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Upper Cervical Fusion in Children with Morquio SyndromeIntermediate to Long-Term Results
Ozgur Dede, MD1; Mihir M. Thacker, MD1; Kenneth J. Rogers, PhD, ATC1; Murat Oto, MD1; Mohan V. Belthur, MD, FRCS (Tr&Orth)1; Wagner Baratela, MD1; William G. Mackenzie, MD, FRCS(C)1
1 Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19803. E-mail address for M.M. Thacker: mthacker@nemours.org
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Investigation performed at the Department of Orthopaedic Surgery, Nemours/Alfred I. duPont Hospital for Children, Wilmington, Delaware

Disclosure: None of the authors received payments or services, either directly or indirectly (i.e., via his or her institution), from a third party in support of any aspect of this work. None of the authors, or their institution(s), have had any financial relationship, in the thirty-six months prior to submission of this work, with any entity in the biomedical arena that could be perceived to influence or have the potential to influence what is written in this work. Also, no author has had any other relationships, or has engaged in any other activities, that could be perceived to influence or have the potential to influence what is written in this work. The complete Disclosures of Potential Conflicts of Interest submitted by authors are always provided with the online version of the article.

Copyright © 2013 by The Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2013 Jul 03;95(13):1228-1234. doi: 10.2106/JBJS.J.01135
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Paraplegia or death secondary to upper cervical spine instability and spinal cord compression are known consequences of Morquio syndrome. Decompression and fusion of the upper cervical spine are indicated to treat spinal cord compression. The purpose of this study was to report the intermediate to long-term results of upper cervical spine fusion in children with Morquio syndrome.


Twenty patients (nine female and eleven male) with Morquio syndrome who underwent upper cervical spine fusion at a mean age of sixty-three months were retrospectively analyzed with use of hospital records. Radiographic and clinical results were reported.


The average follow-up period was eight years and ten months. Fusion was achieved in all patients except one; this patient underwent a revision with transarticular C1-C2 screw fixation. Seven patients developed symptomatic instability below the fusion mass that required extension of fusion to lower levels at a mean of ninety-one months after the initial operation. Two patients required decompression and fusion of a site other than the upper cervical spine. Asymptomatic cervicothoracic and thoracolumbar kyphosis was prevalent among our patients. All patients were neurologically stable at the time of the latest follow-up visit.


Upper cervical spine fusion provides reliable fusion and a stable neural outcome in patients with Morquio syndrome. However, distal junctional instability is a major problem at long-term follow-up. Kyphotic deformity of the cervicothoracic and thoracolumbar junction may be present in a large number of patients with Morquio syndrome and evaluation for spinal stenosis at these levels should also be considered.

Level of Evidence: 

Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

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    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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