Author(s): Lecannellier J, Vischer D
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Abstract We present two own cases of the aglossy-adactyly syndrome and a review of 25 cases of the literature. Our experience concernes two girls. One was followed from birth until 4 1/2 years, the other from 5 to 9 years. This helped us to obtain a good knowledge of the development of these patients. Malformations of the mouth and of the limbs with a normal intelligence characterize the aglossy-adactyly syndrome. The most important features are: shortness of the tongue of variable degree, anomaly of the teeth, hypoplasia of the mandible and ectromelia of different extension with a peripheric predilection and often with an asymmetry. The etiology is unknown. All the known cases are sporadic. The differential diagnosis of the aglossy-adactyly syndrome includes the ankyloglossia superior, the Hanhart syndrome and the oro-farcio-digital syndrome I. Even when the mouth malformations are of a great degree, the functional limitations of the patients with aglossy-adactyly syndrome are very small. This is important in determining the prognosis, in consulting the patients and their parents and for decisions about corrective operations.
This article was published in Helv Paediatr Acta
and referenced in Journal of Communication Disorders, Deaf Studies & Hearing Aids