The carpal angle in the congenital malformation syndromes

Although the carpal angle was initially described by Kosowicz in relationship to the Turner syndrome, it is altered in many other congenital malformation syndromes. The carpal angle was measured in over 600 individuals with congenital malformations both from the C.S. Mott Children's Hospital and The Johns Hopkins Hospital, kindly supplied by Dr. John Dorst. The carpal angle was compared to appropriate standards that the authors derived for different ages for blacks and whites, both male and female. The carpal angle is also affected by position, being greater in ulnar than in radial deviation. The carpal angle was found to be decreased in dyschondrosteosis, Hurler syndrome, Madelung deformity, mesomelic dwarfism, Morquio syndrome and Turner syndrome and increased in arthrogryposis, diastrophic dwarfism, epiphyseal dysplasias, frontometaphyseal dysplasia, hypochondroplasia, Marfan syndrome, Pfeiffer syndrome, spondyloepiphyseal dysplasia and trisomy 21. In the authors' experience the carpal angle was not very discriminating in Turner syndrome. It was more useful in some of the congenital malformation syndromes.