Characterization of dental phenotypes and treatment modalities in Korean patients with Parry–Romberg syndrome

Abstract

Objective: To investigate the dental phenotypes and treatment modalities (Tx-Mod) in Korean patients with Parry-Romberg syndrome (PRS) using longitudinal data. Methods: The samples consisted of 10 PRS patients, who were treated and/or followed-up at Seoul National University Dental Hospital between 1998 and 2019. Using a novel PRS severity index based on the numbers of the atrophy-involved area and asymmetry-involved item, we classified them into mild (n = 3), moderate (n = 2), and severe (n = 5). Dental phenotypes, including congenitally missing tooth (Con-Missing-Tooth), microdontia, tooth with short root (Short-Root), tooth with dilacerated root, and delayed eruption/impacted tooth, were investigated along with Tx-Mod. Results: The side of occurrence of all dental phenotypes showed 100% concordance with the side of PRS involvement. The most two common dental phenotypes were Con-Missing-Tooth and Short-Root (n = 29 and n = 17 in six patients). The sums of the average number of Con-Missing-Tooth and Short-Root increased from mild PRS to moderate PRS and severe PRS cases (1.0, 6.0, and 6.2). In terms of Tx-Mod, growth observation due to mild atrophy, fixed orthodontic treatment, and grafting were used for mild PRS cases. Tx-Mod for moderate PRS cases involved growth observation for surgery due to an early age at the initial visit. For severe PRS cases, diverse Tx-Mod combinations including unilateral functional appliance, fixed orthodontic treatment, growth observation, grafting, and orthognathic surgery were used. Conclusions: The novel PRS severity index may be useful to provide primary data for individualized diagnosis and treatment planning for PRS patients.

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

“…The Cleft Palate Craniofacial Journal 60 (8) beginning dental and orthodontic treatment at an earlier age among this patient population. This study corroborates previous studies noting the possibility of tooth hypoplasia 13,14,16,17,19,21,23,24 , agenesis 15,23 and delayed eruption 14,16,17,20,23,24 in patients with Parry-Romberg Syndrome. Providers should consider these anomalies in their evaluation, planning, and treatment of patients with Parry-Romberg Syndrome.…”

“…There are a number of oral health concerns in Parry-Romberg Syndrome, reported primarily in small series documenting findings that involve soft tissue (ie tongue atrophy, gingival exposure, lip incompetence), the jaws (ie temporomandibular joint dysfunction, malocclusion), and teeth (ie agenesis, delayed eruption, and root atrophy). [13][14][15][16][17][18][19][20][21][22][23][24] The aim of this study was to review the dental findings in patients with Parry-Romberg Syndrome treated at our institution over the past four decades to document anomalies noted in this patient population, to identify situations in which early referral may help prevent worse dental outcomes, and to inform multidisciplinary care for this patient population. This is the largest study of dental anomalies in Parry-Romberg Syndrome reported to date.…”

Combined Surgical-Orthodontic Treatment of Patients With Severe Parry-Romberg Syndrome

“…8 Dental phenotypes generally involve root resorption, tooth curvature and cracks, etc. 9 Glandular features include reduced salivary gland secretion, dry mouth, and atrophy of half tongue. 10 A few cases are accompanied with vitiligo, scleroderma, intracranial aneurysm, etc.…”

Parry Romberg Sendromlu Pedi̇atri̇k Bi̇r Hastanin Nadi̇r Oral Bulgulari: Bi̇r Olgu Sunumu

“…Travma, enfeksiyon, genetik faktör, epilepsi, migren, trigeminal nevrit, lenfositik nörovaskülit, lokalize skleroderma, endokrin bozukluğu, otoimmünite, sempatik sinir sisteminin hiper-veya hipo-aktivitesi ve serebellumun yağ metabolizmasında bozukluk gibi çok sayıda neden ileri sürülmüştür. 11,12 Az sayıda hastada vitiligo, tiroid sorunları, sistemik skleroz, inflamatuar barsak hastalığı, romatoid artrit, ankilozan spondilit, sistemik lupus eritematozus ve multipl skleroz gibi diğer otoimmün bozukluklar da rapor edilmiştir. Eşlik eden ağız ve çevre dokularında alın, yanaklar, çene, dudaklar, dişler, diş etleri ve dil tutulumu görülmüştür.…”