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Case Reports
. 2016 Sep;17(3):238-41.

Langer-Giedion Syndrome: a Rare Case Report

Farhin Ali Katge  1 , Bhavesh Dahyabhai Rusawat  1 , Pooja Ravindra Shivasharan  2 , Devendra Pandurang Patil  2
Affiliations
Case Reports

Langer-Giedion Syndrome: a Rare Case Report

Farhin Ali Katge et al. J Dent (Shiraz). 2016 Sep.

Abstract

Langer-Giedion syndrome is a very uncommon autosomal dominant genetic disorder caused by the deletion of chromosomal material. It is characterized by multiple bony exostosis, short stature, mental retardation, and typical facial features. The characteristic appearance of individuals includes sparse scalp hair, rounded nose, prominent philtral area and thin upper lip. Some cases with this condition have loose skin in childhood which typically resolves with age. Oral and dental manifestations include micrognathia, retrognathia, hypodontia, and malocclusion based on cephalometric analysis. This report presents a case of Langer-Giedion syndrome in a 10-year-old child.

Keywords: Exostosis; Hypodontia; Langer-Giedion Syndrome; Trichorhinophalangeal Syndrome type 2.

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Figures

Figure 1
Figure 1
General features: a: Short stature. b: Sparse scalp hair, outstanding pinnae, prominent philtral area, thin vermillion of upper lip. c: Bony exostosis. d: Overlapping toes.
Figure 2
Figure 2
Intraoral preoperative photographs
Figure 3
Figure 3
Chart showing tooth numbering
Figure 4
Figure 4
Extraoral radiographs: a: Orthopantomogram, b: Cephalometric tracing (Steiner’s analysis)
Figure 5
Figure 5
Post-operative photographs
See this image and copyright information in PMC

References

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