MULTIDISCIPLINARY CARE OF FREEMAN SHELDON SYNDROME |
Ji-hun Kim1, Jeong-Sub Lee2, Cherry Chang1 |
11Department of Pediatric Dentistry, Yonsei University 2Department of Orthodontics, Wonju College of Medicine, Yonsei University |
Freeman-Sheldon Syndrome의 치료에 대한 고찰 |
김지훈1, 이정섭2, 장채리1 |
1연세대학교 원주의과대학 1소아치과학교실 2연세대학교 원주의과대학 교정학교실 |
Correspondence:
Ji-hun Kim, Tel: 033-741-0673, Email: pedo@yonsei.ac.kr |
Received: 25 September 2010 • Accepted: 13 November 2010 |
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Abstract |
Freeman-Sheldon Syndrome (FSS, also known as "Whistling Face Syndrome") is a rare genetic condition which characteristically includes a small "whistling" mouth, a flat mask-like face, club feet, joint contractures usually involving the fingers and hands, and under-development of the cartilage of the nose. Intelligence is usually normal. Most of the features of this syndrome are due to muscle weakness. The patient, 11 years old boy was consulted from pediatrics to pediatric dentistry due to dental management. After clinical & radiographic examinations, severe multiple problems were found. Dental problems were microsomia(whistling mouth) & micrognathia, perioral muscle contracture, restricted mouth opening, poor oral hygiene & care, generalized dental caries, high palatal vault, severe malocclusion & crowding. And Orthopedic problems, ophthalmic & respiratory, anesthetic problems were found. Then He also had psychiatric problem, hospital(dental) phobia due to previous medical history(frequent hospitalization). And he had genital problem, cryptochidism, too. Due to these intricate problems, he suffered with feeding, swallowing difficulties and showed growth retardation. For enhancing patient's oral health, pediatric dentist, orthodontist, oral surgeon, pediatrician, psychiatrist, orthopedist, they all agree with early, cautious intervention and treatment. So, he has been treated by multidisciplinary care, now he is recovering general health maintenance. |
Key Words:
Freeman Sheldon Syndrome, Whistling face syndrome, Multidisciplinary care
Abstract |
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