Concomitant Temporomandibular Joint Ankylosis and Maxillomandibular Fusion in a Child with Klippel- Feil Syndrome: A Case Report

Klippel-Feil syndrome (KFS) is classically characterized by fusion of any of the two of seven cervical vertebrae. It is identified by the presence of a triad of clinical signs including short neck, limitation of head and neck movements and low posterior hairline. Unusual bony malformations leading to facial asymmetry is the most common oral manifestation associated with KFS. Such maxillomandibular fusion can also result in restricted mouth opening in children. It's a challenge to provide complete rehabilitation in such children. This paper presents a report of a type II KFS with both maxillomandibular fusion and temporomandibular joint ankylosis which led to the limited mouth opening in a six-year-old child. Also, the child showed an inadequate development of speech, facial asymmetry and compromised oral health owing to the restricted mouth opening. After thorough investigations, surgery was done which restored limited mouth opening which led to normalizing of speech and oral health.