Barriers to Surgical Care in Craniofacial Scleroderma (Parry-Romberg Syndrome)

ObjectiveTo assess whether demographic characteristics, referral patterns, nomenclature, anatomic features, and/or socioeconomic factors of patients with Craniofacial Scleroderma (CS) are related to delays in diagnosis or referral for reconstructive surgery.DesignA retrospective review of patients with CS from 1980 to 2022.SettingThe study was conducted at a tertiary care pediatric hospital.PatientsPatients were identified by medical record search for terms "Parry-Romberg," "hemifacial atrophy," "localized scleroderma," "progressive facial atrophy," "craniofacial morphea," "craniofacial scleroderma," "linear morphea," and "en coupe de sabre." Patients were excluded if the diagnosing specialist, age of onset, or age of diagnosis were unknown.Main Outcomes MeasuredAge of onset, diagnosis, sex, nomenclature, anatomic variation, Fitzpatrick skin type, median income, ethnicity, language, travel-distance, referral sequence, and operative interventions were recorded. Two-sample t-tests, one-way analysis of variance, and logistic regression were used to measure association between these variables with delays in diagnosis or referrals to plastic surgery.ResultsOf 124 patients identified, 104 met inclusion criteria (62% female, average age of onset 6.4 ± 4.4 years and diagnosis 9.5 ± 5.4 years). Dermatology most often diagnosed CS (64%). Thirty-five patients (33%) were evaluated by plastic surgery; of these, 45% underwent reconstructive procedures. Age of onset, income, and travel-distance were not statistically associated with delays in diagnosis.ConclusionsTimely diagnosis for patients with CS minimizes disease impact; however, we found that many patients experience years of symptoms before being diagnosed and may not be fully informed of potential reconstructive options. Interdisciplinary care and awareness of treatment options is critical to ensure patients receive prompt and comprehensive care. Recommendations for interdisciplinary care are proposed.