PEDIATRICS Vol. 64 No. 4 October 1979, pp. 542-545
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The Langer-Giedion Syndrome: Report of a 22-Year-Old Woman

William G. Wilson MD1, Robert T. Herrington MD1, and Arthur S. Aylsworth MD1

1 Department of Pediatrics, University of North Carolina, Chapel Hill, NC 27514

A 22-year-old woman with the Langer-Giedion syndrome and delayed puberty is presented. Pertinent features include a bulbous nose, sparse hair, protruding ears, multiple cartilaginous exostoses, cone-shaped phalangeal epiphyses, short stature, microcephaly, and mental retardation. She is the oldest patient thus far described with this condition, and is compared to the ten previously published cases. The clinical course of patients with the Langer-Giedion syndrome and the possibility of malignant change in the exostoses have not been established.