Congenital upward displacement of the scapula almost always occurs sporadically. However, Gottesleben (1927) observed 9 cases in 6 sibships of 3 generations of a family with male-to-male transmission. Schwarzweller (1937) found 2 affected sibs in 2 out of ... Congenital upward displacement of the scapula almost always occurs sporadically. However, Gottesleben (1927) observed 9 cases in 6 sibships of 3 generations of a family with male-to-male transmission. Schwarzweller (1937) found 2 affected sibs in 2 out of 9 families. In one of these the father had mild abnormality. Aubert and Arroyo (1967) observed the disorder in father and daughter. In another family reported by Perls (cited by Engel, 1943), a father and 2 sons had unilateral elevated scapulae. Wilson et al. (1971) reported a family in which affected persons were thought to have occurred in multiple sibships of 5 successive generations with instances of male-to-male transmission. Thus, there is probably a simple mendelian form of Sprengel deformity, which represents a minority of cases. Hodgson and Chiu (1981) described a family with transmission of Sprengel deformity with cleft palate through 3 successive generations (grandmother, son, and granddaughter). The great-grandfather had Sprengel deformity only. Some of the affected persons showed Klippel-Feil syndrome (118100).