A rare case of diffuse lipomatosis located at the head of a child

The AA. present a rare case of <> on the head of a child, who early after birth had quickly developed gross asymmetry of the face; it consisted in the hypertrophy of the right side, involving malar, maxillary and palatal bones, upper teeth and soft tissue of the cheeck and upper lip. An X-ray and CAT-scan follow up revealed bone hypertrophy without abnormalities of bone structures. Microscopically, adipose tissue was ectopically present both in dermis and in muscles, but contrarily to intramuscular lipoma, single muscle fibers were spared and failed to show typical entrapping. No lipoblastic activity was detectable and there was no evidence of cellular pleomorphism. A diagnosis of <>, and exceedingly rare, poorly demarcated proliferation of mature adipose tisse, was made. Treatment to data has been restricted to the reduction of the soft tissues with reasonable results. A maxillofacial procedure is mandatory to restore facial symmetry but, owing to the unique anatomical site of this condition, we feel uncertain about the timing, since a major requirement is to avoid recurrences.