Myelodysplasia is a relatively common anomaly occurring in 1 to 5 per thousand live births. It is seen more frequently in girls. The severity of developmental defect and the resultant neurological impairment is variable; however, hydrocephalus is a common finding.1 True precocious puberty, premature activation of the hypothalamic-pituitary-gonadal axis, also occurs more frequently in girls.2,3 It has been our clinical impression that sexual precocity is more common in girls with myelodysplasia than in otherwise healthy children. To assess the degree and nature of this association, if any, we retrospectively studied 62 girls with myelodysplasia.


All female patients with myelomeningocele attending our multidisciplinary myelodysplasia clinic at the New England Medical Center (Floating Hospital for Children) between September 1989 and May 1991 were studied.

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