Dental Anomalies in Parry-Romberg Syndrome: A 40-Year Retrospective Review

Abstract

Objective This study investigates crown and root anomalies in patients with Parry-Romberg Syndrome. Design This is a retrospective review of patients with Parry-Romberg Syndrome who were evaluated at a tertiary care center from 1980-2020. Setting Patients seen in the dental unit from 1980-2020. Patients, Participants Seventeen patients with documented Parry-Romberg Syndrome were referred for dental evaluation. Main Outcome Measures All dental anomalies were documented. Root anomalies were assessed using panoramic radiographs and cone beam CT (CBCT) scans to evaluate buccal-lingual, mesio-distal, and axial measurements of hypoplastic teeth, which were compared to those of contralateral teeth. Results Findings included agenesis (29%, n = 5), hypoplastic teeth (29%, n = 5), delayed canine eruption (24%, n = 4), and mulberry molars (12%, n = 2). Of the five patients with tooth hypoplasia, four had CBCT records and the fifth had panoramic radiographs available for assessment. Axial length was always shorter in hypoplastic teeth relative to contralateral teeth, with differences ranging from 1.2-9.2 mm. Differences in crown size of hypoplastic versus contralateral teeth were unpredictable but always present. Conclusions Patients with Parry-Romberg Syndrome can have hypoplastic roots with atypical crown morphology. A patient’s specific dental anomaly will influence planning and treatment.

Oral characteristics in adult individuals with periodontal Ehlers‐Danlos syndrome

“…Short root anomaly, defined as developmentally short blunt dental roots with root-crown ratios ≤1.0 mostly affecting maxillary incisors and mandibular second premolars has incidence rates of 0.49%-2.4% (Jakobsson & Lind, 1973;Apajalahti et al, 2002;Herrera et al, 2021). It was previously reported as a dental feature of rare diseases as OdontoÀ/Hypophosphatasia, Stevens-Johnson syndrome (MIM #608579), Parry-Romberg syndrome (MIM #141300) and Emanuel syndrome (MIM #609029) (Wang et al, 2016;Puranik & Katechia, 2019;Katyal & Yadav, 2021;Aram et al, 2022). Molecular mechanisms of short root anomaly were previously elucidated, although the connection to complement pathway alterations remains still unclear (Yu et al, 2021).…”