Langer-Giedion Syndrome: a Rare Case Report

Katge, Farhin Ali; Rusawat, Bhavesh Dahyabhai; Shivasharan, Pooja Ravindra; Patil, Devendra Pandurang

Langer-Giedion Syndrome: a Rare Case Report

Document Type : Case Report

Authors

Farhin Ali Katge ¹

Bhavesh Dahyabhai Rusawat ¹

Pooja Ravindra Shivasharan ²

Devendra Pandurang Patil ²

¹ Dept. of Pedodontics & Preventive Dentistry, Terna Dental College, Navi Mumbai, Maharashtra, India.

² Postgraduate Student, Dept. of Pedodontics & Preventive Dentistry, Terna Dental College, Navi Mumbai, India.

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 ● Trichorhinophalangeal Syndrome type 2 ● Exostosis ● Hypodontia ● Langer-Giedion Syndrome