D159. Surgical Outcomes In Juvenile Craniofacial Linear Scleroderma Patients: A 10-year Single Center Cohort

PURPOSE: Juvenile linear scleroderma (LS) subtypes affecting the face include en coupe de sabre, Parry-Romberg syndrome, mixed morphea, and circumscribed morphea. All subtypes can cause marked facial asymmetry corrected with procedures such as autologous fat grafting, hyaluronic filler injection, implant, rhinoplasty, bony reconstruction, and facial reanimation. We report the surgical treatment of craniofacial juvenile LS over a 10-year period. METHODS: A retrospective review was performed on 393 patients diagnosed with LS from January 2012 to October 2022. Out of 77 identified craniofacial LS patients, 24 had undergone surgical treatment and were included. RESULTS: The mean age of diagnosis was 9 years (range: 2-17). Most patients were female (64%) and Hispanic (55%). Diagnoses included en coupe de sabre (39%), Parry-Romberg syndrome (21%), both (18%), mixed morphea (17%), and circumscribed morphea (5%). 71% were on systemic medications including corticosteroids (76.4%), methotrexate (96.4%), mycophenolate mofetil (16.4%), biologics (7.3%), and other (36.4%). Patients underwent surgery at a mean age of 13 (range: 5-18). The most common procedure was autologous fat grafting (n=22) followed by rhinoplasty (n=2), facial implant (n=2), regional flap (n=2), free flap (n=2), facial reanimation (n=1), hyaluronic filler (n=1), and le fort I osteotomy (n=1). The mean number of fat grafts per patient was 2 (range: 1-7). Complication rates were low (4.2%). No complications were seen following fat grafting. CONCLUSION: Most patients achieve adequate facial balance with 2 rounds of autologous fat grafting. Surgical intervention in JLS is safe and does not result in an increased incidence of disease relapse.