Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report.

Authors:
Shakil Ahmed Nagori
Shakil Ahmed Nagori
All India Institute of Medical Sciences
India
Dr Anson Jose, MDS
Dr Anson Jose, MDS
All India Institute of Medical Sciences
Oral and maxillofacial surgeon
Oral and maxillofacial surgery
New delhi, new delhi | India
Bhaskar Agarwal
Bhaskar Agarwal
All India Institute of Medical Sciences
New Delhi | India
Krushna Bhatt
Krushna Bhatt
All India Institute of Medical Sciences
India
Ongkila Bhutia
Ongkila Bhutia
All India Institute of Medical Sciences
India
Ajoy Roychoudhury
Ajoy Roychoudhury
All India Institute of Medical Sciences
India

J Med Case Rep 2014 Nov 25;8:387. Epub 2014 Nov 25.

Department of Oral and Maxillofacial Surgery, CDER, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.

Introduction: Langer-Giedion syndrome (trichorhinophalangeal syndrome type II) is an extremely rare disorder characterized by dysmorphic facial features, multiple exostoses, mental retardation and digit deformities. We report the first case of any maxillofacial pathology in such a syndromic patient.

Case Presentation: A 22-year-old Indian woman with mild intellectual disability presented with malaligned teeth. Routine radiographic screening demonstrated a large multilocular lesion in her right mandible. She had peculiar features such as short stature, short limbs, brachydactyly, and dysmorphic facial characters, which prompted us to evaluate her further. After findings of multiple bony exostoses she was diagnosed with Langer-Giedion syndrome. On surgical exploration of her right mandibular lesion an empty cavity was found suggestive of traumatic bone cyst. The lesion healed completely after 1 year without loss of vitality of any teeth.

Conclusions: Although diagnosis and management of any maxillofacial pathology can be challenging in syndromic patients, our report suggests a possible correlation between traumatic bone cyst and Langer-Giedion syndrome. Clinicians should routinely screen these patients for any undetected maxillofacial pathology. In future cases of this syndrome, one should consider the possibility of traumatic bone cyst which may not require aggressive surgical management.

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Source
http://dx.doi.org/10.1186/1752-1947-8-387DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4364690PMC
November 2014
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