Pediatric Neurosurgery
Original Paper
Progressive Congenital Kyphosis: Report of Five Cases and Review of the LiteraturePhilips M.F. · Dormans J. · Drummond D. · Schut L. · Sutton L.N.Children’s Hospital of Philadelphia, Pa., USA
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Article / Publication Details
Received: May 23, 1997
Accepted: July 16, 1997
Published online: March 07, 2008
Issue release date: 1997
Number of Print Pages: 14
Number of Figures: 0
Number of Tables: 0
ISSN: 1016-2291 (Print)
eISSN: 1423-0305 (Online)
For additional information: https://www.karger.com/PNE
Abstract
For over 60 years congenital kyphotic deformities of the spine have been categorized into two distinct groups, depending on the developmental defect. Those arising from a failure of formation of the vertebral bodies were classified as type 1, while those arising from a failure of segmentation were referred to as type 2. Recognition of the progressive and unstable nature of the type 1 defects alerted physicians to the need for early operative stabilization through decompression and stabilization through instrumentation. As the embryogenesis of the spinal column was further investigated, and as diagnostic imaging methods of the spine improved, unstable congenital kyphoses were further subdivided. Progressive congenital kyphotic deformities now may accompany a host of vertebral column developmental defects as well as genetically mediated mesenchymal tissue defect syndromes. This paper presents 5 patients from The Children’s Hospital of Philadelphia with progressive and symptomatic congenital kyphotic deformities of the spine. Two of these lesions resulted from defects of formation of the vertebral bodies, while one resulted from segmental spinal dysgenesis, maldevelopment of both the anterior and posterior vertebral elements. One patient’s kyphotic deformity was a result of caudal regression syndrome, and the final case presented experienced a high thoracic kyphosis from a syndrome associated diffuse midline mesenchymal tissue abnormalities known as cerebrocostomandibular syndrome. All patients showed evidence of progressive cord compression and required neural element decompression, fusion, and instrumentation. The cases are discussed individually, and the developmental and clinical aspects of each are explored.
© 1997 S. Karger AG, Basel
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Article / Publication Details
Received: May 23, 1997
Accepted: July 16, 1997
Published online: March 07, 2008
Issue release date: 1997
Number of Print Pages: 14
Number of Figures: 0
Number of Tables: 0
ISSN: 1016-2291 (Print)
eISSN: 1423-0305 (Online)
For additional information: https://www.karger.com/PNE
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Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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