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J Oral Maxillofac Surg. 2018 Mar;76(3):646.e1-646.e12. doi: 10.1016/j.joms.2017.11.029. Epub 2017 Nov 26.

Crouzon Syndrome: A Case Series of Craniomaxillofacial Distraction Osteogenesis for Functional Rehabilitation.

Author information

1
Associate Professor and Consultant, Oro-Craniomaxillofacial Research and Surgical Group, Faculty of Dentistry, University of Malaya, Kuala Lumpur, Malaysia. Electronic address: firdaushariri@um.edu.my.
2
Professor and Senior Consultant, Oro-Craniomaxillofacial Research and Surgical Group, Faculty of Dentistry, University of Malaya, Kuala Lumpur, Malaysia.
3
Consultant Neurosurgeon, Division of Neurosurgery, Department of Surgery, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
4
Clinical Specialist, Oro-Craniomaxillofacial Research and Surgical Group, Faculty of Dentistry, University of Malaya, Kuala Lumpur, Malaysia.
5
Senior Lecturer, Mathematics Division, Centre for Foundation Studies in Science, University of Malaya, Kuala Lumpur, Malaysia.
6
Professor and Senior Consultant, Division of Neurosurgery, Department of Surgery, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.

Abstract

Crouzon syndrome (CS) is the most common craniosynostosis syndrome and requires a comprehensive management strategy for the optimization of care and functional rehabilitation. This report presents a case series of 6 pediatric patients diagnosed with CS who were treated with distraction osteogenesis (DO) to treat serious functional issues involving severe orbital proptosis, an obstructed nasopharyngeal airway, and increased intracranial pressure (ICP). Three boy and 3 girls were 8 months to 6 years old at the time of the operation. The mean skeletal advancement was 16.1 mm (range, 10 to 27 mm) with a mean follow-up of 31.7 months (range, 13 to 48 months). Reasonable and successful outcomes were achieved in most patients as evidenced by adequate eye protection, absence of signs and symptoms of increased ICP, and tracheostomy tube decannulation except in 1 patient. Complications were difficult fixation of external stabilizing pins in the distraction device (n = 1) and related to surgery (n = 4). Although DO can be considered very technical and can have potentially serious complications, the technique produces favorable functional and clinical outcomes in treating severe CS.

PMID:
29268076
DOI:
10.1016/j.joms.2017.11.029

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