Aglossia-Adactylia Syndrome

Abstract

EARLY reports, related findings, and an excellent bibliography of the rare aglossia-adactylia syndrome have been published.1There is no apparent familial influence; however, intrauterine environmental factors have been suggested as part of the possible etiology.1Reported oral findings include total or partial absence of the tongue, an underdeveloped mandible, marked enlargement of the sublingual muscular ridges, hypertrophic enlargement of the sublingual and submaxillary glands, cleft and high arched palate, lower lip defects, missing lower incisors, bony fusion of the dental arches, and intraoral bands.1-4Systemic manifestations that have been reported include hypoplasia of extremities, ranging from complete peromelia to absence of distal digit; syndactylia; absence of fingernails; transposed viscera; and dextrocardia.1-4If intelligence is normal and a major portion of the tongue is not absent, the ability to speak is not severely impaired. We believe the present case is typical. In addition to many of the