Gonadotropic status in adult women with pituitary stalk interruption syndrome
Résumé
Objective
Pituitary stalk interruption syndrome (PSIS) is a rare cause of congenital hypopituitarism. Limited data exist on the gonadotropic status and fertility of adult women with PSIS. Our study aims to describe pubertal development and the evolution of gonadotropic function and fertility in adult women with PSIS. Design Retrospective multicentric French study.
Methods
We described gonadotropic function in 56 adult women with PSIS from puberty onwards. We compared live birth rates per woman with PSIS to age-matched controls from the large French epidemiological cohort (CONSTANCES). Additionally, we assessed height, BMI, blood pressure, other metabolic parameters, and socio-economic status.
Results and Conclusions
Among 56 women with PSIS, 36 did not experience spontaneous puberty. Of these, 13 underwent ovarian stimulation resulting in 7 women having a total of 11 children. In the subgroup with spontaneous puberty (n = 20), 4 had a total of 8 pregnancies while 6 developed secondary gonadotropic deficiency. Women with PSIS had fewer children than controls (0.33 versus 0.63, p = 0.04). Median height was also lower (160.5 cm versus 165.0 cm, p < 0.0001). Although mean blood pressure was lower in women with PSIS compared to controls, (111.3/65.9 ± 11.2/8.1 mmHg vs 118.7/72.1 ± 10.1/7.7 mmHg, p < 0.001), there were no significant differences in other metabolic parameters, notably BMI and lipid profile. Employment/academic status was not different in the two groups but fewer women with PSIS were in relationships (42% versus 57.6% in controls, p = 0.02). The fertility prognosis in patients with PSIS needs optimization. Patients should be informed about the likelihood of declining gonadotropic function over time.