Congenital infiltrating lipomatosis of the face: report of three cases and review of the literature.

OBJECTIVES: Three new cases of hemifacial hypertrophy caused by congenital infiltrating lipomatosis of the face are reported. The literature on infiltrating lipomatosis of the face is reviewed with an emphasis on accompanying anomalies and treatment strategies. REPORT OF CASES: In this study, the clinical features, radiographic findings, histopathology, and postoperative results were analyzed in three patients with facial infiltrating lipomatosis. The condition was diagnosed in infancy (one male subject, two female subjects) and characterized by enlargement of the cheek. Most patients had early eruption of ipsilateral deciduous and permanent teeth. Computed tomography and magnetic resonance imaging showed an infiltrated soft-tissue mass of fatty density and skeletal overgrowth. Multiple resections were performed on two patients. DISCUSSION: Described first by Slavin and colleagues in 1989, Facial infiltrating lipomatosis is a rare congenital disorder in which mature lipocytes invade adjacent tissue. The phenotypic features include soft-tissue and skeletal hypertrophy, premature dental eruption, and regional macrodontia. Due to its diffuse infiltration and involvement of important facial structures, complete surgical excision is often impossible. The aetiology, natural history, optimal management, and relationship to other disorders of fatty overgrowth are unclear. Because surgical removal of the mass is usually unsuccessful, specific management of this condition will require insight into its pathogenesis.