LITTLE PEOPLE: LEGENDS OF THE NORTH

Twenty-five years ago, Prof. Peter H. Beighton, our association’s geneticist, presented a paper reminding us that more than 2 000 genetic diseases and disorders have been identified. Many of the conditions are apparently confined to one particular geographical locality or ethnic group. A large proportion of genetic diseases and disorders has skeletal manifestations. The Little People of South Africa (Association of Persons with Restricted Growth) have needed advice about the management of orthopaedic complications such as spinal problems in achondroplasia, axial deviations of the lower limbs, and in particular the possibilities of limb lengthening in disproportionate skeletal dysplasias. From the story of a young achondroplastic woman who suffered from low back pain and was offered an operation by a neurosurgeon, there stemmed a media-driven report on dwarfs in the Land of Legends near Tzaneen, an epidemiological field study on achondroplasia in the Northern Province, and a combined round table consultation between a team of orthopaedic surgeons and a pair of Pedi sangomas. In the village was an index group of three Pedi women and one man who were diagnosed with probable acrome-somelic dysplasia (Grebe), a form of achondrogenesis. The oldest woman and the man were brother and sister, and the two younger women their daughters by spouses of normal stature. While their heads and faces were normal and their spines straight, their dysmorphic features included shortness of stature (mean height 94 cm), disproportionate limb length and ligamentous laxity. The little man’s late father was also a dwarf, as was one of his eight brothers: there were thus six dwarfs in a direct line in three generations. The dwarf man and woman were both sangomas, as their father had been. None of them had low back pain, but they knew how to cure it.