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March 1970

Picture of the Month

Author Affiliations

Boston; New Britain, Conn
From the New Britain Memorial Hospital, New Britain, Conn.

Am J Dis Child. 1970;119(3):255-256. doi:10.1001/archpedi.1970.02100050257014

Denouement and Discussion 

Aglossia-Adactylia Syndrome 

Manifestations  Major manifestations include total absence of the tongue (occasionally only a small portion of the tongue may be present posteriorly) associated with failure of development of the distal portion of the limbs. Micrognathia is usually quite marked. Other oral findings include missing lower incisors, cleft or high arched palate, intraoral bands, and hypertrophic enlargement of the sublingual and submaxillary glands. Speech is usually not severely impaired. There may be a nasal quality to the voice and difficulty in pronouncing various consonants. Swallowing function is only minimally impaired. Any part of the limbs may be missing, but abnormalities of the digits are most frequently found. Transposition of the abdominal organs and dextrocardia have also been reported. Intelligence is usually normal.

Genetics  The syndrome does not appear to be inherited. Chromosomal analyses thus far have been normal. Both sexes are equally affected. The etiology is unknown.

Treatment  The orthopedic and dental abnormalities should be corrected whenever possible.

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