EARLY reports, related findings, and an excellent bibliography of the rare aglossia-adactylia syndrome have been published.1 There is no apparent familial influence; however, intrauterine environmental factors have been suggested as part of the possible etiology.1 Reported 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.1-4 Systemic 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.1-4 If 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
Kelln EE, Bennett CG, Klingberg WG. Aglossia-Adactylia Syndrome. Am J Dis Child. 1968;116(5):549-552. doi:10.1001/archpedi.1968.02100020553020