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Aglossia-Adactylia Syndrome FREE

Elmer E. Kelln, DDS, MSD; Carroll G. Bennett, DDS, MS; William G. Klingberg, AB, MD
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Received for publication June 13, 1966.

Reprint requests to Department of Pedondontics, West Virginia University, Medical Center-School of Dentistry, Morgantown, WVa 26506 (Dr. Bennett).


Am J Dis Child. 1968;116(5):549-552. doi:10.1001/archpedi.1968.02100020553020.
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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

REFERENCES

Gorlin, R.J., and Pindborg, J.J.: Syndrome of the Head and Neck , McGraw-Hill Book Co., Inc., New York, 1964;, pp 37-40.
Ardran, G.M.; Beckett, J.M.; and Kemp, F.H.:  Aglossia Congenita , Arch Dis Child 39:389-392 ( (Aug) ) 1964;.
Thornton, M.:  Pierre Robin Syndrome: A Single Clinical History , J Dent Child 33:27-32 ( (Jan) ) 1966;.
Ernst, T., and Meinhold, G.:  Ein Beitrag zur angeborenen Aglossie , Deutsch Zahn Mund Kieferheilk 43:375-384, 1964;.

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References

Gorlin, R.J., and Pindborg, J.J.: Syndrome of the Head and Neck , McGraw-Hill Book Co., Inc., New York, 1964;, pp 37-40.
Ardran, G.M.; Beckett, J.M.; and Kemp, F.H.:  Aglossia Congenita , Arch Dis Child 39:389-392 ( (Aug) ) 1964;.
Thornton, M.:  Pierre Robin Syndrome: A Single Clinical History , J Dent Child 33:27-32 ( (Jan) ) 1966;.
Ernst, T., and Meinhold, G.:  Ein Beitrag zur angeborenen Aglossie , Deutsch Zahn Mund Kieferheilk 43:375-384, 1964;.

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