The online version of this article (doi:10.1186/1752-1947-8-387) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
SAN, AJ and BA wrote the manuscript, reviewed the literature, treated the patient after the surgical procedure; KB, OB and AR performed the surgical procedure and contributed to the writing of the manuscript and revised it critically. All authors read and approved the final manuscript.
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.
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.
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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- Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report
Shakil Ahmed Nagori
- BioMed Central