Identifying Pain Subtypes in Patients With Craniofacial Lesions of Fibrous Dysplasia/McCune-Albright Syndrome.

BACKGROUND

Fibrous dysplasia/McCune-Albright syndrome (FD/MAS) is a genetic disorder, marked by bone lesions, often affecting the craniofacial skeleton. Pain is a prevalent yet heterogeneous symptom reported by patients with craniofacial FD. Effective treatments are currently lacking, posing a significant clinical challenge to patient care.

PURPOSE

This preliminary study examined pain profiles in craniofacial FD and aimed to identify subtypes of patients based on pain phenotypes and emotional health.

STUDY DESIGN, SETTING, SAMPLE: A prospective, cross-sectional study involving 15 patients with FD/MAS, conducted at Boston Children's Hospital and Massachusetts General Brigham's Hospitals.

PREDICTOR/EXPOSURE/INDEPENDENT VARIABLE: Headache frequency, craniofacial pain severity, neuropathic pain quality, pain interference, allodynia, photophobia, depression, and anxiety were assessed using clinical questionnaires.

MAIN OUTCOME VARIABLE(S): The primary outcome variable was the symptom profile derived from standardized clinical questionnaires and analyzed using principal component analysis and K-means clustering.

COVARIATES

Covariates included demographic data, diagnosis, and lesion location(s).

ANALYSES

Principal component analysis and K-means clustering of patient-reported measures of pain and emotional health were performed. Analysis of variance was conducted to determine significant differences among patient subtypes. Statistical significance was set at (P < .05).

RESULTS

The study included 15 subjects with FD/MAS, with a mean age of 36.2 (13.9) years, including 1 male. Clustering analysis identified 3 subtypes of patients with distinct symptom profiles. Cluster 1 (n = 2) averaged 70 (28.3) headache days in a 90-day period, pain level of 7.5 (0.7) on a 0-10 scale, and severe anxiety, depression, allodynia, photophobia, and pain interference. Cluster 2 (n = 7) patients reported an average of 5.4 (7.5) headache days, an average pain level of 2.7 (2.6), mild or no anxiety, depression, allodynia, photophobia, and pain interference. Cluster 3 (n = 6) patients displayed a mixed symptom profile with an average of 47.3 (36.4) headache days and a pain level of 5.25 (1.4). Notably, patients with temporal and skull base lesions were predominantly found in Clusters 1 and 3, which exhibited the most severe symptomatology.

CONCLUSIONS AND RELEVANCE

This study establishes a basis for future longitudinal research aimed at understanding underlying pain mechanisms and evaluating the response to personalized pain management strategies in subtypes of patients with craniofacial FD.