A case of distal arthrogryposis syndrome with a tongue anomaly

• Published in: Japanese Journal of Oral and Maxillofacial Surgery Vol. 41; no. 9; pp. 826 - 828
• Main Authors: NAKAMURA, Miho, KUWAHARA, Miyoko, OKUI, Kanzoh, HIBI, Goroh, OKA, Tohru, ASANO, Yoshizoh
• Format: Journal Article
• Language: English
• Published: Japanese Society of Oral and Maxillofacial Surgeons 20.09.1995
• Subjects: distal arthrogryposis syndrome; tongue anomaly; tongue function
• ISSN: 0021-5163; 2186-1579
• DOI: 10.5794/jjoms.41.826

We report a rare case of distal arthrogryposis syndrome associated with a tongue anomaly. A 3-year-old girl visited us complaining of articulation and aesthetic disorders, caused by a tongue anomaly and a tongue habit. At birth, she had flexion contracture of both fingers, equinus deformity of both feet, slight abduction disturbance of the right hip joint and a tongue anomaly, in which the tongue was shaped like a cylinder. There were five similar cases of tongue anomaly within her family, whereas no other case had bone or joint disorders. The diagnosis of this case was distal arthrogryposis syndrome type II. The width of the maxillary dentition was decreased. The length of the maxillary and mandibular dentition was increased. The patient had an articulation disorder, evaluated as fricative alveolar/s, z/, but she articulated single sounds almost clearly. She had no difficulty in everyday conversation. To manage her tongue habit, she uses a tongue-guard while asleep.