Parry Romberg syndrome: A long-term retrospective cohort study of 10 patients

• Main Authors: F. Van der Cruyssen, J. Meeus, J. Schoenaers, C. Politis
• Format: Article
• Language: English
• Published: Elsevier 2018-09-01
• Series: Oral and Maxillofacial Surgery Cases
• Online Access: http://www.sciencedirect.com/science/article/pii/S2214541918300245

We performed a retrospective cohort study of patients included in the database of the department of Oral & Maxillo-Facial Surgery between 2006 and 2016 at the University Hospitals Leuven with Parry Romberg syndrome to assess and compare initial presentation, diagnostic methods, and different surgical and non-surgical approaches. Primary outcomes were functional and esthetic results using intra- and extra-oral images, clinical orthognathic measurements, radiological cephalograms, and cone beam computed tomography, including three-dimensional cephalometry analyzing the facial symmetry of hard tissues. The secondary outcome was patient quality of life using the Ferrans and Powers Generic Quality of Life Index. Ten patients were included; two had medical treatment, three had reconstructive surgery, four had orthognathic surgery, and three had lipofilling. Two patients had post-surgical infectious complications. Two patients developed neuropathic pain. Three-dimensional cephalometry showed no significant difference with regards to anatomical bony landmarks between the affected and non-affected sides. A volumetric analysis showed a significant difference (P=0.04) in maxillary volumes. Seven patients were satisfied with their aesthetic and functional outcomes. Mean follow up was eleven years. In conclusion, this study should be interpreted carefully due to small sample size. We feel most patients can be treated conservatively or with minor aesthetic corrections using fat grafting methods. Use of allografts and osteosynthesis materials was associated with an increased risk of postoperative superinfection. Fat grafts produced predictable, noncomplicated results and can be used during disease progression. Three-dimensional analysis showed acceptable symmetry of the bony framework on follow-up. Keywords: Parry Romberg syndrome, Hemifacial atrophy, Facial muscular wasting, Facial scleroderma, Facial lipodystrophy