The syndrome of gonadal dysgenesis, or Turner's syndrome, is essentially a combination of a chromosomal aberration, an endocrine disorder, and a number of congenital somatic malformations. Although coarctation of the aorta is the anomaly most frequently thought of regarding visceral defects associated with gonadal dysgenesis, it is in fact far less commonly found than abnormalities of the urological tract.
In a review of patients with Turner's syndrome seen at the Columbia-Presbyterian Medical Center, Baker et al1 in 1967 found a high incidence of renal anomalies, the most common of which were malrotation and horseshoe kidneys. In addition, varying degrees of renal atrophy from unsuspected vesicoureteral reflux or ureteropelvic junction obstruction were also found. Since this review was published, we have had the opportunity to study another patient with Turner's syndrome, who was found to have a retrocaval ureter with a moderate degree of hydronephrosis on one side, while the