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Radiographic analysis of the progression of congenital scoliosis with rib anomalies during the growth period

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ArgoSpine News & Journal

Abstract

Study design

Retrospective study.

Objectives

To evaluate progression of various types of congenital scoliosis (CS) with rib anomalies (RA) during the various stages of the growth period, and to assess severity of progression in order to make a strategic planning of expansion thoracoplasty.

Summary of background data

VEPTR is approved for the treatment of patients with TIS in more than 30 countries. However, there is no consensus on the surgical indications, or the age or time when VEPTR surgery should be performed. Furthermore, there is no study related to natural history of congenital scoliosis with rib anomalies except two reports that are not sufficient to indicate risk factors of progression during the growth period.

Methods

Based on a survey of CS and RA via questionnaires, 70 patients (32 males and 38 females with an average age of 2.6 years at the first visit.) matched the inclusion criteria: CS with RA, no procedures that could influence natural history during follow-up periods, repeated plain X-ray check-ups with at least two years interval during growth periods. Average follow-up (F/U) time was 5.4 years (2–14). Plain X-ray images of 70 patients were divided into three age groups: infantile (0–5,6), juvenile (5–10,11), and adolescent (11,12-). Each X-ray image was evaluated in terms of laterality, range and type of RA, severity of scoliosis, type of CS, thoracic height ratio, SAL, and associated anomalies.

Results

54 of the 70 patients had unilateral rib anomalies. Rib anomalies included rib fusion in 52, mixed type (fusion and defect) in 8, rib proximity in 6, and rib defect in 4. Vertebral anomalies included formation failure in 1, segmentation failure in 16 and mixed type in 53. The magnitude of scoliosis was 46.9° at the first visit and 65.7° at the final F/U. Scoliosis progressed at the rate of 4.6°/year in 70, 3.6°/year in bilateral RA involvement and 4.9°/year in unilateral. Scoliosis progressed with the rate of 4.6°/year in 70, 3.6°/year in bilateral RA involvement and 4.9°/year in unilateral. Scoliosis progressed most severely during infantile period with the rate of 5.0°/year, followed by adolescent of 3.8°/year and juvenile of 2.3°/year. Patients with rib defect or unilateral unsegmented bar showed higher progression rates (10.7°/year and 7.0°/year) during infantile period. According to the relationship between SAL and scoliosis, four grades in severity of progression (most severe, severe, moderate, mild) were set up with the cut-off value of 70%, 85% of SAL and 45°, 85° of scoliosis for making the strategic planning of ET. Those grades were significantly related with types and location of RA and types of vertebral anomalies.

Conclusions

Progression of scoliosis was analysed in 70 patients with CS & RA. Congenital scoliosis with rib anomalies progressed most rapidly during the early infantile period (7.8°/year), followed by the late infantile period (5.0°/year), and the adolescent period (3.8°/year). Progression of scoliosis in patients with CS and RA occurred in early phase of growth periods and significantly related with types and location of RA as well as type of vertebral anomalies. The results of this study surely suggest the timing of ET for the patients with CS and RA.

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Authors and Affiliations

  1. Department of Orthopedic Surgery & Spine Surgery, Meijo Hospital, Nagoya, Japan

    Noriaki Kawakami & T. Tsuji

  2. Department of Orthopedic Surgery, Hukuoka Children Hospital, Fukuoka, Japan

    H. Yanagida

  3. Department of Orthopedic Surgery, Kobe Medical Center, Kobe, Japan

    K. Uno

  4. Department of Orthopedic Surgery, Keio University, Minato, Japan

    M. Matsumoto & K. Watanabe

  5. Department of Orthopedic Surgery, Kagoshima University, Kagoshima, Japan

    T. Yamamoto

  6. Department of Orthopedic Surgery, Niigata University, Niigata, Japan

    T. Hirano

  7. Department of Orthopedic Surgery, Dokkyo Medical College, Tochigi, Japan

    H. Taneichi

  8. Department of Orthopedic Surgery, Iwate Medical College, Iwate, Japan

    K. Yamazaki

  9. Department of Orthopedic Surgery, Osaka Medical College, Osaka, Japan

    K. Fujiwara

  10. Department of Orthopedic Surgery & Spine Surgery, Meijo Hospital, 1-3-1 Sannomaru Naka-ku, Nagoya, 460-0001, Japan

    Noriaki Kawakami

Authors
  1. Noriaki Kawakami
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  2. T. Tsuji
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  3. H. Yanagida
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  4. K. Uno
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  5. M. Matsumoto
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  6. K. Watanabe
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  7. T. Yamamoto
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  8. T. Hirano
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  9. H. Taneichi
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  10. K. Yamazaki
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  11. K. Fujiwara
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Corresponding author

Correspondence to Noriaki Kawakami.

Additional information

Dr. Noriaki Kawakami completed his medical education in 1981 at Nagoya University (Japan) and was board certified in the same year. He successfully earned his doctoral degree from the same university in 1988. He did his residency training in General Surgery at Tokyo Kouseinennkin Hospital from 1981 to 1983 and became a resident in Orthopaedic Surgery at Nagoya University the following year. From 1984 to 1988 Dr. Kawakami served as an orthopaedic surgeon at Tokyo Kouseinennkin Hospital. He was a visiting fellow at the Minnesota Spine Center, Minneapolis (USA) in 1991. His current appointments include those of Director of the Department of Orthopaedics and Spine Surgery at Meijo Hospital as well as Clinical Professor at Nagoya University School of Medicine. In 2005 N. Kawakami was a visiting Professor at Emory University School of Medicine, Atlanta (USA). He was Past President for the Japanese Scoliosis Society and the Japanese Spinal Instrumentation Society. He is a corresponding author and an active member of several national and international societies such as the Scoliosis Research Society.

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Kawakami, N., Tsuji, T., Yanagida, H. et al. Radiographic analysis of the progression of congenital scoliosis with rib anomalies during the growth period. ArgoSpine News J 24, 56–61 (2012). https://doi.org/10.1007/s12240-012-0042-1

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  • DOI: https://doi.org/10.1007/s12240-012-0042-1

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