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

Abstract

Objective

To 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.

Design

A retrospective review of patients with CS from 1980 to 2022.

Setting

The study was conducted at a tertiary care pediatric hospital.

Patients

Patients 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 Measured

Age 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.

Results

Of 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.

Conclusions

Timely 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.