A striking genetic disorder in which abnormally short
muscles in the jaw make it impossible to open the mouth fully (trismus) and abnormally
short muscles in the hands keep the fingers straight when the hand is flexed back
(pseudocamptodactyly). The difficulty in opening the mouth fully can cause severe
infant feeding problems. It also complicates dental care and intubation for anesthesia.
The hands may be so tightly fisted the infant crawls on his or her knuckles. The
pseudocamptodactyly impairs manual dexterity and causes occupational and social
disability. Many patients require surgical correction of contractures. The syndrome
is considered a type of distal arthrogryposis.
The gene for the syndrome is transmitted as an autosomal dominant trait passing
from parent to child, irrespective of sex. There is variable expressivity but high
penetrance.
The Hecht syndrome has been served as an historic marker. For example, a large
American family with the syndrome was traced to a Dutch girl who migrated to Tennessee
soon after the American Revolution. A large Dutch family with many affected members
has also been described. The Dutch and American families are now believed to be
related.
Hecht syndrome was first described by the pediatrician/geneticist Frederick Hecht
and orthopedist Rodney K. Beals of the Oregon Health Sciences University. They called
the syndrome "Inability to Open the Mouth Fully." It now also goes by the precise
but hard-to-remember name of trismus pseudocamptodactyly syndrome. It has also been
called the Hecht-Beals syndrome and the Dutch-Kentucky syndrome.
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