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Congenital kypho-scoliosis: a case of thoracic insufficiency syndrome and the limitations of treatment

European Spine Journal volume 21, pages 1043–1049 (2012)Cite this article

Abstract

Introduction

Congenital spinal vertebral anomalies may present with deformity resulting in congenital scoliosis and kyphosis. This leads to abnormal spinal growth. The latter when combined with associated rib fusions may impair normal thoracic cage development and resultant pulmonary hypoplasia. Most congenital scoliosis can be detected in utero by ultrasound scan or recognized in the neonatal period, but a few spinal defects can remain undetected.

Materials and Methods

In this Grand Round, we present the case of a 7-year-old girl with a severe scoliosis and thoracic insufficiency syndrome (TIS). 3D CT reconstruction imaging demonstrated a mixed picture of fusion and segmentation abnormalities. A marked kyphoscoliosis was demonstrated at the thoraco-lumbar junction. Via a left thoracotomy, anterior excision of intervertebral discs was performed together with, interbody fusion, and in situ stabilisation of the kyphosis with double allograft (femur) strut grafts.

Conclusions

This article highlights the features of congenital kypho-scoliosis and TIS. The difficulties of treating kyphosis when combined with TIS are discussed together with the limitations of current surgical techniques.

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Conflict of interest

None of the authors has any potential conflict of interest.

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Affiliations

  1. Centre for Spinal Studies and Surgery, D Floor, West Block, Queens Medical Centre, Derby Road, Nottingham, NG7 2UH, UK

    A. D. Chatterjee, K. Hassan & M. P. Grevitt

Authors
  1. A. D. Chatterjee
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  2. K. Hassan
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  3. M. P. Grevitt
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Corresponding author

Correspondence to A. D. Chatterjee.

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Chatterjee, A.D., Hassan, K. & Grevitt, M.P. Congenital kypho-scoliosis: a case of thoracic insufficiency syndrome and the limitations of treatment. Eur Spine J 21, 1043–1049 (2012). https://doi.org/10.1007/s00586-011-2032-y

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  • DOI: https://doi.org/10.1007/s00586-011-2032-y

Keyword

  • Thoracic insufficiency syndrome