Pierre Robin sequence with severe scoliosis in an adult: A case report of clinical and radiological features
10.5624/isd.2019.49.4.323
- Author:
Jae Jun KIM
1
;
Dong Soon CHOI
;
Insan JANG
;
Bong Kuen CHA
;
In Woo PARK
Author Information
1. Department of Orthodontics, College of Dentistry, Gangneung-Wonju National University, Gangneung, Korea. korth@gwnu.ac.kr
- Publication Type:Case Report
- Keywords:
Pierre Robin Syndrome;
Scoliosis;
Micrognathism
- MeSH:
Adult;
Airway Obstruction;
Female;
Glossoptosis;
Humans;
Micrognathism;
Pierre Robin Syndrome;
Ribs;
Scoliosis
- From:Imaging Science in Dentistry
2019;49(4):323-329
- CountryRepublic of Korea
- Language:English
-
Abstract:
Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and airway obstruction. PRS does not have a single pathogenesis, but rather is associated with multiple syndromes. This report presents the case of a 35-year-old woman with PRS and scoliosis. Among the syndromes related to PRS, cerebro-costo-mandibular syndrome (CCMS), which is characterized by posterior rib gap defects and vertebral anomalies, was suspected in this patient. However, no posterior rib gap defect was detected on radiological examinations. Although over 80 cases of CCMS have been reported to date, few cases of PRS with scoliosis alone have been reported. Therefore, this report demonstrated the clinical, radiological, and cephalometric characteristics of an adult patient with PRS and scoliosis, but without rib anomalies.
