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Original Research

Open Access

Oro-dental manifestations of the Schwartz-Jampel syndrome

  • L.X.G. Stephen1
  • P.H. Beighton2

1Dental Genetic Unit, Department of Oral Medicine and Periodontology, Faculty of Dentistry, University of the Western Cape, Cape Town, South Africa

2Department of Human Genetics, Faculty of Health Sciences, University of Cape Town, Cape Town, South Africa

DOI: 10.17796/jcpd.27.1.923863w781k61p83 Vol.27,Issue 1,September 2002 pp.67-70

Published: 01 September 2002

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Abstract

A boy with the Schwartz-Jampel syndrome (chondrodystrophic myotonia) had a number of oro-dental complications.These included difficulty in tooth extraction and orthodontic care due to a small oral aper-ture and rigidity of the temporo-mandibular joints. General anaesthesia was hazardous because of a propensity to malignant hyperthermia, and endotracheal intubation was difficult because of shortness and rigidity of the neck and the small size of the laryngeal structures. Awareness of these potential prob-lems is crucial for anaesthesia and comprehensive dental management. The radiological demonstration of dentigerous cysts is a hitherto unreported observation in this disorder.


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L.X.G. Stephen,P.H. Beighton. Oro-dental manifestations of the Schwartz-Jampel syndrome. Journal of Clinical Pediatric Dentistry. 2002. 27(1);67-70.

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https://www.jjocpd.com/articles/10.17796/jcpd.27.1.923863w781k61p83

References

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