Sir.—I have two comments regarding the December 1985 issue.
It is not clear from the commentary by Hecht1 whether the family that he had initially counseled relative to the EEC [ectrodactyly-ectodermal dysplasia-clefting] syndrome had, in their second child, the problem of ectrodactyly. The commentary states that the second child had a cleft lip and palate, but it does not say (at least as I read it) whether the child had the split hand and foot.
My understanding relative to the EEC syndrome is that ectrodactyly occurs essentially in 100% of patients and that the other manifestations are less common. It would seem to me that if the second child did not have ectrodactyly but only had the cleft lip and palate, then the family still had (1) a 50% chance for the EEC syndrome in any pregnancy, and (2) a polygenic chance (2% to 4% chance) for having
DONLAN MA. Two Problems: Diagnosing the EEC/EECUT Syndrome and Recommending Dietary Fiber. Am J Dis Child. 1986;140(7):617. doi:10.1001/archpedi.1986.02140210015004
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: