Background and objectives : Progressive Facial Hemiatrophy Syndrome (PFH) is characterized by a slow progressive atrophy that appears in early stages of life, affecting the subcutaneous tissue and subjacent fat and, in most serious cases comprises the maxillofacial skeleton on 1 side of the face. Numerous techniques have been described to improve facial contour, however, there is no consensus in the literature concerning treatment. The aim of this work is to present an alternative for treatment of the skeletal asymmetry in a patient with PFH based on a maxillomandibular distraction using a modified Le Fort III osteotomy.

Patient and method : A 15-year-old male patient with a severe PFH was admitted to the oral and maxillofacial surgery department of the Exequiel Gonzalez Cortes Children Hospital, Santiago, Chile. A modified Le Fort III osteotomy was done on the left side via an intraoral and a transconjunctival approach with lateral canthotomy and a Le Fort I osteotomy on the right side. A horizontal mandibular osteotomy on the left side was performed fixing at the same time a bone distractor. Then, with maxillomandibular fixation, the distrator was activated until the maxillae reached a right position. This allowed to achieve a mid and low facial bone distraction in the vertical and sagittal plane.

Results : The outcome of this treatment was an optimal bone contour of the facial skeleton after 20 mm bone distraction and five weeks of contention period. The horizontalization of the occlusal plane, an equal position of mandibular angles in the vertical plane and pogonion sagittal projection was achieved.

Conclusions : The mid and low facial bone distraction with a modified Le fort III osteotomy combined with a mandibular distraction is a good alternative to correct skeletal atrophy in severe cases of PFH.

Key words : modified Le Fort III; Parry–Romberg syndrome; maxillomandibular distraction