PROTEUS SYNDROME - 8 CASES

Introduction. Proteus syndrome most often includes: corporal hemi-hypertrophia, gigantism of the extremities with hyperplasia of the palms and soles, vascular abnormalities and varied types of other hamartomatous tumours. The dysmorphic growth generates functional orthopaedic and orthognatic disabilities that increase with age. Observations. We report, on eight cases, our experience in management of Proteus syndrome abnormalities and the benefits of new techniques in imaging and interventional vascular radiology. The correction of limb orthopaedic abnormalities is complicated with elephantiasic enlargement of soft tisues. In some patients the absence of a deep venous network contra-indicates transcutaneous occlusion of dysplatic vessels. Facial asymmetry and orthognatic anomalies are better managed after permanent teeth have appeared. Commentaries. The management of these disabilities is carried out by staff of different specialities but should be organized by a practitioner who regularly follows the child and detects early the functional disturbances. Aesthetic corrections are performed later.