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Oromandibular and limb hypogenesis syndrome: treatment report. 2013

Mario Cappellette, and Carolina Marins Ferreira da Costa, and Manuel de Nobrega, and Walter Cerveira de Oliveira, and Luc Louis Maurice Weckx
Mouth Breathing Center, Division of Pediatric Otorhinolaryngology, UNIFESP, São Paulo Hospital, São Paulo, Brazil. Electronic address: mjrcappellette@terra.com.br.

The oromandibular and limb hypogenesis syndrome is characterized by aglossia or hypoglossia presenting with limb anomalies. In this case report, we describe congenital hypoglossia associated with glossopalatine ankylosis and middle finger hypomelia, a type III-D malformation in the Hall Classification. The orthodontic and surgical treatment consisted of 3 maxillary expansions, mandibular surgery, and dental alignment. This extensive treatment resulted in acceptable function and esthetic appearance; however, because of lingual hypoglossia, it did not effect perfect occlusion.

UI MeSH Term Description Entries
D009971 Orthodontics, Corrective The phase of orthodontics concerned with the correction of malocclusion with proper appliances and prevention of its sequelae (Jablonski's Illus. Dictionary of Dentistry). Corrective Orthodontics
D010155 Palatal Expansion Technique An orthodontic method used for correcting narrow or collapsed maxillary arches and functional cross-bite. (From Jablonski's Dictionary of Dentistry), Maxillary Expansion,Palatal Expansion Technic,Expansion, Maxillary,Palatal Expansion Technics,Palatal Expansion Techniques,Technic, Palatal Expansion,Technique, Palatal Expansion
D005260 Female Females
D005385 Fingers Four or five slender jointed digits in humans and primates, attached to each HAND. Finger
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000015 Abnormalities, Multiple Congenital abnormalities that affect more than one organ or body structure. Multiple Abnormalities
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes
D018640 Stomatognathic System Abnormalities Congenital structural abnormalities of the mouth and jaws, including the dentition. Abnormalities, Stomatognathic System,Abnormality, Stomatognathic System,Stomatognathic System Abnormality

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