Mandibular Arteriovenous Malformation in a 20-Year-Old Male with McCune Albright Syndrome: A Case Report

A twenty-year-old male with a diagnosis of McCune Albright Syndrome (MAS) presented for biopsy of bilateral mandibular body lesions, presumed to be fibro-osseous in nature, and extraction of impacted third molars.  Intra-operatively, these lesions were found to be arteriovenous malformations (AVM).  Although fibro-osseous lesions associated with MAS can be highly vascularized, there are no reports that conclusively link AVMs to MAS.  Rapid growth associated with fibro-osseous lesions has been linked to aneurysmal bone cysts, which may arise from vascular defects such as AVMs that have hemorrhaged.  This case, however, is the first report of an intra-osseous AVM in a patient with MAS presenting for oral and maxillofacial surgery.

The patient clinically displayed findings consistent with a diagnosis of MAS including multiple fibro-osseous lesions of the long bones and café-au-lait spots.  He had suffered previously from multiple long bone, hip and rib fractures.  Maxillofacial CT scans were obtained.  There was extensive osseous expansion of the frontal bone, sphenoid wings, ethmoid and temporal bones bilaterally with a ground glass appearance.  The mandible showed bilateral lesions with more lytic characteristics with no areas of abnormal enhancement to suggest presence of an AVM.

Biopsy of mandibular lesions and extraction of impacted third molars was performed in the OR setting.  There was copious blood loss during the procedure, estimated at approximately two liters.  Hemorrhage was controlled with electrocautery, gel foam, bone wax and primary closure.  The patient required transfusion of two units of packed red blood cells and admission to the surgical intensive care unit for post-operative observation.  Subsequent arteriogram and consultation with vascular interventional radiology confirmed the diagnosis of a large mandibular AVM.  Embolization was attempted, but due to the tortuous feeding vessels only partial embolization was achievable.  Acrylic glue (N-Butyl-Cyano-Acrylate [NCBA] Histoacryl) was used for embolization.

The association between MAS and intra-osseous mandibular AVM is extremely rare and it is unlikely that both conditions share the same biological pathways.  However, intra-osseous maxillo-mandibular AVM should be considered when managing patients harboring enlarging and bone remodeling lesions.

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