Korean J Orthod.  2020 Nov;50(6):407-417. 10.4041/kjod.2020.50.6.407.

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

• Yim Sj
• Yang IH
• Baek SH

• KMID: 2508449
• DOI: http://doi.org/10.4041/kjod.2020.50.6.407

Abstract

Objective
To investigate the dental phenotypes and treatment modalities (TxMod) 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-MissingTooth 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 TxMod, 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.

Keyword

Parry–Romberg syndrome; Dental phenotype; Treatment modality; Parry–Romberg syndrome severity index

Figure

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  Figure 1 Cephalometric and panoramic radiographs acquired from patients with Parry–Romberg syndrome (PRS) stratified by the PRS severity index. A, A patient with mild PRS (patient #8, left side involvement). B, A patient with moderate PRS (patient #5, right side involvement). C, A patient with severe PRS (patient #9, left side involvement).

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  Figure 2 Facial photographs showing soft tissue atrophy and facial asymmetry in patients with Parry–Romberg syndrome. A, Soft tissue atrophy in the forehead (patient #7, left side involvement). B, Soft tissue atrophy in the peri-orbital tissue (patient #6, right side involvement). C, Soft tissue atrophy in the middle and lower thirds of the face, with a difference in the facial soft tissue volume between the affected and unaffected sides, deviation of the oral commissure, and chin point deviation (patient #2, right side involvement).

• 

  Figure 3 Treatment outcome of Parry–Romberg syndrome (PRS) patients according to the dental phenotype. A, Patient #8 (left side involvement) had congenital missing of #23 and #25 (x-mark), impaction and short root of #24 (arrow), and prolonged retention of #63 and #65. After extraction of #65, #24 was aligned using forced eruption with fixed orthodontic treatment. #63 was maintained for substitution of #23 and spaces were created on the mesial and distal sides of #63 to compensate for the absence of #25. B, Patient #6 (right side involvement) had multiple congenital missing of #12, #14, #16, #17, #41, #44–#47 (x-mark) and microdontia with short root of #13 and #15 (arrow). She was treated with orthognathic surgery with fixed orthodontic treatment. Then, multiple-unit bridges (#11-X-X-13-15 and #31-X-42-43-X) were fabricated; dental implants could not be placed because of severe atrophy of the alveolar bone. C, Patient #9 (left side involvement) had multiple congenital missing of #21–#27, #31, #32, #34, and #35 (x-mark). She was treated with orthognathic surgery with fixed orthodontic treatment. Then, congenitally missing teeth were replaced by retained deciduous teeth and dental implant prosthesis. D, Patient #7 (left side involvement) had dilacerated root of #22 (arrow). It was aligned at an appropriate location without side effect such as root resorption.

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