Precocious pseudopuberty due to autonomous ovarian cysts: a report of ten cases and long-term follow-up.

We report the findings and clinical course of ten girls aged 0.2 to 6.3 years with precocious pseudopuberty due to autonomous ovarian cysts. We found elevated oestrogen levels in five patients and failure of gonadotropin response to GnRH stimulation in four patients during the first episode, of the disease. In one patient, a GnRH stimulation test was not performed. Pelvic ultrasound examination showed large ovarian cysts in all ten patients. Following the initial episode the secondary sexual characteristics of nine patients regressed completely without treatment. The cyst of one girl was removed surgically on demand of her parents. Three girls presented recurrent autonomous ovarian cysts. Two of these girls developed central precocious puberty requiring treatment with a GnRH-agonist after repeated episodes of precocious pseudopuberty. We started treating the third girl with a GnRH agonist after the second relapse of the autonomous ovarian cyst because of rapidly advancing bone age. We conclude that in the majority of cases autonomous ovarian cysts regress spontaneously and that surgery is in general not indicated. Furthermore, autonomous ovarian cysts can relapse before the onset of physiological puberty and accelerate biological maturation leading to central precocious puberty and consequent decrease of height potential.