Sexual Infantilism in Females1

Abstract

Sexual infantilism in [female][female] may be due to (1) primary ovarian deficiency or (2) pituitary deficiency causing secondary hypoovarianism. In the first group of cases the excretion of FSH is increased. Pubic hair is present due to the nearly normal production of adrenal androgen. In the 2d group FSH is decreased and in most instances pubic hair is absent and the 17-ketosteroid excretion is low. The authors illustrate the various types of infantilism in both groups with clinical case reports. The commonest type of primary ovarian deficiency is ovarian agenesis in which the ovary fails to develop beyond the stage of the primitive genital ridge. There are 38 such cases reported in the literature of which 12 have been studied pathologically. Stunted growth occurred in all except one and is believed by the writers to be due to an associated genetic defect. There are a few cases in the literature in which the development of the ovary was apparently arrested at a stage a little more advanced than that of the primitive genital ridge and a few primordial follicles were present. These patients were not all of short stature. Degeneration of the ovary or castration during the preadolescent period lead to the tall eunuchoid type of development. Generalized pituitary deficiency causes dwarfism and sexual infantilism. There are instances in which the clinical picture is not complete. In Froeh-lich''s syndrome sexual infantilism results from failure or interruption of the gonadotrophic stimulation of the pituitary. Such patients may be somewhat short or of normal height and may have eunuchoid proportions. Primary ovarian deficiency is treated by estrins and progestins in cyclic periods with interruptions. Theoretically pituitary infantilism should be treated with gonadotrophins, but results obtained with available prepns. are unsatisfactory. Accordingly pituitary infantilism is also treated with the 5 sex hormones and when pubic hair is absent androgens are given simultaneously.