PROTEUS SYNDROME - A CASE-REPORT OF A HAMARTOMATOUS SYNDROME WITH SEVERE MANDIBULAR HEMIHYPERTROPHY

Proteus syndrome is a rare congenital hamartomatous malformation that is characterized by a wide range oi deformities, including craniofacia l deformities. Skin and skeletal developmental malformations are commo n and may assume tremendous proportions. The syndrome is often mistake n for other, more commonly recognized conditions, including neurofibro matosis. The soft-tissue masses in Proteus syndrome are not nerve tumo rs but are usually hamartomatous proliferations, The case report descr ibes its varied manifestations, which include significant craniofacial dysmorphism, and discusses the differential diagnosis and management.