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

Camryn Berry; Alison M Boyce; Leonard B Kaban; Zachary S Peacock; Michael Mannstadt; Jaymin Upadhyay; Craniofacial Fibrous Dysplasia Research Team

doi:10.1016/j.joms.2024.12.001

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

J Oral Maxillofac Surg. 2024 Dec 4:S0278-2391(24)00977-7. doi: 10.1016/j.joms.2024.12.001. Online ahead of print.

Authors

Camryn Berry¹, Alison M Boyce², Leonard B Kaban³, Zachary S Peacock⁴, Michael Mannstadt⁵, Jaymin Upadhyay⁶; Craniofacial Fibrous Dysplasia Research Team

Collaborators

Craniofacial Fibrous Dysplasia Research Team:
Hanne van der Heijden, Emma Golden, Anthony Westbrook, Nehal Shah, Laura A Drubach, Stephan Voss, Neha Kwatra, Laura Romo, David Ebb, Mariesa Cay

Affiliations

¹ PhD Candidate, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA.
² Principal Investigator, Metabolic Bone Disorders Unit, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD.
³ Chief, Emeritus, Department of Oral & Maxillofacial Surgery, Harvard School of Dental Medicine, Boston, MA; Chief, Emeritus, Division of Oral and Maxillofacial Surgery, Massachusetts General Hospital, Boston, MA.
⁴ Chief, Department of Oral & Maxillofacial Surgery, Harvard School of Dental Medicine, Boston, MA; Chief, Division of Oral and Maxillofacial Surgery, Massachusetts General Hospital, Boston, MA.
⁵ Chief, Endocrine Unit, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
⁶ Assistant Professor, Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, MA; Assistant Professor, Department of Psychiatry, McLean Hospital, Harvard Medical School, Belmont, MA. Electronic address: [email protected].

PMID: 39710366
DOI: 10.1016/j.joms.2024.12.001

Abstract

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.