Proteus Syndrome – A one in a million occurrence
Keywords:Proteus syndrome; craniofacial deformity; AKT1 gene mutation
The syndrome is named after the Greek sea-god Proteus, who could change his shape. Proteus Syndrome (PS) is a rare condition with an incidence of less than 1 in 1 million people. It is characterized by variable, multifocal overgrowth of bones, skin, or other tissue derived from any of the three germinal layers. It is generally not apparent at birth, but signs develop rapidly from as early as 6 months and get more severe with age. Craniofacial deformities are less frequent but overgrowth of facial bones leads to disfigurement, malocclusion and a number of other oral and dental anomalies. The following case report of a young boy with PS was written for three
reasons. Firstly, it will describe this unusual condition to colleagues who have never encountered patients with the syndrome. It then stresses the importance of a holistic approach to treatment planning. This entails addressing the immediate needs, and then basing the definitive treatment on considerations of possible short- and longterm, patient-related developments. Finally, it illustrates
how a complex case was treated successfully with conservative management using modifications of standard clinical procedures.
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