2.25–4)3.6 ± 14 (3–4) 0.533
    SRS-22 subtotal2.82 ± 0.73 (2–3)3.4 ± 0.84 (3–4) 0.020a
    SRS-22 total2.9 ± 0.73 (2–3)3.4 ± 0.84 (3–4) 0.014a
    SF-36
    SF-36 function38.6 ± 8.945.1±9.20.023a 
    SF-36 physical role33.7 ± 12.442.6 ± 12.80.026a 
    SF-36 pain38.3 ± 11.941 ± 10.40.43 
    SF-36 vitality46.5 ± 847.9 ± 9.40.59 
    SF-36 social function34.9 ± 8.537 (27–37)43.7 ± 12.447 (32–57) 0.022a
    SF-36 emotional role40.9 ± 1342.5 ± 11.30.67 
    SF-36 mental health43.8 ± 10.244.8 ± 90.72 
    SF-36 general health41.9 ± 10.443.2 ± 11.30.68 
    SF-36 PCS36.7 ± 10.443.3 ± 10.80.048a 
    SF-36 MCS44.2 ± 11.445.1 ± 10.90.79 
    • Parametric data presented as mean ± SD. Nonparametric data presented as median (IQR).

    • aStatistically significant.

    • COMI, Core Outcome Measurement Index; IQR, interquartile range; MCS, mental component summary; NRS, Numerical Rating Scale; ODI, Oswestry Disability Index; PCS, physical component summary; PROM, patient-reported outcome measures; SF-36, 36-item Short Form Health Survey; SRS-22, Scoliosis Research Society 22-item questionaire.

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International Journal of Spine Surgery
Vol. 15, Issue 6
1 Dec 2021
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Adult Congenital Spine Deformity: Clinical Features and Motivations for Surgical Treatment
Javier Pizones, Lucía Moreno-Manzanaro, Alba Vila-Casademunt, Nicomedes Fernández-Baíllo, José Sánchez-Márquez, Gloria Talavera, Ibrahim Obeid, Ahmet Alanay, Frank Kleinstück, Ferran Pellisé, Francisco Javier Sánchez Perez-Grueso
International Journal of Spine Surgery Dec 2021, 15 (6) 1238-1245; DOI: 10.14444/8157

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Adult Congenital Spine Deformity: Clinical Features and Motivations for Surgical Treatment
Javier Pizones, Lucía Moreno-Manzanaro, Alba Vila-Casademunt, Nicomedes Fernández-Baíllo, José Sánchez-Márquez, Gloria Talavera, Ibrahim Obeid, Ahmet Alanay, Frank Kleinstück, Ferran Pellisé, Francisco Javier Sánchez Perez-Grueso
International Journal of Spine Surgery Dec 2021, 15 (6) 1238-1245; DOI: 10.14444/8157
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